Journal Articles Library

medical-journal-libraryWelcome to the CSF Leak Association’s library of CSF leak-related journal articles.

The library database is built around the PubMed system, a respected biomedical literature database comprising more than 26 million citations, and is updated regularly.

Abstracts are provided for most articles. You can view more information on an article by clicking on a ‘view article’ link (where available). For some articles, viewing the full text will require a subscription, but a reasonable number are available to view free-of-charge.

Our favourite articles, and articles that are likely to be of particular relevance to those with an interest in CSF leaks, are marked with a star. Articles that we consider to be ‘must-read’ are marked with a megaphone.

You can search for articles in the database using titles, keywords or author names.

Articles List

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2017



Article Not in Favourites List Article not in Must-Read List  Cerebrospinal fluid outflow resistance as a diagnostic marker of spontaneous cerebrospinal fluid leakage.
Publication Journal of neurosurgery. Spine (Publication year: 2017)
Author(s) Beck Jürgen, Fung Christian, Ulrich Christian T, Fiechter Michael, Fichtner Jens, Mattle Heinrich P, Mono Marie-Luise, Meier Niklaus, Mordasini Pasquale, Z'Graggen Werner J, Gralla Jan, Raabe Andreas
Abstract OBJECTIVE Spinal CSF leakage causes spontaneous intracranial hypotension (SIH). The aim of this study was to characterize CSF dynamics via lumbar infusion testing in patients with and without proven spinal CSF leakage in order to explore possible discriminators for the presence of an open CSF leak. METHODS This analysis included all patients with suspected SIH who were treated at the authors' institution between January 2012 and February 2015. The gold standard for "proven" CSF leakage is considered to be extrathecal contrast accumulation after intrathecal contrast injection. To characterize CSF dynamics, the authors performed computerized lumbar infusion testing to measure lumbar pressure at baseline (opening pressure) and at plateau, as well as pulse amplitude, CSF outflow resistance (RCSF), craniospinal elastance, and pressure-volume index. RESULTS Thirty-one patients underwent clinical imaging and lumbar infusion testing and were included in the final analysis. A comparison of the 14 patients with proven CSF leakage with the 17 patients without leakage showed a statistically significantly lower lumbar opening pressure (p < 0.001), plateau pressure (p < 0.001), and RCSF (p < 0.001) in the group with leakage. Sensitivity, specificity, and positive and negative predictive values for an RCSF cutoff of ≤ 5 mm Hg/(ml/min) were 0.86, 1.0, 1.0, and 0.89 (area under the curve of 0.96), respectively. The median pressure-volume index was higher (p = 0.003), and baseline (p = 0.017) and plateau (p < 0.001) pulse amplitudes were lower in patients with a proven leak. CONCLUSIONS Lumbar infusion testing captures a distinct pattern of CSF dynamics associated with spinal CSF leakage. RCSF assessed by computerized lumbar infusion testing has an excellent diagnostic accuracy and is more accurate than evaluating the lumbar opening pressure. The authors suggest inclusion of RCSF in the diagnostic criteria for SIH.
Article ID(s) 28574328 (PubMed)
10.3171/2017.1.SPINE16548 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Chiari-like displacement due to spontaneous intracranial hypotension in an adolescent: Successful treatment by epidural blood patch.
Publication European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society (Publication year: 2017)
Author(s) Schönberger Jan, Möhlenbruch Markus, Seitz Angelika, Bußmann Cornelia, Bächli Heidi, Kölker Stefan
Abstract BACKGROUND
Spontaneous intracranial hypotension is a rarely diagnosed cause of headache, especially in children and adolescents. It is due to cerebrospinal fluid (CSF) leakage via spinal fistulae occurring without major trauma.

CASE PRESENTATION
An adolescent patient presented with a 3-month history of strictly postural headache. Cranial magnetic resonance imaging (MRI) showed pronounced Chiari-like prolapse of the cerebellar tonsils, narrow ventricles and enlarged cerebral veins. On spinal MRI, myelographic sequences revealed a large collection of CSF around the first sacral roots. CT myelography proved extensive spinal CSF leakage. Hence, we applied epidural patches at multiple levels. Afterwards, symptoms and radiologic findings, including Chiari-like displacement, completely resolved.

CONCLUSION
A Chiari-like descent of the cerebellar tonsils alone does not secure the diagnosis of a Chiari I malformation. Especially if other findings indicate spinal CSF leakage, a systematic work-up should be initiated. In most cases, interventional techniques seal the leak successfully, resulting in a favorable outcome.
Article ID(s) 28283371 (PubMed)
10.1016/j.ejpn.2017.02.004 (DOI)
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Article in Favourites List Article not in Must-Read List  Digital subtraction myelography in the investigation of post-dural puncture headache in 27 patients: technical note.
Publication Journal of neurosurgery. Spine (Publication year: 2017)
Author(s) Schievink Wouter I, Maya M Marcel, Moser Franklin G
Abstract OBJECTIVE Post-dural puncture headaches are common, and the treatment of such headaches can be complex when they become chronic. Among patients with spontaneous spinal CSF leaks, digital subtraction myelography (DSM) can localize the exact site of the leak when an extradural CSF collection is present, and it can also demonstrate CSF-venous fistulas in those without an extradural CSF collection. The authors now report on the use of DSM in the management of patients with chronic post-dural puncture headaches. METHODS The patient population consisted of a consecutive group of 27 patients with recalcitrant post-dural puncture headache that had lasted from 2 to 150 months (mean 26 months). RESULTS The mean age of the 17 women and 10 men was 39.1 years (range 18-77 years). An extensive extradural CSF collection was present in 5 of the 27 patients, and DSM was able to localize the exact site of the dural defect in all 5 patients. Among the 22 patients who did not have an extradural CSF collection, DSM showed a CSF-venous fistula in 1 patient (5%). Three other patients had a small pseudomeningocele at the level of the dural puncture. Percutaneous glue injection or microsurgical repair resulted in resolution of symptoms in 8 of the 9 patients in whom an abnormality had been identified on imaging. CONCLUSIONS Digital subtraction myelography is able to precisely localize the dural puncture site in patients with a post-dural puncture headache and an extensive extradural CSF collection, and it may rarely detect a CSF-venous fistula in such patients without an extradural CSF collection.
Article ID(s) 28362213 (PubMed)
10.3171/2016.11.SPINE16968 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Epidural patch with autologous platelet rich plasma: a novel approach.
Publication Journal of anesthesia (Publication year: 2017)
Author(s) Gunaydin Berrin, Acar Muberra, Emmez Gokcen, Akcali Didem, Tokgoz Nil
Abstract We aimed to perform an epidural patch using platelet rich plasma (PRP), which has the potential to regenerate and heal tissues via degranulation of platelets, in a 34-year-old parturient suffering from persistent post-dural puncture headache (PDPH) after failed epidural blood patch (EBP). After her admission to our unit, we reconfirmed the clinical and radiologic diagnosis of PDPH. Cranial MRI with contrast showed diffuse pachymeningeal thickening and contrast enhancement with enlarged pituitary consistent with intracranial hypotension. Clinical and radiological improvements were observed 1 week after the epidural patch using autologous PRP. Therefore, we recommend using autologous PRP for epidural patching in patients with incomplete recovery after standard EBP as a novel successful approach.
Article ID(s) 28823090 (PubMed)
10.1007/s00540-017-2400-9 (DOI)
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Article in Favourites List Article is Must-Read  Factors affecting cerebrospinal fluid opening pressure in patients with spontaneous intracranial hypotension.
Publication Journal of Zhejiang University. Science. B 2017 Jul; 18(7) (Publication year: 2017)
Author(s) Yao Ling-Ling, Hu Xing-Yue
Abstract OBJECTIVE
Spontaneous intracranial hypotension (SIH) is recognized far more commonly than ever before. Though usually characterized by low cerebrospinal fluid (CSF) pressure, some patients with SIH are observed to have normal pressure values. In this study, we aimed to confirm the proportion of patients with normal CSF opening pressure (CSF OP) and explore the factors affecting CSF OP in SIH patients.

METHODS
We retrospectively reviewed 206 consecutive SIH patients and analyzed their clinical and imaging variables (including demographic data, body mass index (BMI), duration of symptoms, and brain imaging findings). Univariate and multivariate analyses were performed to identify the potential factors affecting CSF OP.

RESULTS
In a total of 114 (55.3%) cases the CSF OP was ≤60 mmH2O (1 mmH2O=9.806 65 Pa), in 90 (43.7%) cases it was between 60 and 200 mmH2O, and in 2 (1.0%) cases it was >200 mmH2O. Univariate analysis showed that the duration of symptoms (P< 0.001), BMI (P< 0.001), and age (P=0.024) were positively correlated with CSF OP. However, multivariate analysis suggested that only the duration of symptoms (P< 0.001) and BMI (P< 0.001) were strongly correlated with CSF OP. A relatively high R(2) of 0.681 was obtained for the multivariate model.

CONCLUSIONS
Our study indicated that in patients without a low CSF OP, a diagnosis of SIH should not be excluded. BMI and the duration of symptoms can influence CSF OP in SIH patients, and other potential factors need further investigation.
Article ID(s) 28681582 (PubMed)
PMC5498838 (PMC)
10.1631/jzus.B1600343 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Factors predicting response to the first epidural blood patch in spontaneous intracranial hypotension.
Publication Brain : a journal of neurology (Publication year: 2017)
Author(s) Wu Jr-Wei, Hseu Shu-Shya, Fuh Jong-Ling, Lirng Jiing-Feng, Wang Yen-Feng, Chen Wei-Ta, Chen Shih-Pin, Wang Shuu-Jiun
Abstract UNASSIGNED
Spontaneous intracranial hypotension results from cerebrospinal fluid leakage. Currently, the treatment of choice for spontaneous intracranial hypotension is the epidural blood patch, which has a variable response rate and no clear outcome predictors. This study aimed to identify predictors for response rate of a first targeted epidural blood patch in patients with spontaneous intracranial hypotension. We reviewed cases of patients with spontaneous intracranial hypotension who received targeted epidural blood patch at our hospital between 1 January 2007 and 1 July 2014. The outcome measure was first epidural blood patch response. We analysed demographics, clinical manifestations, neuroimaging findings (non-contrast heavily T2-weighted magnetic resonance myelography and brain magnetic resonance imaging), and blood volume as potential outcome predictors. Significant predictors were tested and a decision tree was used to construct a predictive model. In total, 150 patients with spontaneous intracranial hypotension were included for final analyses. Their overall first targeted epidural blood patch response rate was 58.7%. Among patients with a greater injected blood volume (≥22.5 versus < 22.5 ml), the response rate was higher (67.9% versus 47.0%, P = 0.01). In brain and spinal magnetic resonance imaging studies, significant predictors included anterior epidural cerebrospinal fluid collection length (< 8 versus ≥8 segments; 72.5% versus 37.3%, odds ratio = 4.4, 95% confidence interval: 2.2-8.9, P < 0.001) and midbrain-pons angle (≥40° versus < 40°; 71.3% versus 37.5%, odds ratio = 4.1, 95% confidence interval 2.1-8.3, P < 0.001). Decision tree analyses showed that patients with anterior epidural CSF collection involving < 8 segments and an injected blood volume ≥22.5 ml had an 80.0% response rate. Patients with anterior epidural cerebrospinal fluid collection involving ≥8 segments and a midbrain-pons angle < 40° had a 21.2% response rate. These three variables predicted first epidural blood patch response in 71.3% of patients. Brain and spinal neuroimaging findings and epidural blood patch blood volume can be used to predict targeted first epidural blood patch response in patients with spontaneous intracranial hypotension.
Article ID(s) 28043956 (PubMed)
10.1093/brain/aww328 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Intracranial hypotension causing pituitary enlargement.
Publication BMJ case reports 2017 May 12; 2017 (Publication year: 2017)
Author(s) Chan Daniela Wc, Wu Angel Iy, Wynne Katie
Abstract No abstract available.
Article ID(s) 28500117 (PubMed)
10.1136/bcr-2017-220057 (DOI)
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Article in Favourites List Article is Must-Read  Needle gauge and tip designs for preventing post-dural puncture headache (PDPH).
Publication The Cochrane database of systematic reviews 2017 Apr 07; 4 (Publication year: 2017)
Author(s) Arevalo-Rodriguez Ingrid, Muñoz Luis, Godoy-Casasbuenas Natalia, Ciapponi Agustín, Arevalo Jimmy J, Boogaard Sabine, Roqué I Figuls Marta
Abstract BACKGROUND
Post-dural puncture headache (PDPH) is one of the most common complications of diagnostic and therapeutic lumbar punctures. PDPH is defined as any headache occurring after a lumbar puncture that worsens within 15 minutes of sitting or standing and is relieved within 15 minutes of the patient lying down. Researchers have suggested many types of interventions to help prevent PDPH. It has been suggested that aspects such as needle tip and gauge can be modified to decrease the incidence of PDPH.

OBJECTIVES
To assess the effects of needle tip design (traumatic versus atraumatic) and diameter (gauge) on the prevention of PDPH in participants who have undergone dural puncture for diagnostic or therapeutic causes.

SEARCH METHODS
We searched CENTRAL, MEDLINE, Embase, CINAHL and LILACS, as well as trial registries via the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP) search portal in September 2016. We adopted the MEDLINE strategy for searching the other databases. The search terms we used were a combination of thesaurus-based and free-text terms for both interventions (lumbar puncture in neurological, anaesthesia or myelography settings) and headache.

SELECTION CRITERIA
We included randomized controlled trials (RCTs) conducted in any clinical/research setting where dural puncture had been used in participants of all ages and both genders, which compared different tip designs or diameters for prevention of PDPH DATA COLLECTION AND ANALYSIS: We used the standard methodological procedures expected by Cochrane.

MAIN RESULTS
We included 70 studies in the review; 66 studies with 17,067 participants were included in the quantitative analysis. An additional 18 studies are awaiting classification and 12 are ongoing. Fifteen of the 18 studies awaiting classification mainly correspond to congress summaries published before 2010, in which the available information does not allow the complete evaluation of all their risks of bias and characteristics. Our main outcome was prevention of PDPH, but we also assessed the onset of severe PDPH, headache in general and adverse events. The quality of evidence was moderate for most of the outcomes mainly due to risk of bias issues. For the analysis, we undertook three main comparisons: 1) traumatic needles versus atraumatic needles; 2) larger gauge traumatic needles versus smaller gauge traumatic needles; and 3) larger gauge atraumatic needles versus smaller gauge atraumatic needles. For each main comparison, if data were available, we performed a subgroup analysis evaluating lumbar puncture indication, age and posture.For the first comparison, the use of traumatic needles showed a higher risk of onset of PDPH compared to atraumatic needles (36 studies, 9378 participants, risk ratio (RR) 2.14, 95% confidence interval (CI) 1.72 to 2.67, I(2) = 9%).In the second comparison of traumatic needles, studies comparing various sizes of large and small gauges showed no significant difference in effects in terms of risk of PDPH, with the exception of one study comparing 26 and 27 gauge needles (one study, 658 participants, RR 6.47, 95% CI 2.55 to 16.43).In the third comparison of atraumatic needles, studies comparing various sizes of large and small gauges showed no significant difference in effects in terms of risk of PDPH.We observed no significant difference in the risk of paraesthesia, backache, severe PDPH and any headache between traumatic and atraumatic needles. Sensitivity analyses of PDPH results between traumatic and atraumatic needles omitting high risk of bias studies showed similar results regarding the benefit of atraumatic needles in the prevention of PDPH (three studies, RR 2.78, 95% CI 1.26 to 6.15; I(2) = 51%).

AUTHORS' CONCLUSIONS
There is moderate-quality evidence that atraumatic needles reduce the risk of post-dural puncture headache (PDPH) without increasing adverse events such as paraesthesia or backache. The studies did not report very clearly on aspects related to randomization, such as random sequence generation and allocation concealment, making it difficult to interpret the risk of bias in the included studies. The moderate quality of the evidence for traumatic versus atraumatic needles suggests that further research is likely to have an important impact on our confidence in the estimate of effect.
Article ID(s) 28388808 (PubMed)
10.1002/14651858.CD010807.pub2 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Reversible Holmes tremor due to spontaneous intracranial hypotension.
Publication BMJ case reports 2017 Jul 27; 2017 (Publication year: 2017)
Author(s) Iyer Rajesh Shankar, Wattamwar Pandurang, Thomas Bejoy
Abstract Holmes\' tremor is a low-frequency hand tremor and has varying amplitude at different phases of motion. It is usually unilateral and does not respond satisfactorily to drugs and thus considered irreversible. Structural lesions in the thalamus and brainstem or cerebellum are usually responsible for Holmes\' tremor. We present a 23-year-old woman who presented with unilateral Holmes\' tremor. She also had hypersomnolence and headache in the sitting posture. Her brain imaging showed brain sagging and deep brain swelling due to spontaneous intracranial hypotension (SIH). She was managed conservatively and had a total clinical and radiological recovery. The brain sagging with the consequent distortion of the midbrain and diencephalon was responsible for this clinical presentation. SIH may be considered as one of the reversible causes of Holmes\' tremor.
Article ID(s) 28754752 (PubMed)
10.1136/bcr-2017-220348 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous intracranial hypotension diagnosed as Chiari I malformation.
Publication Neurology 2017 Mar 28; 88(13) (Publication year: 2017)
Author(s) Kingston William, Hoxworth Joseph, Halker-Singh Rashmi
Abstract No abstract available.
Article ID(s) 28348118 (PubMed)
10.1212/WNL.0000000000003775 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous Intracranial Hypotension Presenting as a "Pseudo-Chiari 1.
Publication Cureus 2017 Feb 16; 9(2) (Publication year: 2017)
Author(s) Haider Ali S, Sulhan Suraj, Watson Ian T, Leonard Dean, Arrey Eliel N, Khan Umair, Nguyen Phu, Layton Kennith F
Abstract Spontaneous intracranial hypotension (SIH) is classified as a decrease in cerebrospinal fluid (CSF) pressure secondary to a CSF leakage and consequent descent of the brain into the foramen magnum. Diagnosing SIH can be difficult due to its overlapping findings with Arnold-Chiari type 1 Malformation (CM1) where the cerebellar tonsils herniate into the foramen magnum. The similarity of both conditions calls for a more reliable imaging technique to localize the CSF leak which could narrow the differential diagnosis and aid in choosing the correct treatment. Here, we present a case of a 28-year-old female, ten weeks post-partum with symptoms similar to SIH. MRI of the brain was remarkable for tonsillar herniation below the foramen magnum. Literature was reviewed for additional neuroradiology techniques that would aid in narrowing our differential diagnosis. Interestingly, computed tomography-, digital subtraction-, and magnetic resonance myelography with intrathecal gadolinium are the preferred techniques for diagnosis of high flow and low flow CSF leaks, respectively. These modalities further aid in choosing the correct treatment while avoiding complications. Literature suggests that treatment for CM1 involves posterior fossa decompression, whereas the mainstay of treatment for SIH involves an epidural blood patch (EBP). Thus, our patient was treated with an EBP and recovered without complication.
Article ID(s) 28357166 (PubMed)
PMC5354398 (PMC)
10.7759/cureus.1034 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Surgical management of spontaneous spinal cerebrospinal fluid epidural fistula.
Publication World neurosurgery (Publication year: 2017)
Author(s) Siedler Declan G, Ibbett Imogen M, Thani Nova B
Abstract BACKGROUND
Intracranial hypotension secondary to spontaneous spinal cerebrospinal fluid (CSF) fistula is a rare condition which can have serious sequelae. Early diagnosis and treatment can be challenging.

CASE DESCRIPTION
We present a case of a 17-year-old man who presented with a history of sudden onset, postural headaches, associated with upper thoracic back pain. Magnetic Resonance Imaging (MRI) demonstrated a thoracic extradural fluid collection and slumping of the brain within the posterior fossa. The patient was initially managed with a period of bed rest, followed by a thoracic epidural blood patch. Symptoms recurred and subsequent operative exploration found a large arachnoid cyst with CSF egress through a linear split in the axilla of the right T7 nerve root. The arachnoid cyst was resected and the defect was closed primarily. All symptoms completely resolved. MRI at three months post-operatively demonstrated normal spinal configuration and resolution of brain sagging.

CONCLUSIONS
Spontaneous CSF leaks are a rare cause of postural headache. Although epidural blood patching is an easy and safe intervention, early serial imaging to ascertain the evolution of the pathology may identify cases that are amenable to early surgical management.
Article ID(s) 28057591 (PubMed)
10.1016/j.wneu.2016.12.106 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Targeted epidural blood patch under O-arm-guided stereotactic navigation in patients with intracranial hypotension associated with a spinal CSF leak and ventral dural defect.
Publication World neurosurgery (Publication year: 2017)
Author(s) Takai Keisuke, Taniguchi Makoto
Abstract OBJECTIVE
Targeted epidural blood patch (EBP) at the site of a presumed cerebrospinal fluid leak reportedly has better outcomes than non-targeted EBP; however, it is associated with a higher risk of wrong-site injection such as iatrogenic subarachnoid or intramuscular injections, which lead to reintervention due to the insufficient coverage of injected blood.

METHODS
Eight patients with intracranial hypotension due to a CSF leak diagnosed by myelographic CT and thin-cut MRI received an epidural blood patch under O-arm-guided stereotactic navigation.

RESULTS
The leak site was identified based on myelographic CT findings of a micro-spur and epidural contrast medium extravasations as well as MRI findings of a ventral dural defect. During the EBP procedure, no iatrogenic dural puncture or subarachnoid injection occurred because O-arm-guided stereotactic navigation provided real-time feedback on the needle trajectory. O-arm CT revealed the sufficient coverage of injected blood following the first injection in six out of eight patients. In the two remaining patients, a second injection was performed during the same session due to insufficient coverage at the previous site. In all patients, complete recovery from orthostatic headaches was achieved after a single session.

CONCLUSIONS
O-arm-guided navigation facilitated EBP by enabling real-time observations of the needle trajectory and distribution of injected blood while simultaneously avoiding major complications such as wrong-site injections or reintervention.
Article ID(s) 28790007 (PubMed)
10.1016/j.wneu.2017.07.168 (DOI)
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Article in Favourites List Article not in Must-Read List  The status of diagnosis and treatment to intracranial hypotension, including SIH
Publication The Journal of Headache and Pain 708 2017 (Publication year: 2017)
Author(s) Lin Jin-ping, Zhang Shu-dong, He Fei-fang, Liu Min-jun, Ma Xiao-xu
Abstract No abstract available.
Article ID(s) 28091819 (PubMed)
PMC5236046 (PMC)
10.1186/s10194-016-0708-8 (DOI)
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Article in Favourites List Article is Must-Read  Update on the Diagnosis and Treatment of Spontaneous Intracranial Hypotension.
Publication Current pain and headache reports 2017 Aug; 21(8) (Publication year: 2017)
Author(s) Kranz Peter G, Malinzak Michael D, Amrhein Timothy J, Gray Linda
Abstract PURPOSE OF REVIEW
The purpose of this study is to provide an update on recent developments in the understanding, diagnosis, and treatment of spontaneous intracranial hypotension (SIH).

RECENT FINDINGS
SIH is an important cause of headaches caused by spinal cerebrospinal fluid (CSF) leaks, with an increasingly broad spectrum of clinical presentations and diagnostic findings. A simple conception of the condition as being defined by the presence of low CSF pressure is no longer sufficient or accurate. A number of etiologies for spinal CSF leaks have been identified, including the recent discovery of CSF-venous fistulas, and these various etiologies may require different diagnostic and therapeutic pathways in order to affect a cure. Familiarity with the spectrum of presentations and causes of SIH is critical to accurate and timely diagnosis and management. Challenges exist in both diagnosis and treatment, and require understanding of the underlying pathogenesis of the condition in order to appropriately select testing and treatment. Prospective studies are needed going forward in order to inform workup and guide treatment decisions.
Article ID(s) 28755201 (PubMed)
10.1007/s11916-017-0639-3 (DOI)
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2016



Article in Favourites List Article is Must-Read  A classification system of spontaneous spinal CSF leaks.
Publication Neurology 2016 Aug 16; 87(7) (Publication year: 2016)
Author(s) Schievink Wouter I, Maya M Marcel, Jean-Pierre Stacey, Nuño Miriam, Prasad Ravi S, Moser Franklin G
Abstract OBJECTIVE
Spontaneous spinal CSF leaks cause spontaneous intracranial hypotension but no systematic study of the different types of these CSF leaks has been reported. Based on our experience with spontaneous intracranial hypotension, we propose a classification system of spontaneous spinal CSF leaks.

METHODS
We reviewed the medical records, radiographic studies, operative notes, and any intraoperative photographs of a group of consecutive patients with spontaneous intracranial hypotension.

RESULTS
The mean age of the 568 patients (373 [65.7%] women) was 45.7 years. Three types of CSF leak could be identified. Type 1 CSF leaks consisted of a dural tear (151 patients [26.6%]) and these were almost exclusively associated with an extradural CSF collection. Type 1a represented ventral CSF leaks (96%) and type 1b posterolateral CSF leaks (4%). Type 2 CSF leaks consisted of meningeal diverticula (240 patients [42.3%]) and were the source of an extradural CSF collection in 53 of these patients (22.1%). Type 2a represented simple diverticula (90.8%) and type 2b complex meningeal diverticula/dural ectasia (9.2%). Type 3 CSF leaks consisted of direct CSF-venous fistulas (14 patients [2.5%]) and these were not associated with extradural CSF collections. A total of 163 patients (28.7%) had an indeterminate type and extradural CSF collections were noted in 84 (51.5%) of these patients.

CONCLUSIONS
We identified 3 types of spontaneous spinal CSF leak in this observational study: the dural tear, the meningeal diverticulum, and the CSF-venous fistula. These 3 types and the presence or absence of extradural CSF form the basis of a comprehensive classification system.
Article ID(s) 27440149 (PubMed)
10.1212/WNL.0000000000002986 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Accidental Dural Puncture Management: 10-Year Experience at an Academic Tertiary Care Center.
Publication Regional anesthesia and pain medicine 2016 Mar-Apr; 41(2) (Publication year: 2016)
Author(s) Bolden Norman, Gebre Ermias
Abstract BACKGROUND AND OBJECTIVES
The use of spinal catheters for extended periods after accidental dural puncture (ADP) and administration of intrathecal saline via spinal catheters have been advocated to decrease the incidence of postdural puncture headache and the subsequent need for epidural blood patch (EBP), with mixed results observed.

METHODS
We reviewed the medical records of 218 patients with ADP who either had the epidural resited or had a spinal catheter (with or without the administration of intrathecal saline). We compared the incidence of headache and the need for blood patch between these groups. We also assessed complications when a standard lidocaine epidural test dose was administered intrathecally and compared this with complications when a solution normally used for labor combined spinal epidurals was administered.

RESULTS
There was no difference in the incidence of postdural puncture headache between the resited epidural group and the spinal catheter group, 68.0% versus 55.9% (odds ratio [OR], 1.7; 95% confidence interval [95% CI], 1.0-2.9; P = 0.07). Resiting the epidural catheter was associated with a significant increase in the number of EBPs when compared with using a spinal catheter, 52.0% versus 20.3% (OR, 4.2; 95% CI, 2.4-7.6; P < 0.001) and when compared with spinal catheters with intrathecal saline, 52.0% versus 8.1% (OR, 12.3; 95% CI, 4.3-35.4; P < 0.001). There was a significant difference in the number of blood patches between normal body mass index patients and morbidly obese patients, 55.2% versus 25.0% (OR, 3.7; 95% CI, 1.2-11.2; P = 0.02). Complications (hypotension prompting pressors, high spinal, and emergency cesarean delivery because of nonreassuring fetal status) occurred more frequently when a lidocaine test dose was immediately administered after ADP versus administering a labor combined spinal epidural solution.

CONCLUSIONS
Insertion of spinal catheters after ADP and administration of intrathecal normal saline via spinal catheters reduce the need for EBP compared with resiting the epidural. Administration of the standard epidural test dose intrathecally is associated with frequent and significant complications.
Article ID(s) 26735153 (PubMed)
10.1097/AAP.0000000000000339 (DOI)
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Article in Favourites List Article not in Must-Read List  An Unusual Case of Post-Traumatic Headache Complicated by Intracranial Hypotension.
Publication Brain sciences 2016 Dec 29; 7(1) (Publication year: 2016)
Author(s) Siavoshi Sara, Dougherty Carrie, Ailani Jessica, Yadwadkar Kaustubh, Berkowitz Frank
Abstract UNASSIGNED
We present a case of post-traumatic headache complicated by intracranial hypotension resulting in an acquired Chiari malformation and myelopathy with syringomyelia. This constellation of findings suggest a possible series of events that started with a traumatic cerebral spinal fluid (CSF) leak, followed by descent of the cerebellar tonsils and disruption of CSF circulation that caused spinal cord swelling and syrinx. This unusual presentation of post-traumatic headache highlights the varying presentations and the potential sequelae of intracranial hypotension. In addition, the delayed onset of upper motor neuron symptoms along with initially normal head computerized tomography scan (CT) findings, beg the question of whether or not a post-traumatic headache warrants earlier magnetic resonance imaging (MRI).
Article ID(s) 28036062 (PubMed)
10.3390/brainsci7010003 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Atypical Presentations of Intracranial Hypotension: Comparison with Classic Spontaneous Intracranial Hypotension.
Publication AJNR. American journal of neuroradiology 2016 Jul; 37(7) (Publication year: 2016)
Author(s) Capizzano A A, Lai L, Kim J, Rizzo M, Gray L, Smoot M K, Moritani T
Abstract BACKGROUND AND PURPOSE
Atypical clinical presentations of spontaneous intracranial hypotension include obtundation, memory deficits, dementia with frontotemporal features, parkinsonism, and ataxia. The purpose of this study was to compare clinical and imaging features of spontaneous intracranial hypotension with typical-versus-atypical presentations.

MATERIALS AND METHODS
Clinical records and neuroimaging of patients with spontaneous intracranial hypotension from September 2005 to August 2014 were retrospectively evaluated. Patients with classic spontaneous intracranial hypotension (n = 33; mean age, 41.7 ± 14.3 years) were compared with those with intracranial hypotension with atypical clinical presentation (n = 8; mean age, 55.9 ± 14.1 years) and 36 controls (mean age, 41.4 ± 11.2 years).

RESULTS
Patients with atypical spontaneous intracranial hypotension were older than those with classic spontaneous intracranial hypotension (55.9 ± 14.1 years versus 41.7 ± 14.3 years; P = .018). Symptom duration was shorter in classic compared with atypical spontaneous intracranial hypotension (3.78 ± 7.18 months versus 21.93 ± 18.43 months; P = .015). There was no significant difference in dural enhancement, subdural hematomas, or cerebellar tonsil herniation. Patients with atypical spontaneous intracranial hypotension had significantly more elongated anteroposterior midbrain diameter compared with those with classic spontaneous intracranial hypotension (33.6 ± 2.9 mm versus 27.3 ± 2.9 mm; P < .001) and shortened pontomammillary distance (2.8 ± 1 mm versus 5.15 ± 1.5 mm; P < .001). Patients with atypical spontaneous intracranial hypotension were less likely to become symptom-free, regardless of treatment, compared with those with classic spontaneous intracranial hypotension (χ(2) = 13.99, P < .001).

CONCLUSIONS
In this sample of 8 patients, atypical spontaneous intracranial hypotension was a more chronic syndrome compared with classic spontaneous intracranial hypotension, with more severe brain sagging, lower rates of clinical response, and frequent relapses. Awareness of atypical presentations of spontaneous intracranial hypotension is paramount.
Article ID(s) 26939631 (PubMed)
10.3174/ajnr.A4706 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Chronic Subdural Hematoma Associated with Spontaneous Intracranial Hypotension: Therapeutic Strategies and Outcomes of 55 Cases.
Publication Neurologia medico-chirurgica 2016; 56(2) (Publication year: 2016)
Author(s) Takahashi Koichi, Mima Tatsuo, Akiba Yoichi
Abstract Spontaneous intracranial hypotension (SIH) has increasingly been recognized, and it is well known that SIH is sometimes complicated by chronic subdural hematoma (SDH). In this study, 55 cases of SIH with SDH were retrospectively analyzed, focusing on therapeutic strategies and outcomes. Of 169 SIH cases (75 males, 84 females), 55 (36 males, 19 females) were complicated by SDH. SIH was diagnosed based on clinical symptoms, neuroimaging, and/or low cerebrospinal fluid pressure. Presence of orthostatic headache and diffuse meningeal enhancement on magnetic resonance imaging were regarded as the most important criteria. Among 55 SIH with SDH cases, 13 improved with conservative treatment, 25 initially received an epidural blood patch (EBP), and 17 initially underwent irrigation of the hematomas. Of the 25 initially treated with EBP, 7 (28.0%) needed SDH surgery and 18 (72.0%) recovered fully without surgery. Of 17 SDH cases initially treated with surgery, 6 (35.7%) required no EBP therapy and the other 11 (64.3%) needed EBP and/or additional SDH operations. In the latter group, 2 cases had transient severe complications during and after the procedures. One of these 2 cases developed a hoarse voice complication. Despite this single, non-severe complication, all enrolled in this study achieved good outcomes. The present study suggests that patients initially receiving SDH surgery may need additional treatments and may occasionally have complications. If conservative treatment is insufficient, EBP should be performed prior to hematoma irrigation.
Article ID(s) 26489406 (PubMed)
10.2176/nmc.oa.2015-0032 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Clinical characteristics of 15 cases of chronic subdural hematomas due to spontaneous intracranial hypotension with spinal cerebrospinal fluid leak.
Publication Acta neurologica Belgica 2016 Dec; 116(4) (Publication year: 2016)
Author(s) Wan Yingfeng, Xie Jixi, Xie Dajiang, Xue Zhaoliang, Wang Yirong, Yang Shuxu
Abstract UNASSIGNED
The etiology of chronic subdural hematoma (CSDH) in patients is diverse. The primary objective of this article was to discuss one of the causes, spontaneous intracranial hypotension with spinal cerebrospinal fluid (CSF) leak, which is usually neglected by the neurosurgeon. All the consecutive 15 patients who underwent operation for CSDHs between June 2012 and June 2014 at Sir Run Run Shaw Hospital of Zhejiang University were included in this retrospective cohort study. The clinical and imaging data of these patients with CSDHs due to spinal CSF leak were retrospectively studied. Fifteen patients, with a mean age of 53.8 ± 8.3 years, underwent operations for CSDH. Hematomas were unilateral in 4 patients and bilateral in 11 patients. Among these patients, eight patients had recurrence of hematomas after operation due to neglect of spinal CSF leak. All patients had fully recovery. Spinal CSF leak is a cause of cSDH, which is overlooked by the doctor.
Article ID(s) 26769700 (PubMed)
10.1007/s13760-016-0597-2 (DOI)
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Article in Favourites List Article is Must-Read  CT Fluoroscopy-Guided Blood Patching of Ventral CSF Leaks by Direct Needle Placement in the Ventral Epidural Space Using a Transforaminal Approach.
Publication AJNR. American journal of neuroradiology (Publication year: 2016)
Author(s) Amrhein T J, Befera N T, Gray L, Kranz P G
Abstract BACKGROUND AND PURPOSE
Epidural blood patch treatment of spontaneous intracranial hypotension arising from ventral CSF leaks can be difficult secondary to challenges in achieving ventral spread of patching material. The purpose of this study was to determine the technical success rates and safety profile of direct needle placement into the ventral epidural space via a posterior transforaminal approach.

MATERIALS AND METHODS
We retrospectively reviewed consecutive CT fluoroscopy-guided epidural blood patches from June 2013 through July 2015. Cases were included if a posterior transforaminal approach was taken to place the needle directly in the ventral epidural space. Rates of technical success (defined as contrast in the spinal canal ventral epidural space) and optimal epidurogram (defined as contrast spreading into or beyond the middle third of the spinal canal ventral epidural space) were determined. Factors influencing these rates were assessed. All complications, inadvertent intravascular injections, and intrathecal punctures were recorded.

RESULTS
A total of 72 ventral epidural blood patches were identified; immediate technical success was achieved in 95.8% and an optimal epidurogram in 47.2%. Needle position within the spinal canal ventral epidural space was associated with obtaining an optimal epidurogram (P = .005). Inadvertent intravascular injection was identified in 29.3% of cases, but all were venous. There were no inadvertent intrathecal punctures or complications.

CONCLUSIONS
Direct needle placement in the ventral epidural space via a transforaminal approach for treatment of ventral CSF leaks has an excellent technical success rate and safety profile. This technique can be considered as a treatment option in selected patients with ventral CSF leaks for whom traditional techniques are unsuccessful.
Article ID(s) 27390315 (PubMed)
10.3174/ajnr.A4842 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Diagnosis and treatment of spontaneous intracranial hypotension due to cerebrospinal fluid leakage
Publication Springer Plus (Publication year: 2016)
Author(s) Yake Zheng, Yajun, Chuanjie Wu, Chen Chen, Haifeng Zhang, Peng Zhao
Abstract Introduction
Spontaneous intracranial hypotension is one of the causes of a postural headache in young people. In this study, the diagnosis and treatment results of a case of intracranial hypotension headache due to spinal cerebrospinal fluid leakage were reported. Up to now, there is not absolutely effective treatment for intracranial hypotension headache.

Case description
A 32-year-old woman complained, a headache after prolonged sitting that presented with nausea; vomiting; increased pain during walking; and decreased or absent pain after lying down. The dramatic improvement of this cephalalgia with epidural blood patch treatment confirmed the diagnosis.

Discussion and Evaluation
To the best of our knowledge, this is the first reported of radiographic contrast before and after epidural blood patch. Improved clinical diagnosis and treatment of spontaneous intracranial hypotension. The patient didn\'t feel any discomfort, no complications such as infection etc. were observed. A small dose of intrathecal gadolthis is the first reported case ofinium during CEMRM allows for improved detection of CSF leakage.

Conclusions
Leakage of spinal CSF is a major cause of spontaneous intracranial hypotension. In order to improve clinical diagnosis and provide effective treatment, the precise etiology of spontaneous intracranial hypotension should be investigated in each patient.
Article ID(s) 10.1186/s40064-016-3775-z (DOI)
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Article Not in Favourites List Article not in Must-Read List  Diffuse non-aneurysmal SAH in spontaneous intracranial hypotension: Sequela of ventral CSF leak?
Publication Cephalalgia : an international journal of headache 2016 May; 36(6) (Publication year: 2016)
Author(s) Schievink Wouter I, Maya M Marcel
Abstract BACKGROUND
Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid (CSF) leak has become a well-recognized cause of headaches. Recently, various unusual neurological syndromes have been described in such patients with chronic ventral CSF leaks, including superficial siderosis and an amyotrophic lateral sclerosis-like syndrome. The authors now report two patients with spontaneous intracranial hypotension due to a chronic ventral CSF leak who suffered a diffuse non-aneurysmal subarachnoid hemorrhage (SAH).

DESCRIPTION OF CASES
A 62-year-old woman underwent uneventful microsurgical repair of a ventral thoracic CSF leak that had been present for 13 years. Seventeen months after surgery, she was found unresponsive and CT showed a diffuse intracranial SAH. Cerebral angiography and spine and brain MRI did not reveal a source of the SAH. A 73-year-old woman was found unresponsive and CT showed a diffuse intracranial SAH. Cerebral angiography and brain MRI did not reveal a source of the SAH, although superficial siderosis was detected. Spine MRI showed a ventral thoracic CSF leak that by history had been present for 41 years. She underwent uneventful microsurgical repair of the CSF leak.

DISCUSSION
The authors suggest that patients with a ventral spinal CSF leak of long duration may be at risk of diffuse non-aneurysmal SAH.
Article ID(s) 26346560 (PubMed)
10.1177/0333102415604473 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Digital Subtraction Cystography for Detection of Communicating Holes of Spinal Extradural Arachnoid Cysts
Publication Korean Journal of Radiology 10.3348/kjr.2016.17.1.111 2016 (Publication year: 2016)
Author(s) Gu Kyowon, Kwon Jong Won, Kim Eun-Sang
Abstract Objective
The purpose of this study was to demonstrate the usefulness of digital subtraction cystography to identify communicating holes between a spinal extradural arachnoid cyst (SEAC) and the subarachnoid space prior to cyst removal and hole closure.

Materials and Methods
Six patients with SEAC were enrolled in this retrospective study. Digital subtraction cystography and subsequent CT myelography were performed for every patient. The presence and location of the communicating holes on cystography were documented. We evaluated the MRI characteristics of the cysts, including location, size, and associated spinal cord compression; furthermore, we reviewed cystographic images, CT myelograms, procedural reports, and medical records for analysis. If surgery was performed after cystography, intraoperative findings were compared with preoperative cystography.

Results
The location of the communicating hole between the arachnoid cyst and the subarachnoid space was identified by digital subtraction cystography in all cases (n = 6). Surgical resection of SEAC was performed in 4 patients, and intraoperative location of the communicating hole exactly corresponded to the preoperative identification.

Conclusion
Fluoroscopic-guided cystography for SEAC accurately demonstrates the presence and location of dural defects. Preoperative digital subtraction cystography is useful for detection of a communicating hole between a cyst and the subarachnoid space.
Article ID(s) PMC4720798 (PMC)
10.3348/kjr.2016.17.1.111 (DOI)
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Article in Favourites List Article is Must-Read  Digital subtraction myelography for the identification of spontaneous spinal CSF-venous fistulas.
Publication Journal of neurosurgery. Spine 2016 Jun; 24(6) (Publication year: 2016)
Author(s) Schievink Wouter I, Moser Franklin G, Maya M Marcel, Prasad Ravi S
Abstract OBJECTIVE In most patients with spontaneous intracranial hypotension, a spinal CSF leak can be found, but occasionally, no leak can be demonstrated despite extensive spinal imaging. Failure to localize a CSF leak limits treatment options. The authors recently reported the discovery of CSF-venous fistulas in patients with spontaneous intracranial hypotension and now report on the use of digital subtraction myelography in patients with spontaneous intracranial hypotension but no CSF leak identifiable on conventional spinal imaging (i.e., non-digital subtraction myelography). METHODS The patient population consisted of 53 consecutive patients with spontaneous intracranial hypotension who underwent digital subtraction myelography but in whom no spinal CSF leak (i.e., presence of extradural CSF) was identifiable on conventional spinal imaging. RESULTS The mean age of the 33 women and 20 men was 53.4 years (range 29-71 years). A CSF-venous fistula was demonstrated in 10 (19%) of the 53 patients. A CSF-venous fistula was found in 9 (27%) of the 33 women and in 1 (5%) of the 20 men (p = 0.0697). One patient was treated successfully with percutaneous injection of fibrin sealant. Nine patients underwent surgery for the fistula. Surgery resulted in complete resolution of symptoms in 8 patients (follow-up 7-25 months), and in 1 patient, symptoms recurred after 4 months. CONCLUSIONS In this study, the authors found a CSF-venous fistula in approximately one-fifth of the patients with recalcitrant spontaneous intracranial hypotension but no CSF leak identifiable on conventional spinal imaging. The authors suggest that digital subtraction myelography be considered in this patient population.
Article ID(s) 26849709 (PubMed)
10.3171/2015.10.SPINE15855 (DOI)
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Article in Favourites List Article is Must-Read  Diskogenic microspurs as a major cause of intractable spontaneous intracranial hypotension.
Publication Neurology 2016 Sep 20; 87(12) (Publication year: 2016)
Author(s) Beck Jürgen, Ulrich Christian T, Fung Christian, Fichtner Jens, Seidel Kathleen, Fiechter Michael, Hsieh Kety, Murek Michael, Bervini David, Meier Niklaus, Mono Marie-Luise, Mordasini Pasquale, Hewer Ekkehard, Z'Graggen Werner J, Gralla Jan, Raabe Andreas
Abstract OBJECTIVE
To visualize and treat spinal dural CSF leaks in all patients with intractable spontaneous intracranial hypotension (SIH) who underwent spinal microsurgical exploration.

METHODS
Patients presenting between February 2013 and July 2015 were included in this consecutive case series. The workup included spinal MRI without and with intrathecal contrast, dynamic myelography, postmyelography CT, and microsurgical exploration.

RESULTS
Of 69 consecutive patients, 15 had intractable symptoms. Systematic imaging revealed a suspicious single location of the leak in these 15 patients. Fourteen patients underwent microsurgical exploration; 1 patient refused surgery. Intraoperatively, including intradural exploration, we identified the cause of the CSF leaks as a longitudinal dural slit (6.1 ± 1.7 mm) on the ventral (10), lateral (3), or dorsal (1) aspect of the dura. In 10 patients (71%), a ventral, calcified microspur originating from the intervertebral disk perforated the dura like a knife. Three patients (22%) had a lateral dural tear with an associated spinal meningeal diverticulum, and in 1 patient (7%), a dorsal osteophyte was causal. The microspurs were removed and the dural slits sutured with immediate cessation of CSF leakage.

CONCLUSION
The nature of the CSF leak is a circumscribed longitudinal slit at the ventral, lateral, or dorsal dura mater. An extradural pathology, diskogenic microspurs, was the single cause for all ventral CSF leaks. These findings challenge the notion that CSF leaks in SIH are idiopathic or due to a weak dura. Microsurgery is the treatment of choice in cases with intractable SIH.
Article ID(s) 27566748 (PubMed)
10.1212/WNL.0000000000003122 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Dural Reduction Surgery: A Treatment Option for Frontotemporal Brain Sagging Syndrome.
Publication The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques 2016 Jul; 43(4) (Publication year: 2016)
Author(s) Mostofi Emily, Schievink Wouter I, Sim Valerie L
Abstract Frontotemporal brain sagging syndrome is a dementia associated with hypersomnolence, personality changes, and features of intracranial hypotension on magnetic resonance imaging. The literature is sparse with respect to treatment options; many patients simply worsen. We present a case in which this syndrome responded to lumbar dural reduction surgery. Postoperative magnetic resonance imaging indicated normalization of brain sagging and lumbar intrathecal pressure. Although no evidence of cerebrospinal leak was found, extremely thin dura was noted intraoperatively, suggesting that a thin and incompetent dura could result in this low-pressure syndrome. Clinicians who encounter this syndrome should consider dural reduction surgery as a treatment strategy.
Article ID(s) 26972054 (PubMed)
10.1017/cjn.2016.3 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Efficacy of epidural blood patch with fibrin glue additive in refractory headache due to intracranial hypotension: preliminary report.
Publication SpringerPlus 2016; 5 (Publication year: 2016)
Author(s) Elwood Justin J, Dewan Misha, Smith Jolene M, Mokri Bahram, Mauck William D, Eldrige Jason S
Abstract BACKGROUND
Injection of fibrin glue mixed with blood into the epidural space to reliably and effectively treat medically refractory orthostatic headache caused by spinal cerebrospinal fluid (CSF) leaks and subsequent intracranial hypotension has recently been described. The study described in this article utilizes an analogous technique to gauge the therapeutic reproducibility of this novel technique.

METHODS
Eight patients with medically refractory headache resulting from intracranial hypotension caused by spinal CSF leaks received epidural injections of combined fibrin glue, autologous blood, and Isovue contrast at the L1-2 vertebral level using intermittent fluoroscopic guidance. Pre-procedure, 1-week post-procedure, and 3-month post-procedure headache pain scores were collected and used for comparison.

RESULTS
Three out of 8 patients reported relief at 1 week, although 1 of these 3 patients had returned to their baseline pain intensity at 3 months. Four patients reported no change at 1 week, though 2 of these patients had reduction of their chronic headache pain at 3 months. A single patient reported increased pain 1 week after the procedure, which persisted at 12 weeks. Overall, 4 out of the 8 patients had decreased pain scores at 3-month follow-up.

CONCLUSIONS
We did not achieve a similar frequency of headache resolution as reported in prior original studies. However, a subset of patients did appear to receive substantial benefit from the combined fibrin glue-blood patching procedure. This technique may prove to be useful in medically refractory cases, including those patients who continue to have symptoms despite the prior administration of conventional epidural blood patches.
Article ID(s) 27066348 (PubMed)
10.1186/s40064-016-1975-1 (DOI)
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Article in Favourites List Article not in Must-Read List  Epidural analgesia complicated by dural ectasia in the Marfan syndrome.
Publication Proceedings (Baylor University. Medical Center) 2016 Oct; 29(4) (Publication year: 2016)
Author(s) Vacula Benjamin B, Gray Chelsea, Hofkamp Michael P, Noonan Patrick T, McAllister Russell K, Pilkinton Kimberly A, Diao Zhiying
Abstract UNASSIGNED
Patients with the Marfan syndrome are considered to be high risk during pregnancy and warrant a complete multidisciplinary evaluation. One goal is to minimize hemodynamic fluctuations during labor since hypertensive episodes may result in aortic dissection or rupture. Although they may prevent these complications, neuraxial techniques may be complicated by dural ectasia. The case of a parturient with the Marfan syndrome and mild dural ectasia is presented. During attempted labor epidural placement, unintentional dural puncture occurred. A spinal catheter was used for adequate labor analgesia, and a resultant postdural puncture headache was alleviated by an epidural blood patch under fluoroscopic guidance.
Article ID(s) 27695168 (PubMed)
PMC5023290 (PMC)
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Article Not in Favourites List Article not in Must-Read List  Epidural Anesthesia Complicated by Subdural Hygromas and a Subdural Hematoma.
Publication Case reports in anesthesiology 2016; 2016 (Publication year: 2016)
Author(s) Vien Christine, Marovic Paul, Ingram Brendan
Abstract Inadvertent dural puncture during epidural anesthesia leads to intracranial hypotension, which if left unnoticed can cause life-threatening subdural hematomas or cerebellar tonsillar herniation. The highly variable presentation of intracranial hypotension hinders timely diagnosis and treatment. We present the case of a young laboring adult female, who developed subdural hygromas and a subdural hematoma following unintentional dural puncture during initiation of epidural anesthesia.
Article ID(s) 27651956 (PubMed)
10.1155/2016/5789504 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Epidural Blood Patch for the Treatment of Spontaneous and Iatrogenic Orthostatic Headache.
Publication Pain physician 2016 Nov-Dec; 19(8) (Publication year: 2016)
Author(s) So Yun, Park Jung Min, Lee Pil-Moo, Kim Cho Long, Lee Cheolhan, Kim Jae Hun
Abstract BACKGROUND
The cerebrospinal fluid (CSF) leakage could be happened spontaneously or related to the procedures such as spinal anesthesia, epidural anesthesia, CSF tapping, intrathecal chemotherapy or other spinal procedures. The leakage of CSF leads to intracranial hypotension of which distinguishing clinical feature is orthostatic headache. The epidural blood patch is a gold-standard treatment for intracranial hypotension-related orthostatic headaches.

OBJECTIVE
We conducted this study to compare the efficacy and number of epidural blood patches for spontaneous and iatrogenic orthostatic headaches.

STUDY DESIGN
Retrospective study.

SETTING
University hospital inpatient and outpatient referred to our pain clinic.

METHODS
Sex, weight, height, cause of orthostatic headache, leakage site evaluation test, epidural blood patch injection level, number of administered epidural blood patches, and pain intensity data were collected. We classified patients into two groups according to the cause of orthostatic headache: spontaneous (Group S) and iatrogenic (Group I). Patients with myelograms were also divided into 2 groups: multiple cerebrospinal fluid (CSF) leakages and no multiple leakages.

RESULTS
Overall, 133 patients (162 procedures) were managed using epidural blood patches. Groups S and I included 34 and 99 patients, respectively. In Group I, 90.9% of the patients achieved complete recovery following a single procedure, whereas 44.1% of Group S patients required repeated procedures. The average number of administered epidural blood patches was significantly higher in Group S (1.48 ± 0.64) than in Group I (1.11 ± 0.35; P = 0.007). Among 23 patients evaluated via myelography, 12 had multiple CSF leakages. Patients with multiple leakages required a significantly higher number of epidural blood patches, compared to patients without multiple leakages (P = 0.023).

LIMITATIONS
This retrospective study reveals several limitations including insufficient evaluation of CSF leakage site by myelogram and the retrospective nature of the study itself.

CONCLUSIONS
Most patients with iatrogenic orthostatic headache required a single epidural blood patch, although most did not undergo a myelogram or similar test. Patients with spontaneous orthostatic headache or multiple CSF leakages were more likely to require a repeated epidural blood patch.Key words: CSF leakage, dural puncture, epidural blood patch, intracranial hypotension, orthostatic headache, spinal headache.
Article ID(s) 27906941 (PubMed)
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Article Not in Favourites List Article not in Must-Read List  Frequency of Nerve Root Sleeve Cysts in Autosomal Dominant Polycystic Kidney Disease
Publication Balkan Medical Journal bmj-33-6-652 2016 (Publication year: 2016)
Author(s) Aşık Murat, Tufan Fatih, Akpınar Timur Selçuk, Akalın Nilgül, Ceyhan Elvan, Tunç Necmeddin, Hasıloğlu Zehra Işık, Altıparmak Mehmet Rıza, Ecder Tevfik, Albayram Sait
Abstract Background
There is sporadic data about the occurrence of spinal meningeal cysts in patients with autosomal dominant polycystic kidney disease (ADPKD). We suggest that there is a relationship with the frequency and size of spinal meningeal cysts and headache, intracranial aneurysms, and cerebrospinal fluid leakage in patients with ADPKD.

Aim
To investigate the relationship with spinal meningeal cyst, cerebrospinal fluid leakage, and headache in patients with ADPKD.

Study Design
Cross-sectional study.

Methods
We enrolled 50 patients with ADPKD and 37 healthy volunteers. This cross-sectional study included patients with ADPKD and matched healthy volunteers. Magnetic resonance imaging myelography was performed using the 3D-T2 HASTE technique in an MRI scanner. We questioned our subjects regarding presence of headache and evaluated headache severity using a visual analog scale. The relationship between the number and size of spinal meningeal cysts with headache, intracranial aneurysms, and liver cysts was also investigated.

Results
Spinal meningeal cysts were more numerous and larger in patients than in controls (14.8±11.6 vs. 6.4±4.6 cysts respectively, p< 0.001, 68.3±49.3 vs. 25.4±20.1 mm, p< 0.001, respectively). Spinal cyst number and size were similar in APDKD patients with or without intracranial aneurysms. Headache score was correlated with the size and number of spinal meningeal cysts. This was valid only in patients with ADPKD.

Conclusion
Abnormality involving the vessel wall in ADPKD may explain the increased number of spinal meningeal cysts in ADPKD. Moreover, leakage of cerebrospinal fluid secondary to spinal meningeal cyst may be responsible for recurrent severe headache by causing spontaneous intracranial hypotension in these patients.
Article ID(s) 27994919 (PubMed)
PMC5156465 (PMC)
10.5152/balkanmedj.2016.151093 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Headache Rounds: Sudden Onset Chronic Daily Headache.
Publication Headache 2016 Feb; 56(2) (Publication year: 2016)
Author(s) Wrobel Goldberg Stephanie, Young William
Abstract No abstract available.
Article ID(s) 26638182 (PubMed)
10.1111/head.12724 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Impact of obesity on post-dural puncture headache.
Publication International journal of obstetric anesthesia (Publication year: 2016)
Author(s) Song J, Zhang T, Choy A, Penaco A, Joseph V
Abstract INTRODUCTION
Previous studies have suggested an inverse relationship between obesity and development of dural puncture headache following spinal anesthesia. However, few have investigated the relationship between obesity and headaches after accidental dural puncture with an epidural needle. This study explored whether obesity has any association with headaches following an accidental dural puncture.

METHODS
Records of patients who received epidural analgesia for labor and vaginal delivery between January 2011 and June 2015 were reviewed. Body mass index, American Society of Anesthesiologists Physical Status and age were analyzed. Chi-squared analysis was performed to determine the relationship between the incidence and severity of dural puncture headaches with body mass index.

RESULTS
A total of 17497 epidurals were placed for vaginal deliveries. Of these 164 patients met our criteria for accidental dural puncture, of whom 51.2% developed dural puncture headaches. Of patients who developed a dural puncture headache 35.7% required an epidural blood patch. Data analysis showed no significant difference between body mass index and incidence of dural puncture headaches regardless of body mass index classification (P>0.05). There was no association between body mass index and the intensity of dural puncture headaches (P=0.29).

CONCLUSION
Patients with a high body mass index do not appear to be protected from experiencing a dural puncture headache after an accidental dural puncture during placement of labor epidural analgesia. Additionally, the intensity of dural puncture headaches does not vary with body mass index.
Article ID(s) 28012862 (PubMed)
10.1016/j.ijoa.2016.10.009 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Intracranial hypotension: diagnosis by trial of Trendelenburg positioning and imaging.
Publication Internal and emergency medicine (Publication year: 2016)
Author(s) Tipirneni Anita, Shah Nirav H, Atchaneeyasakul Kunakorn, Berry Andrew C, Adams David J
Abstract No abstract available.
Article ID(s) 27126682 (PubMed)
10.1007/s11739-016-1456-0 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Intraspinal hemorrhage in spontaneous intracranial hypotension: link to superficial siderosis? Report of 2 cases.
Publication Journal of neurosurgery. Spine 2016 Mar; 24(3) (Publication year: 2016)
Author(s) Schievink Wouter I, Wasserstein Philip, Maya M Marcel
Abstract Spontaneous intracranial hypotension due to a spinal CSF leak has become a well-recognized cause of headaches, but such spinal CSF leaks also are found in approximately half of patients with superficial siderosis of the CNS. It has been hypothesized that friable vessels at the site of the spinal CSF leak are the likely source of chronic bleeding in these patients, but such an intraspinal hemorrhage has never been visualized. The authors report on 2 patients with spontaneous intracranial hypotension and intraspinal hemorrhage, offering support for this hypothesis. A 33-year-old man and a 62-year-old woman with spontaneous intracranial hypotension were found to have a hemorrhage within the ventral spinal CSF collection and within the thecal sac, respectively. Treatment consisted of microsurgical repair of a ventral dural tear in the first patient and epidural blood patching in the second patient. The authors suggest that spontaneous intracranial hypotension should be included in the differential diagnosis of spontaneous intraspinal hemorrhage, and that the intraspinal hemorrhage can account for the finding of superficial siderosis when the CSF leak remains untreated.
Article ID(s) 26588500 (PubMed)
10.3171/2015.6.SPINE15428 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Management of spontaneous intracranial hypotension.
Publication Practical neurology 2016 Apr; 16(2) (Publication year: 2016)
Author(s) Tyagi Alok
Abstract No abstract available.
Article ID(s) 26837373 (PubMed)
10.1136/practneurol-2015-001343 (DOI)
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Article in Favourites List Article is Must-Read  Myelographic Techniques for the Detection of Spinal CSF Leaks in Spontaneous Intracranial Hypotension.
Publication AJR. American journal of roentgenology 2016 Jan; 206(1) (Publication year: 2016)
Author(s) Kranz Peter G, Luetmer Patrick H, Diehn Felix E, Amrhein Timothy J, Tanpitukpongse Teerath Peter, Gray Linda
Abstract OBJECTIVE
Spinal leakage of CSF causes almost all cases of spontaneous intracranial hypotension. Leak detection and localization are important for both diagnosis and treatment. The myelographic appearance of the leaks may vary, however, depending on the cause of the leak, rate of leakage, and imaging modality used.

CONCLUSION
The purpose of this article is to review the imaging of spinal CSF leaks and to assist in the selection of appropriate imaging modalities in this condition.
Article ID(s) 26700332 (PubMed)
10.2214/AJR.15.14884 (DOI)
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Article in Favourites List Article not in Must-Read List  Novel orbital findings of intracranial hypotension.
Publication Clinical imaging 2016 Oct 29; 41 (Publication year: 2016)
Author(s) Holbrook John F, Hudgins Patricia A, Bruce Beau B, Saindane Amit M
Abstract PURPOSE
To determine whether orbital findings on routine brain MRI can be used to differentiate patients with intracranial hypotension from controls.

METHODS
The authors evaluated axial T2-weighted images for the amount of optic nerve sheath CSF and 3D-T1-weighted images for optic nerve angle of sixteen patients with intracranial hypotension and 60 controls.

RESULTS
Patients with intracranial hypotension demonstrated significantly decreased CSF in the optic nerve sheath. Optic nerve angle was higher in the intracranial hypotension group compared to controls.

CONCLUSIONS
Decreased optic nerve sheath CSF and straightened optic nerve angle are significantly more common in the setting of intracranial hypotension.
Article ID(s) 27840264 (PubMed)
10.1016/j.clinimag.2016.10.019 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Patterns of cerebrospinal fluid (CSF) distribution in patients with spontaneous intracranial hypotension: Assessed with magnetic resonance myelography.
Publication Journal of the Chinese Medical Association : JCMA (Publication year: 2016)
Author(s) Chen Chuan-Han, Chen Jeon-Hor, Chen Hung-Chieh, Chai Jyh-Wen, Chen Po-Lin, Chen Clayton Chi-Cheng
Abstract BACKGROUND
Diagnosis of spontaneous intracranial hypotension (SIH) relies on the ability of medical staff to recognize cerebrospinal fluid (CSF) leakage at the spine. However, difficulties with interobserver discrepancy sometimes occurred while reading magnetic resonance myelography (MRM) because clear image definition was lacking. In this study, we tried to determine which pattern of CSF distribution is more reliable for diagnosis of CSF leakage by using MRM.

METHODS
From January 2012 to August 2014, 19 SIH patients and 27 healthy controls (HC) were recruited into our study; 10 of the 19 patients were recovered (SIH-R) after treatment. Whole spine MRM was performed using the 3D-SPACE (three-dimensional sampling perfection with application-optimized contrasts using different flip-angle evolutions) sequence, and interpreted by two experienced neuroradiologists. Two 4-point classification systems of CSF distribution were used to evaluate the three-dimensional maximum intensity projection (3D MIP) and the thin-slice axial multiplanar reconstruction (MPR) images, respectively.

RESULTS
The interobserver agreement between the two readers interpreting the 3D MIP and thin-slice axial MPR MRM were moderate to good (κ=0.60-0.78). Grade 3 of 3D MIP and Type D of axial MPR MRM were only noticed in the SIH. Overall, Grade 3 of MIP and Type D of MPR showed significant difference (p<0.008) between the SIH and the HC in the whole spine. Type C at the T-spine was more frequently noted in the SIH than in the HC (p<0.038). By using "Grade 3", "Type D", "Type D and Type C at T-spine" as the diagnostic criteria of CSF leakage, the sensitivity, specificity, positive predict value (PPV), and negative predict value (NPV) were all > 70%.

CONCLUSION
Grade 3 on 3D MIP and Type D on axial MPR MRM were definite criteria of MRM for localizing CSF leakage, and Type C in the T-spine was a probable leakage sign with high sensitivity and NPV.
Article ID(s) 27743810 (PubMed)
10.1016/j.jcma.2016.02.013 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Prominent Inferior Intercavernous Sinus on Sagittal T1-Weighted Images: A Sign of Intracranial Hypotension.
Publication AJR. American journal of roentgenology 2016 Apr; 206(4) (Publication year: 2016)
Author(s) Alcaide-Leon Paula, López-Rueda Antonio, Coblentz Ailish, Kucharczyk Walter, Bharatha Aditya, de Tilly Lyne Noël
Abstract OBJECTIVE
The purpose of this study is to describe the diagnostic accuracy of the dilatation of the inferior intercavernous sinus as a sign of intracranial hypotension and to raise awareness of this anatomic structure, which can be mistaken for a focal pituitary lesion.

MATERIALS AND METHODS
Sagittal T1-weighted images of 26 patients with intracranial hypotension and 28 control subjects were evaluated to determine the presence of a distended inferior intercavernous sinus. Information about the shape, size, and signal of the inferior intercavernous sinus was also collected. The chi-square test was used to compare both groups. Sensitivity and specificity of the dilatation of the inferior intercavernous sinus as a sign of intracranial hypotension were calculated.

RESULTS
A visible inferior intercavernous sinus was found in 13 of 26 patients with intracranial hypotension (50%) and in four of 28 control subjects (14.3%). These percentages were significantly different (p = 0.005). There was no significant difference in size of the inferior intercavernous sinus in the intracranial hypotension group (median, 5.86 mm(2); interquartile range, 6.28 mm(2)) compared with the control group (median, 8.25 mm(2); interquartile range, 16.69 mm(2)). Changes in the size of the inferior intercavernous sinus were detected in congruence with the appearance or resolution of intracranial hypotension.

CONCLUSION
Dilatation of the inferior intercavernous sinus is frequently associated with intracranial hypotension, although it can also be found in the healthy adult as a normal anatomic variant. Recognition of this anatomic structure is important to avoid mistaking it for a focal pituitary lesion.
Article ID(s) 27003051 (PubMed)
10.2214/AJR.15.14872 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Pseudomeningocele Aspiration and Blood Patch Effectively Treats Positional Headache Associated with Post-Operative Lumbosacral Pseudomeningocele.
Publication Spine (Publication year: 2016)
Author(s) Sandwell Stephen, Walter Kevin, Westesson Per-Lennart
Abstract STUDY DESIGN
Retrospective chart review with limited prospective follow-up survey.

OBJECTIVE
To evaluate the efficacy and safety of blood patch injection for the treatment of positional headaches caused by postoperative lumbosacral pseudomeningoceles.

SUMMARY OF BACKGROUND DATA
Pseudomeningocele is one of the most common complications after posterior lumbosacral spinal surgery. Common treatments include bedrest, abdominal binder use, subarachnoid lumbar drainage, and surgical re-exploration for durotomy closure. To date, only small case reports support the use of epidural blood patch injection for symptomatic pseudomeningocele treatment.

METHODS
A retrospective chart review analyzed the outcomes and complications of nineteen consecutive patients who underwent blood patch injection, with and without pseudomeningocele aspiration, for symptomatic postoperative lumbosacral pseudomeningoceles between 2009 and 2015. An attempt was made to survey patients by phone regarding satisfaction.

RESULTS
As of last follow up (average time = 22.3 months), sixteen patients (84%) experienced headache resolution after blood patch injection and did not require further treatment of their pseudomeningocele. In addition to symptomatic improvement, twelve of the sixteen successful patients had imaging, which demonstrated pseudomeningocele resolution. Persistent pseudomeningoceles were demonstrated on imaging among all three unsuccessful patients.

CONCLUSION
Pseudomeningocele aspiration followed by blood patch is an effective treatment for symptomatic postoperative lumbosacral pseudomenigocele. This is a minimally invasive alternative to surgical re-exploration with durotomy closure. Injections are most effective when performed early after pseudomeningocele development.

LEVEL OF EVIDENCE
Level 4.
Article ID(s) 27922581 (PubMed)
10.1097/BRS.0000000000002003 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Reversible alterations of the neuronal activity in spontaneous intracranial hypotension.
Publication Cephalalgia : an international journal of headache 2016 Feb; 36(2) (Publication year: 2016)
Author(s) Amemiya Shiori, Takahashi Koichi, Mima Tatsuo, Yoshioka Naoki, Miki Soichiro, Ohtomo Kuni
Abstract AIM
The aim of this article is to investigate the pathophysiology underlying the alternation of the cognitive function and neuronal activity in spontaneous intracranial hypotension (SIH).

METHODS
Fifteen patients with SIH underwent resting-state functional magnetic resonance imaging and working-memory (WM) test one day before and one month after a surgical operation. Alternation of the cognitive function and spontaneous neuronal activity measured as amplitude of the low-frequency fluctuations (ALFF) and the functional connectivity of the default-mode network (DMN) and frontoparietal networks (FPNs) were evaluated.

RESULTS
WM performance significantly improved post-operatively. Whole-brain linear regression analysis of the ALFF revealed a positive correlation between cognitive performance change and ALFF change in the precuneus while a negative correlation was found in the bilateral orbitofrontal cortices (OFCs) and right medial frontal cortex (MFC). The ALFF changes normalised with the WM performance improvement post-operatively. The FPN activity in the right OFC was also increased pre-operatively. Partial correlation analysis revealed a significant correlation between WM performance and right OFC activity controlled for right FPN activity.

CONCLUSIONS
The abnormal activity of the OFCs and MFC that is not originating from the synchronous intrinsic network activity, together with the decreased activity of the central node of the DMN, could lead to cognitive impairment in SIH that is reversible through restoration of the cerebrospinal fluid.
Article ID(s) 25934316 (PubMed)
10.1177/0333102415585085 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Reversible coma and Duret hemorrhage after intracranial hypotension from remote lumbar spine surgery: case report.
Publication Journal of neurosurgery. Spine 2016 Mar; 24(3) (Publication year: 2016)
Author(s) Bonow Robert H, Bales James W, Morton Ryan P, Levitt Michael R, Zhang Fangyi
Abstract Intracranial hypotension is a rare condition caused by spontaneous or iatrogenic CSF leaks that alter normal CSF dynamics. Symptoms range from mild headaches to transtentorial herniation, coma, and death. Duret hemorrhages have been reported to occur in some patients with this condition and are traditionally believed to be associated with a poor neurological outcome. A 73-year-old man with a remote history of spinal fusion presented with syncope and was found to have small subdural hematomas on head CT studies. He was managed nonoperatively and discharged with a Glasgow Coma Scale score of 15, only to return 3 days later with obtundation, fixed downward gaze, anisocoria, and absent cranial nerve reflexes. A CT scan showed Duret hemorrhages and subtle enlargement of the subdural hematomas, though the hematomas remained too small to account for his poor clinical condition. Magnetic resonance imaging of the spine revealed a large lumbar pseudomeningocele in the area of prior fusion. His condition dramatically improved when he was placed in the Trendelenburg position and underwent repair of the pseudomeningocele. He was kept flat for 7 days and was ultimately discharged in good condition. On long-term follow-up, his only identifiable deficit was diplopia due to an internuclear ophthalmoplegia. Intracranial hypotension is a rare condition that can cause profound morbidity, including tonsillar herniation and brainstem hemorrhage. With proper identification and treatment of the CSF leak, patients can make functional recoveries.
Article ID(s) 26588496 (PubMed)
10.3171/2015.6.SPINE1521 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous Intracranial Hypotension Associated with Kinetic Tremor and Ataxia.
Publication Tremor and other hyperkinetic movements (New York, N.Y.) 2016; 6 (Publication year: 2016)
Author(s) Salazar Richard
Abstract BACKGROUND
Spontaneous intracranial hypotension (SIH) is a clinically variable syndrome caused by low cerebrospinal fluid (CSF) pressure due to a non-traumatic CSF leak.

PHENOMENOLOGY SHOWN
This case describes a 68-year-old gentleman who presents with chronic and slightly progressive kinetic tremor of bilateral hands associated with gait ataxia and gait start hesitation.

EDUCATIONAL VALUE
This case underscores the importance of having a high index of suspicion for the diagnosis of SIH when encountering a patient presenting with late-onset progressive kinetic tremor and gait ataxia syndrome.
Article ID(s) 27351232 (PubMed)
10.7916/D8HQ3ZN5 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous Intracranial Hypotension: An Etiology for Consciousness Disorder and Coma.
Publication A & A case reports 2016 Nov 15; 7(10) (Publication year: 2016)
Author(s) Collange Olivier, Wolff Valérie, Cebula Hélène, Pradignac Alain, Meyer Alain, Kindo Michel, Diemunsch Pierre, Proust François, Mertes Paul-Michel, Kremer Stéphane
Abstract UNASSIGNED
We report 3 cases of spontaneous intracranial hypotension (SIH) associated with consciousness disorder and coma. In patients, SIH was suspected on a computed tomography scan and diagnosed by cerebral magnetic resonance imaging (MRI). Spinal MRI confirmed cerebrospinal fluid leakage. SIH should be seen as an underestimated cause of consciousness disorder and coma, especially in patients with a history of orthostatic headache, spinal injury, or oculomotor signs. Computed tomography scans should be examined for signs of SIH before operating on patients with a spontaneous subdural hematoma. Brain and spine MRI should be performed when SIH is suspected. Our 3 patients have shown good recovery without any neurological sequelae.
Article ID(s) 27552236 (PubMed)
10.1213/XAA.0000000000000385 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous intracranial hypotension: diagnosis to management.
Publication Acta neurologica Belgica 2016 Jun; 116(2) (Publication year: 2016)
Author(s) Limaye Kaustubh, Samant Rohan, Lee Ricky W
Abstract Spontaneous Intracranial Hypotension typically occurs from spontaneous CSF leak. CSF volume depletion rather than decrease in CSF pressure is thought to be the main causative feature for intracranial hypotension. More and more cases of intracranial hypotension are getting diagnosed with the advances in the imaging. The advances in the imaging have also led to the better understanding of the dynamic changes that occur with intracranial hypotension. The old theories of CSF overproduction or CSF underproduction have not been substantially associated with intracranial hypotension. It has also led to the fore different atypical clinical features and presentations. Although, it has been known for a long time, the diagnosis is still challenging and dilemma persists over one diagnostic modality over other and the subsequent management. Spontaneous CSF leaks occur at the spinal level and the skull base and other locations are rare. The anatomy of spontaneous intracranial hypotension is a very complex process with significant overlap in connective tissue disorders, previous dural weakness or meningeal diverticula. To localize the location of the CSF leak-CT myelography is the modality of choice. CSF cysternography may provide additional confirmation in uncertain cases and also MRI spine imaging may be of significant help in some cases. Spontaneous intracranial hypotension continues to be a diagnostic dilemma and our effort was to consolidate available information on the clinical features, diagnostics, and management for a practicing neurologist for a "15-20 min quick update of the topic".
Article ID(s) 26661291 (PubMed)
10.1007/s13760-015-0577-y (DOI)
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Article Not in Favourites List Article not in Must-Read List  Subarachnoid Hemorrhage and Spinal Subdural Hematoma Due to Acute CSF Hypotension.
Publication Neurocritical care (Publication year: 2016)
Author(s) Graffeo Christopher S, Perry Avital, Wijdicks Eelco F M
Abstract BACKGROUND
Intracranial subarachnoid hemorrhage (SAH) and spinal subdural hematoma (SDH) are rare complications of spine surgery, thought to be precipitated by cerebrospinal fluid (CSF) hypotension in the setting of an intraoperative durotomy or postoperative CSF leak. Considerable clinical variability has been reported, requiring a high level of clinical suspicion in patients with a new, unexplained neurologic deficit after spine surgery.

METHODS
Case report.

RESULTS
An 84-year-old man developed symptomatic spinal stenosis with bilateral lower extremity pseudoclaudication. He underwent L3-5 laminectomy at an outside institution, complicated by a small, incidental, unrepairable intraoperative durotomy. On postoperative day 2, he became confused; and head CT demonstrated intracranial SAH with blood products along the superior cerebellum and bilateral posterior Sylvian fissures. He was transferred to our neurosciences ICU for routine SAH care, with improvement in encephalopathy over several days of supportive care. On postoperative day 10, the patient developed new bilateral lower extremity weakness; MRI of the lumbar spine demonstrated worsening acute spinal SDH above the laminectomy defect, from L4-T12. He was taken to the OR for decompression, at which time a complex 1.5-cm lumbar durotomy was identified and repaired primarily.

CONCLUSIONS
We report the first case of simultaneous intracranial SAH and spinal SDH attributable to postoperative CSF hypotension in the setting of a known intraoperative durotomy. Although rare, each of these entities has the potential to precipitate a poor neurologic outcome, which may be mitigated by early recognition and treatment.
Article ID(s) 27660177 (PubMed)
10.1007/s12028-016-0327-x (DOI)
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Article Not in Favourites List Article not in Must-Read List  Successful Treatment of Post-Dural Puncture Headache Using Epidural Fibrin Glue Patch after Persistent Failure of Epidural Blood Patches.
Publication Pain practice : the official journal of World Institute of Pain (Publication year: 2016)
Author(s) Wong Kevin, Monroe Brian R
Abstract OBJECTIVES
Epidural blood patch is the gold standard for the treatment of post-dural puncture headache (PDPH) when conservative treatments have failed to provide any relief. However, alternative therapies are lacking when epidural blood patch persistently fails to improve symptoms. Failure to treat PDPH may lead to significant functional impairment of daily living. Alternative therapies should be sought to accelerate recovery from PDPH.

CASE REPORT
This case describes a woman who developed PDPH secondary to accidental dural puncture during a spinal cord stimulator trial. She was successfully treated with epidural fibrin glue patch after multiple trials of epidural blood patches.

CONCLUSION
Percutaneous injection of fibrin glue to seal the dural defect demonstrated promising outcomes for both immediate and long-lasting resolution of persistent PDPH in our patient. In the event of epidural blood patch failure, epidural fibrin glue patch may be a reasonable alternative for treatment of persistent PDPH. This article is protected by copyright. All rights reserved.
Article ID(s) 27910226 (PubMed)
10.1111/papr.12541 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Successful Treatment of Spontaneous Intracranial Hypotension by Plugging the Cerebrospinal Fluid Leak with Percutaneous Cyanoacrylate Injection: A Report of 2 Cases.
Publication World neurosurgery 2016 Jul; 91 (Publication year: 2016)
Author(s) Tonnelet Romain, Colnat-Coulbois Sophie, Mione Gioia, Richard Sébastien, Bouaziz Hervé, Audibert Gérard, Anxionnat René, Bracard Serge, Braun Marc
Abstract Spontaneous intracranial hypotension (SIH) is a well-known, but under- or misdiagnosed, condition caused by cerebrospinal fluid leak resulting from idiopathic dural breach. Blind lumbar epidural blood patch is an effective treatment in most cases, but occasionally, even targeted epidural blood patch fails to lead to improvement. In these cases, the cerebrospinal fluid leak is usually repaired surgically, especially for large dural breaches (>5 mm), once the site has been identified by imaging techniques (magnetic resonance myelography/computed tomography [CT] myelography/isotopic transit). We describe a less invasive percutaneous technique consisting of direct puncture into the epidural space with a 25-G needle to access the injection site under CT control. We report 2 cases with good technical and clinical outcome after 1 and 8 years of follow-up (clinical evaluation and brain imaging control by CT). The technique we describe here is of high interest in refractory SIH or for the serious form of the disease, before considering surgical repair. Further prospective studies are required to provide general guidelines in treatment options for patients with SIH.
Article ID(s) 27113404 (PubMed)
10.1016/j.wneu.2016.04.051 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Syringomyelia Caused by Traumatic Intracranial Hypotension: Case Report and Literature Review.
Publication World neurosurgery 2016 Jul; 91 (Publication year: 2016)
Author(s) Richard Sébastien, Humbertjean Lisa, Mione Gioia, Braun Marc, Schmitt Emmanuelle, Colnat-Coulbois Sophie
Abstract BACKGROUND
Syringomyelia due to intracranial hypotension is rarely described. As a consequence, intracranial hypotension is less recognized as a potential cause of syringomyelia or mistaken with Chiari type 1 malformation. The pathogeny is poorly understood, and we lack diagnostic and therapeutic strategies for this particular setting.

CASE DESCRIPTION
We describe a 45-year-old patient who developed syringomyelia after about 10 years of undiagnosed intracranial hypotension caused by traumatic C6 cerebrospinal fluid (CSF) leak. Surgical closing of the leak was required to treat intracranial hypotension after failure of conservative measures and blind epidural patches. It led to a marked improvement of cerebral and spinal signs. We discuss the pathogeny of syringomyelia caused by intracranial hypotension and highlight a mechanical theory of hyperpressure against the cervical spine due to blockage of CSF flow by descent of cerebellar tonsils at the foramen magnum level. We describe discriminating clinical and radiologic signs to differentiate intracranial hypotension from Chiari type 1 malformation and discuss mechanisms and causality relating trauma and intracranial hypotension.

CONCLUSIONS
Syringomyelia can be a consequence of long-term progression of intracranial hypotension, which must be differentiated from Chiari type 1 malformation. In our case, resolution was achieved by detecting and closing the CSF leak causing the intracranial hypotension. Reports of similar cases are necessary to understand the origin of CSF leak in traumatic intracranial hypotension and assess the best therapeutic strategy.
Article ID(s) 27126910 (PubMed)
10.1016/j.wneu.2016.04.062 (DOI)
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Article Not in Favourites List Article not in Must-Read List  The "Hyperdense Paraspinal Vein" Sign: A Marker of CSF-Venous Fistula.
Publication AJNR. American journal of neuroradiology 2016 Jul; 37(7) (Publication year: 2016)
Author(s) Kranz P G, Amrhein T J, Schievink W I, Karikari I O, Gray L
Abstract CSF-venous fistula is a recently reported cause of spontaneous intracranial hypotension that may occur in the absence of myelographic evidence of CSF leak. Information about this entity is currently very limited, but it is of potential importance given the large percentage of cases of spontaneous intracranial hypotension associated with negative myelography findings. We report 3 additional cases of CSF-venous fistula and describe the "hyperdense paraspinal vein" sign, which may aid in its detection.
Article ID(s) 26869470 (PubMed)
10.3174/ajnr.A4682 (DOI)
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Article Not in Favourites List Article not in Must-Read List  The Relief of Unilateral Painful Thoracic Radiculopathy without Headache from Remote Spontaneous Spinal Cerebrospinal Fluid Leak.
Publication Pain research & management 2016; 2016 (Publication year: 2016)
Author(s) Son Byung-Chul, Ha Sang-Woo, Lee Si-Hoon, Choi Jin-Gyu
Abstract Spontaneous intracranial hypotension (SIH) caused by spontaneous spinal cerebrospinal fluid (CSF) leaks produces orthostatic headaches. Although upper arm pain or paresthesia is reportedly associated with SIH from spontaneous spinal CSF leak in the presence of orthostatic headache, low thoracic radicular pain due to spontaneous spinal CSF leak unassociated with postural headache is extremely rare. We report a 67-year-old female who presented with chronic, positional radicular right T11 pain. Computed tomography myelography showed a spontaneous lumbar spinal CSF leak at L2-3 and repeated lumbar epidural blood patches significantly alleviated chronic, positional, and lower thoracic radiculopathic pain. The authors speculate that a chronic spontaneous spinal CSF leak not severe enough to cause typical orthostatic headache or epidural CSF collection may cause local symptoms such as irritation of a remote nerve root. There might be considerable variabilities in the clinical features of SIH which can present a diagnostic challenge.
Article ID(s) 27445613 (PubMed)
10.1155/2016/4798465 (DOI)
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Article Not in Favourites List Article not in Must-Read List  The role of ICP monitoring in patients with persistent cerebrospinal fluid leak following spinal surgery: a case series.
Publication Acta neurochirurgica 2016 Sep; 158(9) (Publication year: 2016)
Author(s) Craven Claudia, Toma Ahmed K, Khan Akbar A, Watkins Laurence D
Abstract BACKGROUND
Cerebrospinal fluid (CSF) leak following spinal surgery is a relatively common surgical complication. A disturbance in the underlying CSF dynamics could be the causative factor in a small group of patients with refractory CSF leaks that require multiple surgical repairs and prolonged hospital admission.

METHODS
A retrospective case series of patients with persistent post spinal surgery CSF leak referred to the hydrocephalus service for continuous intracranial pressure (ICP) monitoring. Patients' notes were reviewed for medical history, ICP data, radiological data, and subsequent management and outcome.

RESULTS
Five patients (two males/three females, mean age, 35.4 years) were referred for ICP monitoring over a 12-month period. These patients had prolonged CSF leak despite multiple repair attempts 252 ± 454 days (mean ± SD). On ICP monitoring, all five patients had abnormal results, with the mean ICP 8.95 ± 4.41 mmHg. Four had abnormal pulse amplitudes, mean 6.15 mmHg ± 1.22 mmHg. All five patients underwent an intervention. Three patients underwent insertion of ventriculoperitoneal (VP) shunts. One patient had venous sinus stent insertion and one patient underwent medical management with acetazolamide. All five of the patients' CSF leak resolved post intervention. The mean time to resolution of CSF leak post intervention was 10.8  ± 12.9 days.

CONCLUSIONS
Abnormal cerebrospinal fluid dynamics could be the underlying factor in patients with a persistent and treatment-refractory CSF leak post spinal surgery. Treatments aimed at lowering ICP may be beneficial in this group of patients. Whether abnormal pressure and dynamics represent a pre-existing abnormality or is induced by spinal surgery should be a subject of further study.
Article ID(s) 27393191 (PubMed)
10.1007/s00701-016-2882-5 (DOI)
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Article Not in Favourites List Article not in Must-Read List  The utility of radioisotope cisternography in low CSF/volume syndromes compared to myelography.
Publication Cephalalgia : an international journal of headache (Publication year: 2016)
Author(s) Monteith Teshamae S, Kralik Stephen F, Dillon William P, Hawkins Randall A, Goadsby Peter J
Abstract OBJECTIVE
The objective of this report is to compare computed tomography (CT) and magnetic resonance (MR) myelography with radioisotope cisternography (RC) for detection of spinal cerebrospinal (CSF) leaks.

METHODS
We retrospectively reviewed 12 spontaneous intracranial hypotension (SIH) patients; CT and RC were performed simultaneously. Three patients had MR myelography.

RESULTS
CT and/or MR myelography identified CSF leaks in four of 12 patients. RC detected spinal leaks in all three patients confirmed by CT myelography; RC identified the CSF leak location in two of three cases, and these were due to osteophytic spicules and/or discs. RC showed only enlarged perineural activity. Only intrathecal gadolinium MR myelography clearly identified a slow leak from a perineural cyst. In eight remaining cases, the leak site was unknown; however, two of these showed indirect signs of CSF leak on RC. CSF slow leaks from perineural cysts were the most common presumed etiology; and the cysts were best visualized on myelography.

CONCLUSION
RC is comparable to CT myelography but has spatial limitations and should be limited to atypical cases.
Article ID(s) 26823556 (PubMed)
10.1177/0333102416628467 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Time-Dependent Changes in Dural Enhancement Associated With Spontaneous Intracranial Hypotension.
Publication AJR. American journal of roentgenology 2016 Dec; 207(6) (Publication year: 2016)
Author(s) Kranz Peter G, Amrhein Timothy J, Choudhury Kingshuk Roy, Tanpitukpongse Teerath Peter, Gray Linda
Abstract OBJECTIVE
The objective of our study was to determine whether the presence of individual imaging signs of spontaneous intracranial hypotension (SIH) is correlated with increasing duration of headache symptoms. Of particular interest is the relationship of symptom duration to dural enhancement because it is the most commonly identified imaging sign in patients with SIH.

MATERIALS AND METHODS
Eighty-nine patients with SIH who underwent pretreatment brain MRI and total-spine CT myelography and whose medical record included data on the duration of clinical symptoms were included in this cross-sectional retrospective study. Brain imaging was reviewed for the presence of dural enhancement, brain sagging, and the "venous distention" sign. CT myelograms were assessed for CSF leak. If present, a leak was subcategorized as a high-flow or low-flow leak. Differences in headache duration between subjects with and those without individual imaging signs were compared.

RESULTS
Subjects without dural enhancement on brain MRI had a longer average duration of symptoms than those with dural enhancement present (average symptom duration: 45.3 ± 59.0 [SD] vs 15.1 ± 33.0 weeks, respectively; p = 0.002). No difference in symptom duration was observed between subjects whose MRI studies showed and those whose MRI studies did not show brain sagging (p = 0.10) or the venous distention sign (p = 0.21). The presence of a CSF leak on CT myelography was not associated with symptom duration (p = 0.56) except in the subgroup of patients with low-flow leaks.

CONCLUSION
Increasing symptom duration in SIH is associated with decreased prevalence of abnormal dural enhancement on brain MRI. Because dural enhancement is considered a hallmark imaging feature of this condition, its absence may exacerbate the problem of underdiagnosis in chronic cases of SIH.
Article ID(s) 27557149 (PubMed)
10.2214/AJR.16.16381 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Transpedicular surgical approach for the management of thoracic osteophyte-induced intracranial hypotension refractory to non-operative modalities: case report and review of literature.
Publication European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society 2016 May; 25 Suppl 1 (Publication year: 2016)
Author(s) Dash Debadutta, Jalali Ali, Harsh Viraat, Omeis Ibrahim
Abstract PURPOSE
In this article, we aim to describe the presentation and management of a case of spontaneous intracranial hypotension caused by a dural tear from a ventral thoracic osteophyte at the T12 level that was refractory to non-surgical treatment modalities. A review of the literature has been performed. Also a proposal of diagnostic and treatment algorithm is presented. Intracranial hypotension and CSF leak as a result of dural tear is a common phenomenon. However, the detection of the source of CSF leak from a thoracic spinal osteophyte has rarely been reported.

METHODS
Diagnostic workup including MRI and CT Myelogram as well as application of epidural blood patches and surgical technique of hemilaminectomy and osteophytectomy by transpedicular approach have been described. Literature review was conducted using relevant search terms in PubMed.

RESULTS
The patient's spontaneous intracranial hypotension symptoms resolved and this persisted on follow up visits. Review our experience as well as similar cases in the literature pointed us towards a diagnostic and treatment algorithm.

CONCLUSIONS
Spontaneous resolution is the norm for intracranial hypotension of most etiologies and management of all such cases begins with fluid resuscitation coupled with bed rest. On failure of conservative therapy, autologous epidural blood patches into the spinal epidural space should be tried, which often produce an immediate relief of symptoms. Osteophyte-induced dural tear and consequent intracranial hypotension may require surgical intervention if the symptoms are refractory to conservative treatment. Under all circumstances a careful step-wise approach for diagnosis and treatment of spontaneous intracranial hypotension needs to be followed, as we have proposed in our article.
Article ID(s) 26831535 (PubMed)
10.1007/s00586-016-4408-5 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Treatment and prognosis of subdural hematoma in patients with spontaneous intracranial hypotension.
Publication Cephalalgia : an international journal of headache 2016 Mar; 36(3) (Publication year: 2016)
Author(s) Chen Ying-Chu, Wang Yen-Feng, Li Jie-Yuan, Chen Shih-Pin, Lirng Jiing-Feng, Hseu Shu-Shya, Tung Hsin, Chen Po-Lin, Wang Shuu-Jiun, Fuh Jong-Ling
Abstract OBJECTIVE
The objective of this article is to elucidate the outcome, prognostic predictors and timing of surgical intervention for subdural hematoma (SDH) in patients with spontaneous intracranial hypotension (SIH).

METHODS
Patients with SDH were identified retrospectively from 227 consecutive SIH patients. Data were collected on demographics, clinical courses, neuroimaging findings, and treatment of SDH, which was later divided into conservative treatment, epidural blood patches (EBP), and surgical intervention. Poor outcome was defined as severe neurological sequelae or death.

RESULTS
Forty-five patients (20%) with SDH (mean maximal thickness 11.9 ± 6.2 mm) were recruited. All 15 patients with SDH < 10 mm achieved good outcomes by either conservative treatment or EBP. Of 30 patients with SDH ≥10 mm, patients with uncal herniation (n = 3) had poor outcomes, even after emergent surgical evacuation (n = 2), compared to those without (n = 27) (100% vs. 0%, p < 0.001). Fourteen patients underwent surgical evacuation, resulting in good outcomes in all 12 who received early intervention and poor outcomes in the remaining two who received delayed intervention after Glasgow Coma Scale (GCS) score ≤8 (100% vs. 0%, p = 0.01).

CONCLUSIONS
Uncal herniation results in poor outcomes in patients with SIH complicated with SDH. In individuals with SDH ≥10 mm and decreased GCS scores, early surgical evacuation might prevent uncal herniation.
Article ID(s) 25944817 (PubMed)
10.1177/0333102415585095 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Treatment strategy for cerebral hypotension caused by spontaneous cerebrospinal fluid leaks.
Publication Acta neurochirurgica 2016 Feb; 158(2) (Publication year: 2016)
Author(s) Janssen Insa, Gempt Jens, Gerhardt Julia, Meyer Bernhard, Ryang Yu-Mi
Abstract OBJECTIVE
Spontaneous spinal cerebrospinal fluid (CSF) leaks are rare (5 per 100,000 per year). Treatment generally consists of conservative therapy or interventional therapy with epidural blood patching. Surgical treatment is conducted rarely, usually in cases when conservative or interventional treatment has failed. The aim of our case series was to assess the clinical outcome after surgery.

METHODS
Our clinical database was reviewed for patients with spontaneous spinal CSF leaks who underwent surgical exploration between 2010 and 2013. Etiology, symptoms, preoperative imaging, type of required surgical method, intraoperative findings, and clinical outcome were reported.

RESULTS
We identified five patients with a mean age of 62 years with spontaneous spinal CSF leaks who were treated surgically. Two patients received surgery after failure of interventional treatment. The origin of the CSF leak could be identified intraoperatively in three cases. Surgical technique in all cases consisted of an interlaminar fenestration or hemilaminectomy and a complete foraminotomy to explore the thecal sack and the exiting nerve roots and identify the CSF leak. After surgery, the preoperative symptoms improved in all patients. In one case, there was a relapse after 4 weeks.

CONCLUSIONS
Preoperative identification of a CSF leak with MRI was positive in only one case. In all other cases, a post-myelography CT had to be performed. In all cases, the preoperative symptoms improved after surgery. Surgical treatment is an effective treatment of spontaneous cerebrospinal fluid leaks in cases of refractory symptoms after failed conservative or interventional treatment.
Article ID(s) 26638152 (PubMed)
10.1007/s00701-015-2653-8 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Uncommon Manifestations of Intervertebral Disk Pathologic Conditions.
Publication Radiographics : a review publication of the Radiological Society of North America, Inc 2016 May-Jun; 36(3) (Publication year: 2016)
Author(s) Diehn Felix E, Maus Timothy P, Morris Jonathan M, Carr Carrie M, Kotsenas Amy L, Luetmer Patrick H, Lehman Vance T, Thielen Kent R, Nassr Ahmad, Wald John T
Abstract Beyond the familiar disk herniations with typical clinical features, intervertebral disk pathologic conditions can have a wide spectrum of imaging and clinical manifestations. The goal of this review is to illustrate and discuss unusual manifestations of intervertebral disk pathologic conditions that radiologists may encounter, including disk herniations in unusual locations, those with atypical imaging features, and those with uncommon pathophysiologic findings. Examples of atypical disk herniations presented include dorsal epidural, intradural, symptomatic thoracic (including giant calcified), extreme lateral (retroperitoneal), fluorine 18 fluorodeoxyglucose-avid, acute intravertebral (Schmorl node), and massive lumbar disk herniations. Examples of atypical pathophysiologic conditions covered are discal cysts, fibrocartilaginous emboli to the spinal cord, tiny calcified disks or disk-level spiculated osteophytes causing spinal cerebrospinal fluid (CSF) leak and intracranial hypotension, and pediatric acute calcific discitis. This broad gamut of disease includes a variety of sizes of disk pathologic conditions, from the tiny (eg, the minuscule calcified disks causing high-flow CSF leaks) to the extremely large (eg, giant calcified thoracic intradural disk herniations causing myelopathy). A spectrum of clinical acuity is represented, from hyperacute fibrocartilaginous emboli causing spinal cord infarct, to acute Schmorl nodes, to chronic intradural herniations. The entities included are characterized by a range of clinical courses, from the typically devastating cord infarct caused by fibrocartilaginous emboli, to the usually spontaneously resolving pediatric acute calcific discitis. Several conditions have important differential diagnostic considerations, and others have relatively diagnostic imaging findings. The pathophysiologic findings are well understood for some of these entities and poorly defined for others. Radiologists' knowledge of this broad scope of unusual disk disease is critical for accurate radiologic diagnoses. Online supplemental material is available for this article. (©)RSNA, 2016.
Article ID(s) 27082664 (PubMed)
10.1148/rg.2016150223 (DOI)
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Article in Favourites List Article not in Must-Read List  Use of Prothrombin Complex Concentrate for Warfarin Reversal Before the Performance of an Epidural Blood Patch in a Patient With Cortical Vein Thrombosis and Subdural Hematoma.
Publication A & A case reports (Publication year: 2016)
Author(s) Chaudhuri Kallol, Phillips Cooper W, Chaudhuri Swapna, Wasnick John
Abstract UNASSIGNED
Compared to conventional therapy, several studies with prothrombin complex concentrate (PCC) have recently demonstrated its superior efficacy in rapidly replacing vitamin K-dependent factors for patients with life-threatening hemorrhage. We present a novel use of PCC in a patient with intracranial hypotension, who had received warfarin for treatment of cortical vein thrombosis. However, after anticoagulation, she proceeded to develop bilateral subdural hematomas with descent of cerebellar tonsils. Given the possibility of an occult dural puncture during labor analgesia, an epidural blood patch was performed after administration of PCC and normalization of coagulation parameters, with prompt improvement of the patient's headache.
Article ID(s) 27861178 (PubMed)
10.1213/XAA.0000000000000417 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Vanishing calcification associated with a spontaneous ventral spinal cerebrospinal fluid leak.
Publication Cephalalgia : an international journal of headache (Publication year: 2016)
Author(s) Schievink Wouter I, Ross Lindsey, Prasad Ravi S, Maya M Marcel
Abstract BACKGROUND
Some patients with spontaneous intracranial hypotension have a ventral spinal cerebrospinal fluid (CSF) leak and these CSF leaks may be associated with calcified disk herniations. Identifying these calcifications is helpful in directing treatment. We report here the unusual case of a patient with a ventral CSF leak in whom the associated calcification absorbed over a five-month period.

CASE REPORT
A 42-year-old woman developed orthostatic headaches and bilateral abducens nerve palsies. Magnetic resonance imaging of her brain showed typical findings of spontaneous intracranial hypotension. Magnetic resonance imaging of her spine showed an extensive cervicothoracic CSF leak. Computed tomographic myelography showed calcification at the Th1-2 disk space. Three epidural blood patches were performed, but her symptoms persisted. Digital subtraction myelography performed five months later showed an upper thoracic ventral CSF, but the calcification was no longer present. A dural tear, found at surgery at the Th1-2 level, was repaired and the patient made an uneventful recovery.

DISCUSSION
The resorption of calcifications at the level of a ventral spinal CSF leak could explain the absence of any calcifications in at least some patients with such leaks and demonstrates the usefulness of reviewing previous imaging in patients with ventral CSF leaks if the exact site of the leak remains unknown.
Article ID(s) 26792915 (PubMed)
10.1177/0333102416628468 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Venous infarction mimicking a neoplasm in spontaneous intracranial hypotension: an unusual cause of Parinaud's syndrome.
Publication Journal of surgical case reports 2016 Mar 17; 2016(3) (Publication year: 2016)
Author(s) Bray Timothy James Pengilley, Chandrashekar Hoskote, Rees Jeremy, Burke Ailbhe, Merve Ashirwad, Thust Stefanie
Abstract We present a case of longstanding, undiagnosed spontaneous intracranial hypotension (SIH) with an acute presentation of Parinaud's syndrome, in whom serial imaging demonstrated development of a midbrain mass. The patient was ultimately diagnosed with tumefactive venous infarction secondary to SIH. However, this patient underwent a brainstem biopsy, which in retrospect may have been avoidable. This case demonstrates the imaging features of tumefactive venous infarction in SIH and highlights the risk of misinterpretation as a neoplasm with potentially catastrophic consequences.
Article ID(s) 26987945 (PubMed)
10.1093/jscr/rjw037 (DOI)
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2015



Article Not in Favourites List Article not in Must-Read List  [A case-series study on clinical presentation, neuroradiological characteristics, and outcome of 56 consecutive patients suspected of having spontaneous intracranial hypotension].
Publication Rinsho shinkeigaku = Clinical neurology 2015; 55(9) (Publication year: 2015)
Author(s) Arai Motomi
Abstract The author reviewed the clinical records and neuroradiologic examinations of 86 consecutive patients with orthostatic headache who visited our clinic between April 1995 and December 2014. Fifty-six patients were suspected to have spontaneous intracranial hypotension (SIH). The baseline characteristics of these patients were essentially similar to those reported in other published case series of SIH: female preponderance, mean age of approximately 40 years, and frequent association with nausea, hearing disturbances, or vertigo. In 43 patients who underwent gadolinium-enhanced MRI, 15 had partial dural enhancement and 15 had diffuse enhancement. Of 13 patients who underwent radionuclide cisternography, a direct finding of cerebrospinal fluid (CSF) leakage was demonstrated in six patients. Ordinal scales were formulated for regression of the extent of dural enhancement on cranial MRI (none: 0, partial: 1, diffuse: 2) and severity of orthostatic headache (not so severe: 1, severe: 2). Ordinal logistic regression analysis demonstrated that the extent of dural enhancement was negatively associated with the severity of orthostatic headache. A possible explanation was that patients suspected of having SIH who showed severe orthostatic headache may lack the ability to compensate for CSF loss. Epidural blood patch (EBP) is targeted at the CSF leak site or at the lumbar level when the site of CSF leak has not been determined. The interval from EBP to disappearance of orthostatic headache did not significantly differ in six patients treated with targeted EBP and five patients with lumbar EBP. Linear regression analysis demonstrated that the duration of orthostatic headache was associated with the interval from onset of headache to initial visit to our clinic, with the slope of the regression line 1.243 and intercept 14.8 days. Thus, early diagnosis of SIH appeared to correlate with earlier disappearance of orthostatic headache. No other factors were found to predict the outcome of SIH.
Article ID(s) 26156257 (PubMed)
10.5692/clinicalneurol.cn-000716 (DOI)
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Article Not in Favourites List Article not in Must-Read List  A Practical Approach to the Diagnosis of Spontaneous Intracranial Hypotension.
Publication Current pain and headache reports 2015 Aug; 19(8) (Publication year: 2015)
Author(s) Steenerson Kristen, Halker Rashmi
Abstract Spontaneous intracranial hypotension can be difficult to diagnose as there are a number of tests available and knowing how to appropriately choose amongst them is not always easy. In this article, we will review the available diagnostic options and provide a practical approach to the workup of a patient with suspected intracranial hypotension.
Article ID(s) 26077206 (PubMed)
10.1007/s11916-015-0509-9 (DOI)
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Article Not in Favourites List Article not in Must-Read List  A short review on a complication of lumbar spine surgery: CSF leak.
Publication Clinical neurology and neurosurgery 2015 Dec; 139 (Publication year: 2015)
Author(s) Menon Sajesh K, Onyia Chiazor U
Abstract Cerebrospinal fluid (CSF) leak is a common complication of surgery involving the lumbar spine. Over the past decades, there has been significant advancement in understanding the basis, management and techniques of treatment for post-operative CSF leak following lumbar spine surgery. In this article, we review previous work in the literature on the various factors and technical errors during or after lumbar spine surgery that may lead to this feared complication, the available options of management with focus on the various techniques employed, the outcomes and also to highlight on the current trends. We also discuss the presentation, factors contributing to its development, basic concepts and practical aspects of the management with emphasis on the different techniques of treatment. Different outcomes following various techniques of managing post-operative CSF leak after lumbar spine surgery have been well described in the literature. However, there is currently no most ideal technique among the available options. The choice of which technique to be applied in each case is dependent on each surgeon's cumulative experience as well as a clear understanding of the contributory underlying factors in each patient, the nature and site of the leak, the available facilities and equipment.
Article ID(s) 26523872 (PubMed)
10.1016/j.clineuro.2015.10.013 (DOI)
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Article Not in Favourites List Article not in Must-Read List  A wearable epidural catheter infusion system for patients with intractable spontaneous intracranial hypotension.
Publication Regional anesthesia and pain medicine 2015 Jan-Feb; 40(1) (Publication year: 2015)
Author(s) Schievink Wouter I, Rosner Howard L, Louy Charles
Abstract BACKGROUND AND OBJECTIVES
Spontaneous intracranial hypotension is an important cause of secondary headaches, and most patients respond well to epidural blood patching or direct repair of the underlying spinal cerebrospinal fluid leak. However, options are limited for those patients who have exhausted these traditional treatments, especially when spinal imaging is normal. We describe a wearable epidural catheter infusion system for patients with intractable spontaneous intracranial hypotension.

METHODS
Six patients with intractable spontaneous intracranial hypotension (4 women and 2 men; mean age, 53 years; mean duration of symptoms, 50 months) underwent placement of a permanent indwelling spinal epidural catheter attached to an external infusion pump. The Migraine Disability Assessment questionnaire was used to assess the severity of the symptoms, before and during treatment.

RESULTS
The infusion resulted in complete or near-complete symptom relief in 5 of 6 patients (Migraine Disability Assessment score decreased from grade IV to grade I or II). However, the epidural catheter infusion system was removed in 2 patients because of infection, in 1 patient because of delayed failure to provide adequate symptom control, and in 1 patient because of minimal symptom relief. Two patients reported excellent and sustained symptom relief over 27 and 36 months of follow-up.

CONCLUSIONS
This wearable epidural catheter infusion system showed promising efficacy results but the high rate of complications limits its use to a very select group of patients.
Article ID(s) 25474623 (PubMed)
10.1097/AAP.0000000000000192 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Audiovestibular impairments associated with intracranial hypotension.
Publication Journal of the neurological sciences 2015 Oct 15; 357(1-2) (Publication year: 2015)
Author(s) Choi Jae-Hwan, Cho Kee-Yong, Cha Seung-Yi, Seo Jae-Deuk, Kim Min-Ji, Choi Yu Ri, Kim Sung-Hee, Kim Ji-Soo, Choi Kwang-Dong
Abstract OBJECTIVE
To investigate the patterns and mechanisms of audiovestibular impairments associated with intracranial hypotension.

METHODS
We had consecutively recruited 16 patients with intracranial hypotension at the Neurology Center of Pusan National University Hospital for two years. Spontaneous, gaze-evoked, and positional nystagmus were recorded using 3D video-oculography in all patients, and the majority of them also had pure tone audiometry and bithermal caloric tests.

RESULTS
Of the 16 patients, five (31.3%) reported neuro-otological symptoms along with the orthostatic headache while laboratory evaluation demonstrated audiovestibular impairments in ten (62.5%). Oculographic analyses documented spontaneous and/or positional nystagmus in six patients (37.5%) including weak spontaneous vertical nystagmus with positional modulation (n=4) and pure positional nystagmus (n=2). One patient presented with recurrent spontaneous vertigo and tinnitus mimicking Meniere's disease, and showed unidirectional horizontal and torsional nystagmus with normal head impulse tests during the attacks. Bithermal caloric tests were normal in all nine patients tested. Audiometry showed unilateral (n=6) or bilateral (n=1) sensorineural hearing loss in seven (53.8%) of the 13 patients tested.

CONCLUSIONS
Intracranial hypotension frequently induces audiovestibular impairments. In addition to endolymphatic hydrops and irritation of the vestibulocochlear nerve, compression or traction of the brainstem or cerebellum due to loss of CSF buoyancy may be considered as a mechanism of frequent spontaneous or positional vertical nystagmus in patients with intracranial hypotension.
Article ID(s) 26165775 (PubMed)
10.1016/j.jns.2015.07.002 (DOI)
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Article in Favourites List Article not in Must-Read List  C1–C2 cryptic cerebrospinal fluid leak directly identified by pressurized radionuclide cisternography: Case report and review of the literature
Publication Surgical Neurology International 10.4103/2152-7806.161787 2015 (Publication year: 2015)
Author(s) Falatko Stephanie Reed, Kelkar Prashant, Setty Pradeep, Tong Doris, Soo Teck Mun
Abstract Background
Patients with chronic postural headaches may suffer from spontaneous intracranial hypotension (SIH). Trauma, degenerative disc spurring and connective tissue disorders are documented risk factors; in most cases there is no inciting event. Despite sophisticated means of evaluating the neuraxis, many cerebrospinal fluid (CSF) leaks are radiographically occult and treatment is focused on thoracic and cervical-thoracic regions. Although lumbar epidural blood patch (EBP) is the initial treatment of choice after failed conservative management, several studies document the need for treatment aimed at the specific leak area.

Case Description
This report describes the case of a 42-year-old female with scleroderma and sudden onset postural headaches. Magnetic resonance imaging revealed diffuse pachymeningeal enhancement suggestive of intracranial hypotension. Computed tomographic myelography demonstrated a collection of fluid ventral to the cervical thecal sac; an exact location for CSF egress was not identified. Conservative measures followed by lumbar EBP failed to alleviate her symptoms. The patient underwent placement of a lumbar drain and dynamic radionuclide cisternography (RIC). Panoramic images of the spine were taken at the time of the pressurized saline injection. The CSF leak was clearly visualized at C1–2. Treatment was focused at this region using percutaneous injection of autologous blood and fibrin glue.

Conclusion
SIH is disabling if left untreated. Spinal CSF leaks are often discrete and difficult to identify using static imaging. The use of pressurized, RIC by lumbar drain injection allows for the real-time evaluation of CSF dynamics and can more precisely identify slow flow leaks often missed with static imaging.
Article ID(s) 26257984 (PubMed)
PMC4524007 (PMC)
10.4103/2152-7806.161787 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Cerebrospinal fluid leakage and headache after lumbar puncture: a prospective non-invasive imaging study.
Publication Brain : a journal of neurology 2015 Jun; 138(Pt 6) (Publication year: 2015)
Author(s) Wang Yen-Feng, Fuh Jong-Ling, Lirng Jiing-Feng, Chen Shih-Pin, Hseu Shu-Shya, Wu Jaw-Ching, Wang Shuu-Jiun
Abstract The spatial distribution and clinical correlation of cerebrospinal fluid leakage after lumbar puncture have not been determined. Adult in-patients receiving diagnostic lumbar punctures were recruited prospectively. Whole-spine heavily T2-weighted magnetic resonance myelography was carried out to characterize post-lumbar puncture spinal cerebrospinal fluid leakages. Maximum rostral migration was defined as the distance between the most rostral spinal segment with cerebrospinal fluid leakage and the level of lumbar puncture. Eighty patients (51 female/29 male, mean age 49.4 ± 13.3 years) completed the study, including 23 (28.8%) with post-dural puncture headache. Overall, 63.6% of periradicular leaks and 46.9% of epidural collections were within three vertebral segments of the level of lumbar puncture (T12-S1). Post-dural puncture headache was associated with more extensive and more rostral distributions of periradicular leaks (length 3.0 ± 2.5 versus 0.9 ± 1.9 segments, P = 0.001; maximum rostral migration 4.3 ± 4.7 versus 0.8 ± 1.7 segments, P = 0.002) and epidural collections (length 5.3 ± 6.1 versus 1.0 ± 2.1 segments, P = 0.003; maximum rostral migration 4.7 ± 6.7 versus 0.9 ± 2.4 segments, P = 0.015). In conclusion, post-dural puncture headache was associated with more extensive and more rostral distributions of periradicular leaks and epidural collections. Further, visualization of periradicular leaks was not restricted to the level of dural defect, although two-thirds remained within the neighbouring segments.
Article ID(s) 25688077 (PubMed)
10.1093/brain/awv016 (DOI)
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Article in Favourites List Article is Must-Read  Changing the needle for lumbar punctures: results from a prospective study.
Publication Clinical neurology and neurosurgery 2015 Mar; 130 (Publication year: 2015)
Author(s) Engedal Thorbjørn S, Ørding Helle, Vilholm Ole Jakob
Abstract OBJECTIVE
Post-dural puncture headache (PDPH) is a common complication of diagnostic lumbar punctures. Both a non-cutting needle design and the use of smaller size needles have been shown to greatly reduce the risk of PDPH. Nevertheless, larger cutting needles are still widely used. This study describes the process of changing the needle in an outpatient clinic of a Danish neurology department.

METHODS
Prospective interventional trial. Phase 1: 22G cutting needle. Phase 2: 25G non-cutting needle. Practical usability of each needle was recorded during the procedure, while the rate of PDPH and the occurrence of socioeconomic complications were acquired from a standardized questionnaire.

RESULTS
651 patients scheduled for diagnostic lumbar punctures were screened for participation and 501 patients were included. The response rate was 80% in both phases. In phase 2, significant reductions were observed in occurrence of PDPH (21 vs. 50, p=0.001), number of days spent away from work (55 vs. 175, p< 0.001), hospitalizations (2 vs. 17, p< 0.001), and number of bloodpatch treatments (2 vs. 10, p=0.019). Furthermore, during the procedure, both the need for multiple attempts (30% vs. 44%, p=0.001), and the failure-rate of the first operator (17% vs. 29%, p=0.005) were reduced.

CONCLUSIONS
Our study showed that smaller, non-cutting needles reduce the incidence of PDPH and are easily implemented in an outpatient clinic. Changing the needle resulted in fewer socioeconomic complications and fewer overall costs, while also reducing procedural difficulty.
Article ID(s) 25590665 (PubMed)
10.1016/j.clineuro.2014.12.020 (DOI)
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Article in Favourites List Article is Must-Read  False localizing sign of cervico-thoracic CSF leak in spontaneous intracranial hypotension.
Publication Neurology 2015 Jun 16; 84(24) (Publication year: 2015)
Author(s) Schievink Wouter I, Maya M Marcel, Chu Ray M, Moser Franklin G
Abstract OBJECTIVE
Spontaneous spinal CSF leaks are an important cause of new-onset headaches. Such leaks are reported to be particularly common at the cervico-thoracic junction. The authors undertook a study to determine the significance of these cervico-thoracic CSF leaks.

METHODS
The patient population consisted of a consecutive group of 13 patients who underwent surgery for CSF leak repair based on CT myelography showing CSF extravasation at the cervico-thoracic junction but without any evidence of an underlying structural lesion.

RESULTS
The mean age of the 9 women and 4 men was 41.2 years. Extensive extrathecal longitudinal CSF collections were demonstrated in 11 patients. At surgery, small leaking arachnoid cysts were found in 2 patients. In the remaining 11 patients, no clear source of CSF leakage could be identified at surgery. Resolution of symptoms was achieved in both patients with leaking arachnoid cysts, but in only 3 of the 11 patients with negative intraoperative findings. Postoperative spinal imaging was performed in 9 of the 11 patients with negative intraoperative findings and showed persistence of the longitudinal intraspinal extradural CSF. Further imaging revealed the site of the CSF leak to be ventral to the thoracic spinal cord. Five of these patients underwent microsurgical repair of the ventral CSF leak with resolution of symptoms in all 5 patients.

CONCLUSIONS
Cervico-thoracic extravasation of dye on myelography does not necessarily indicate the site of the CSF leak. Treatment directed at this site should not be expected to have a high probability of sustained improvement of symptoms.
Article ID(s) 25979700 (PubMed)
10.1212/WNL.0000000000001697 (DOI)
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Article in Favourites List Article is Must-Read  How common is normal cerebrospinal fluid pressure in spontaneous intracranial hypotension?
Publication Cephalalgia : an international journal of headache (Publication year: 2015)
Author(s) Kranz Peter G, Tanpitukpongse Teerath P, Choudhury Kingshuk Roy, Amrhein Timothy J, Gray Linda
Abstract OBJECTIVES
To determine the proportion of patients with spontaneous intracranial hypotension (SIH) who had a cerebrospinal fluid (CSF) pressure >6 cm H2O and to investigate the clinical and imaging variables associated with CSF pressure (PCSF) in this condition.

METHODS
We retrospectively reviewed 106 patients with SIH. PCSF was measured by lumbar puncture prior to treatment. Clinical and imaging variables - including demographic data, brain imaging results, symptom duration, and abdominal circumference - were collected. Univariate and multivariate analyses were performed to determine the correlation of these variables with PCSF.

RESULTS
Sixty-one percent of patients had a PCSF between 6 and 20 cm H2O; only 34% had a PCSF ≤6 cm H2O. The factors associated with increased PCSF included abdominal circumference (p < 0.001), symptom duration (p = 0.015), and the absence of brain magnetic resonance imaging findings of SIH (p = 0.003). A wide variability in PCSF was observed among all patients, which was not completely accounted for by the variables included in the model.

CONCLUSIONS
Normal CSF pressure is common in patients with SIH; the absence of a low opening pressure should not exclude this condition. Body habitus, symptom duration, and brain imaging are correlated with PCSF measurements, but these factors alone do not entirely explain the wide variability in observed pressures in this condition and this suggests the influence of other factors.
Article ID(s) 26682575 (PubMed)
10.1177/0333102415623071 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Hyperprolactinemia due to spontaneous intracranial hypotension.
Publication Journal of neurosurgery 2015 May; 122(5) (Publication year: 2015)
Author(s) Schievink Wouter I, Nuño Miriam, Rozen Todd D, Maya M Marcel, Mamelak Adam N, Carmichael John, Bonert Vivien S
Abstract OBJECT Spontaneous intracranial hypotension is an increasingly recognized cause of headaches. Pituitary enlargement and brain sagging are common findings on MRI in patients with this disorder. The authors therefore investigated pituitary function in patients with spontaneous intracranial hypotension. METHODS Pituitary hormones were measured in a group of 42 consecutive patients with spontaneous intracranial hypotension. For patients with hyperprolactinemia, prolactin levels also were measured following treatment. Magnetic resonance imaging was performed prior to and following treatment. RESULTS The study group consisted of 27 women and 15 men with a mean age at onset of symptoms of 52.2 ± 10.7 years (mean ± SD; range 17-72 years). Hyperprolactinemia was detected in 10 patients (24%), ranging from 16 ng/ml to 96.6 ng/ml in men (normal range 3-14.7 ng/ml) and from 31.3 ng/ml to 102.5 ng/ml in women (normal range 3.8-23.2 ng/ml). In a multivariate analysis, only brain sagging on MRI was associated with hyperprolactinemia. Brain sagging was present in 60% of patients with hyperprolactinemia and in 19% of patients with normal prolactin levels (p = 0.02). Following successful treatment of the spontaneous intracranial hypotension, hyperprolactinemia resolved, along with normalization of brain MRI findings in all 10 patients. CONCLUSIONS Spontaneous intracranial hypotension is a previously undescribed cause of hyperprolactinemia. Brain sagging causing distortion of the pituitary stalk (stalk effect) may be responsible for the hyperprolactinemia.
Article ID(s) 25380110 (PubMed)
10.3171/2014.9.JNS132687 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Incidence and treatment of delayed symptoms of CSF leak following lumbar spinal surgery.
Publication European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society 2015 Sep; 24(9) (Publication year: 2015)
Author(s) Khazim R, Dannawi Z, Spacey K, Khazim M, Lennon S, Reda A, Zaidan A
Abstract PURPOSE
Dural tear (DT) resulting in cerebrospinal fluid (CSF) leak is a common complication of spinal surgery. Most cases of DT are recognised and addressed intraoperatively; however, a small percentage of cases may present at a later stage with delayed symptoms of CSF leak, either due to an unrecognised intraoperative DT or as a result of a de novo delayed DT. Apart from few reports describing delayed symptomatic CSF leaks, most studies tend not to separate intraoperatively recognised DTs from delayed symptomatic CSF leaks. To our knowledge, there are no long-term studies describing specifically the incidence and management of this complication. The aim of this study is to determine the incidence of late presentation of dural tear (LPDT) following lumbar spinal surgery, its treatment, associated complications and clinical outcomes from long-term follow-up in a consecutive series of patients.

METHODS
A retrospective review was conducted on 2052 consecutive patients who underwent spinal surgery by two spinal surgeons from 2000 to 2005 and 2007 to 2013 at two institutions.

RESULTS
A total of 2052 patient records were reviewed. Seventeen patients (0.83%) were found to have LPDT, unrecognised intraoperatively. Fifteen patients required surgical intervention, one patient was treated with insertion of a subarachnoid drain and only one patient settled with conservative measures. Out of the 15 patients who underwent surgery, two patients required another operation and 2 patients were treated with a subarachnoid drain. At 9 months mean follow-up, there was no significant difference in outcome in cases with LPDT compared to those without.

CONCLUSION
A delayed symptomatic presentation of DT unrecognised intraoperatively is a specific complication that needs to be recognised and treated appropriately. A high suspicion and vigilance can help discover and address delayed CSF leaks with no long-term sequelae.
Article ID(s) 25711914 (PubMed)
10.1007/s00586-015-3830-4 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Intrathecal preservative-free normal saline challenge magnetic resonance myelography for the identification of cerebrospinal fluid leaks in spontaneous intracranial hypotension.
Publication Journal of neurosurgery 2015 Sep; 123(3) (Publication year: 2015)
Author(s) Griauzde Julius, Gemmete Joseph J, Pandey Aditya S, Chaudhary Neeraj
Abstract OBJECT
A CSF leak can be difficult to locate in patients who present with spontaneous intracranial hypotension (SIH). The purpose of this case series was to describe the authors' experience with intrathecal preservative-free normal saline challenge coupled with contrast-enhanced MR myelography (CEMRM), which was used to provoke and detect a CSF leakage site in patients with SIH.

METHODS
The authors performed a retrospective review of the records of patients who underwent preservative-free normal saline challenge followed by intrathecal gadolinium (Gd) contrast infusion and MR myelography from 2010 to 2012.

RESULTS
The records survey identified 5 patients who underwent 6 procedures. Intrathecal preservative-free normal saline challenge followed by CEMRM identified a CSF leak during 5 of the 6 procedures. Previous CT myelograms were available from 4 patients, which did not reveal a leakage site. A CT myelogram of 1 patient showed a single leak, but the authors' saline challenge-CEMRM technique identified multiple additional leakage sites. Three patients exhibited transient postprocedural symptoms related to the saline infusion, but no long-term or permanent adverse effects related to the procedure were observed.

CONCLUSIONS
Instillation of preservative-free normal saline into the thecal sac followed by intrathecal Gd infusion is a safe technique that may increase the detection of a CSF leak on MR myelography images in patients with SIH.
Article ID(s) 26140486 (PubMed)
10.3171/2014.12.JNS142057 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Posterior Reversible Encephalopathy Syndrome Secondary to CSF Leak and Intracranial Hypotension: A Case Report and Literature Review.
Publication Case reports in neurological medicine 2015; 2015 (Publication year: 2015)
Author(s) Hammad Tariq, DeDent Alison, Algahtani Rami, Alastal Yaseen, Elmer Lawrence, Medhkour Azedine, Safi Fadi, Assaly Ragheb
Abstract Posterior Reversible Encephalopathy Syndrome (PRES) is a clinical neuroradiological condition characterized by insidious onset of neurological symptoms associated with radiological findings indicating posterior leukoencephalopathy. PRES secondary to cerebrospinal fluid (CSF) leak leading to intracranial hypotension is not well recognized etiology of this condition. Herein, we report a case of PRES that occurred in the setting of CSF leak due to inadvertent dural puncture. Patient underwent suturing of the dural defect. Subsequently, his symptoms resolved and a repeated brain MRI showed resolution of brain lesions. The pathophysiology and mechanistic model for developing PRES in the setting of intracranial hypotension were discussed. We further highlighted the importance of tight blood pressure control in patients with CSF leak and suspected intracranial hypotension because they are more vulnerable to develop PRES with normal or slightly elevated bleed pressure values.
Article ID(s) 26106495 (PubMed)
10.1155/2015/538523 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Posterior thoracic laminoplasty with dorsal, intradural identification of ventral defect and transdural discectomy for a spontaneous cerebrospinal fluid leak: case report.
Publication Journal of neurosurgery. Spine 2015 May; 22(5) (Publication year: 2015)
Author(s) Pricola Fehnel Katie, Borges Lawrence F
Abstract Spontaneous intracranial hypotension (SIH) has been increasingly reported in the literature concomitant with the improved sensitivity of imaging modalities. Although typically associated with meningeal weakening, a handful of cases of SIH secondary to thoracic disc osteophytes have been reported. Five of 7 reported cases were treated with epidural blood patch (EBP) alone while 2 required surgical management. There is no standard operative approach; both anterior and posterolateral approaches can be cumbersome and associated with morbidity, particularly in young, healthy patients. The authors report a case of SIH in which a ventral dural tear secondary to a calcified thoracic disc was repaired via posterior thoracic laminoplasty with dorsal durotomy and intradural exposure of the ventral defect with transdural discectomy followed by primary closure. A 34-year-old man presented with low-pressure headaches following axial load injury from a ski accident 5 years earlier. The patient's symptoms were refractory to a trial of conservative treatment and EBP, and he developed bilateral upper-extremity paresthesias. MRI of the spine demonstrated an extrathecal collection spanning the thoracic spine, and dynamic CT myelography identified contrast extravasation adjacent to a calcified paramedian disc at T9-10. The patient underwent posterior laminoplasty with neuromonitoring. A ventral dural defect was visualized via a dorsal durotomy, the penetrating disc osteophyte was removed transdurally, and the ventral and dorsal dura maters were closed primarily. Both somatosensory and motor evoked potentials were unchanged during surgery. The patient has remained asymptomatic more than 10 months postoperatively and he has resumed work as a surgeon. Cases of SIH secondary to a calcified thoracic disc are rare with little precedent as to optimal surgical intervention. This case illustrates the potential usefulness of posterior laminectomy in nonmyelopathic patients in whom there is no evidence of canal compromise and for whom neuromonitoring is available. Additionally, surgeon experience and patient preference may guide surgical planning.
Article ID(s) 25658466 (PubMed)
10.3171/2014.10.SPINE14439 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Retrospective study of epidural blood patch use for spontaneous intracranial hypotension.
Publication Regional anesthesia and pain medicine 2015 Jan-Feb; 40(1) (Publication year: 2015)
Author(s) Joo Eun Young, Hwang Bo Young, Kong Yu Gyeong, Lee Jong Hyuk, Hwang Beom Sang, Suh Jeong Hun
Abstract BACKGROUND AND OBJECTIVES
Spontaneous intracranial hypotension (SIH) is characterized by a severe and disabling headache that is usually orthostatic in nature. Cisternography is a useful diagnostic test for evaluating the presence and location of cerebrospinal fluid (CSF) leakage, and a targeted epidural blood patch (EBP) based on the cisternography findings is a very effective treatment modality for SIH. However, the effects of EBPs are not predictable, making repeat EBPs essential in some cases. The aim of the present study was to find the relationship between the EBP response and cisternographic findings, hypothesizing that the number of required EBPs would increase with an increased number of CSF leakage levels as determined by radionuclide cisternography.

METHODS
All patients who underwent an EBP and had been discharged with significant improvements in symptoms of SIH during 2006 to 2011 were enrolled. Patients who had no radionuclide cisternographic results were excluded. The demographic variables, number of EBPs, cisternographic findings (location, bilaterality, and number of leakage sites), and preprocedural and postprocedural pain scores were reviewed.

RESULTS
There was no correlation found between the cisternographic findings and the number of EBPs. Only the preprocedural pain scores showed a statistically significant correlation with the number of EBPs.

CONCLUSIONS
Our study suggests that the response to the EBP is related to the severity of symptoms but not to the number and locations of cisternographic CSF leakages.
Article ID(s) 25493688 (PubMed)
10.1097/AAP.0000000000000194 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Risk factors for subdural haematoma in patients with spontaneous intracranial hypotension.
Publication PloS one 2015; 10(4) (Publication year: 2015)
Author(s) Xia Ping, Hu Xing-Yue, Wang Jin, Hu Bei-Bei, Xu Qing-Lin, Zhou Zhi-Jie, Lou Min
Abstract Subdural haematoma (SDH) is a potentially life-threatening complication in patients with spontaneous intracranial hypotension (SIH). In serious cases, SIH patients who present with SDHs develop neurological deficits, a decreased level of consciousness, or cerebral herniation, and may even require an urgent neurosurgical drainage. Despite numerous publications on SDHs, few report its potential risk factors in patients with SIH. In this study, we retrospectively investigated 93 consecutive SIH patients and divided them into an SDH group (n = 25) and a non-SDH (NSDH) group (n = 68). The clinical and radiographic characteristics of these 93 patients were analyzed, and then univariate analysis and further multiple logistic regression analysis were performed to identify the potential risk factors for the development of SDHs. The univariate analysis showed that advanced age, male gender, longer clinical course, dural enhancement, and the venous distension sign were associated with the development of SDHs. However, multivariate analysis only included the latter three factors. Our study reveals important radiological manifestations for predicting the development of SDHs in patients with SIH.
Article ID(s) 25853681 (PubMed)
10.1371/journal.pone.0123616 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous intracranial hypotension as first symptom of aneurysms-osteoarthritis syndrome: a case report.
Publication Headache 2015 May; 55(5) (Publication year: 2015)
Author(s) Koppen Hille, Baars Marieke J H, van Gils Adrianus, Vis Jeroen C
Abstract No abstract available.
Article ID(s) 25877775 (PubMed)
10.1111/head.12551 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous intracranial hypotension syndrome treated with fludrocortisone.
Publication A & A case reports 2015 Jan 1; 4(1) (Publication year: 2015)
Author(s) Rizk Marwan, El Khatib Mohammad, Yamout Bassem, Hujeily Elissar, Ayoub Sophie, Ayoub Chakib, Skaf Ghassan
Abstract Spontaneous intracranial hypotension is a rare syndrome characterized by orthostatic headache not associated with trauma or dural puncture. In most cases, it is caused by a spontaneous spinal cerebrospinal fluid leakage as demonstrated by neuroradiological studies. The standard of care consists of conservative treatment including bed rest, hydration, and administration of caffeine or glucocorticoids. When such conservative therapy fails, an epidural blood patch is recommended. In this report, we describe the treatment of 2 patients with spontaneous intracranial hypotension who failed conservative treatment and went on to have complete and sustained resolution of their symptoms after the administration of oral fludrocortisone.
Article ID(s) 25612272 (PubMed)
10.1213/XAA.0000000000000105 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous Intracranial Hypotension: Characteristics of the Serious Form in a Series of 24 Patients.
Publication World neurosurgery 2015 Dec; 84(6) (Publication year: 2015)
Author(s) Idrissi Aïcha Lyoubi, Lacour Jean-Christophe, Klein Olivier, Schmitt Emmanuelle, Ducrocq Xavier, Richard Sébastien
Abstract BACKGROUND
Recommended treatments for spontaneous intracranial hypotension (SIH) range from bed rest only to neurosurgery. However, the serious form of SIH is poorly defined. A better description of patient characteristics and their outcome may help define therapeutic options.

METHODS
We reviewed 24 cases of patients with SIH and separated them into 2 groups according to whether or not they presented with signs of severity at admission: disturbance of consciousness, subdural hematomas (SDHs), and cerebral venous thrombosis.

RESULTS
Nine patients (37%) were classified as having a serious form of SIH: six (25%) presented SDHs; three (12%) disturbance of consciousness; and one (4%) cerebral venous thrombosis. Bed rest and epidural blood patches (EBPs) were sufficient to treat all patients in the nonserious form group and 4 patients in the serious form group. Two patients (8%) had to undergo cerebrospinal fluid leak repair, and 3 others (12%) evacuation of SDHs. Outcome was good in both groups, with only one (4%) death due to extensive SDHs. Times to diagnosis in the serious form group (63 vs. 35 days, P = 0.052) and to recovery (9 months vs. 5 months, P = 0.088) tended to be higher without reaching difference.

CONCLUSIONS
The presence of SDHs, disturbance of consciousness, and a trend toward a longer time to diagnosis and recovery seem to define the serious form of SIH. These patients may require exploration and surgical repair of cerebrospinal fluid leak, only after failure of conservative measures--bed rest and time--and EBP, with good outcome.
Article ID(s) 26165144 (PubMed)
10.1016/j.wneu.2015.07.002 (DOI)
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Article in Favourites List Article is Must-Read  Spontaneous Intracranial Hypotension.
Publication Continuum (Minneapolis, Minn.) 2015 Aug; 21(4 Headache) (Publication year: 2015)
Author(s) Mokri Bahram
Abstract PURPOSE OF REVIEW
Spontaneous intracranial hypotension results from CSF volume depletion, nearly always from spontaneous CSF leaks. Spontaneous intracranial hypotension is increasingly diagnosed in practice; the number of atypical, unconfirmed, and doubtful cases is also increasing, as are treatment failures. These confront neurologists and create many challenges. This review provides neurologists with a guide to diagnosis, evaluation, and treatment of spontaneous intracranial hypotension.

RECENT FINDINGS
The clinical spectrum of spontaneous intracranial hypotension is expanding. Spontaneous CSF leak is considered a disorder with a variety of clinical manifestations and imaging features, sometimes quite different from what may be seen after dural puncture. The anatomy of the spontaneous CSF leak is frequently complex, with contributions from disorders of the connective tissue matrix and associated preexisting areas of dural weakness and meningeal diverticula. To locate the site of the leak, CT myelography is still the study of choice. For rapid-flow leaks, dynamic CT myelography has been very helpful, while slow-flow leaks can remain a lingering challenge. The fundamental question of whether a CSF leak is present in uncertain cases can be best answered by radioisotope cisternography. In most cases, epidural blood patch is the main treatment; however, bilevel or multilevel epidural injections are gaining some momentum as treatment for selected cases.

SUMMARY
This article outlines various clinical aspects of spontaneous intracranial hypotension, including headache characteristics, CSF changes, and imaging findings and their underlying mechanisms, as well as treatments and disease complications.
Article ID(s) 26252593 (PubMed)
10.1212/CON.0000000000000193 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Successful treatment of spontaneous intracranial hypotension due to prominent cervical cerebrospinal fluid leak with cervical epidural blood patch.
Publication Pain medicine (Malden, Mass.) 2015 May; 16(5) (Publication year: 2015)
Author(s) Wang Eugene, Wang Dajie
Abstract OBJECTIVE
To report a case of successful treatment of a patient with spontaneous intracranial hypotension correlated with MRI finding of cerebrospinal fluid (CSF) leak with extradural collection at the upper cervical spinal level.

DESIGN
Case report.

SETTING
An academic tertiary pain management center.

METHODS
Fluoroscopically guided placement of an 18-gauge epidural needle into epidural space at the C7-T1 level was performed; an epidural catheter (Braun Perifix 20G) was advanced to C2 level (first patch) and C3 level (second patch). An epidurogram with Omnipaque injections confirmed contrast in the posterior and lateral epidural space. Autologous venous blood was then administered through the catheter.

RESULTS
This patient received two lumbar epidural blood patches without lasting relief. Given the radiographic evidence of prominent CSF leak with extradural fluid collection at C1-2 level, the patient was then treated with a cervical epidural blood patch, which provided headache pain relief lasting 6 months. A second cervical epidural blood patch was performed, and the patient has been headache-free for nearly one year to date.

CONCLUSION
Based on the successful treatment of this patient's spontaneous intracranial hypotension, we advocate that patients undergo epidural blood patches to target the site of any CSF leak identified by imaging studies to improve the efficacy of this intervention. This case demonstrates that cervical epidural blood patch, despite its inherent risks, may be more effective than lumbar epidural blood patch in treatment of cervical CSF leak.
Article ID(s) 24666583 (PubMed)
10.1111/pme.12418 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Thoracic Epidural Blood Patches in the Treatment of Spontaneous Intracranial Hypotension: A Retrospective Case Series.
Publication Pain physician 2015 Jul-Aug; 18(4) (Publication year: 2015)
Author(s) Feltracco Paolo, Galligioni Helmut, Barbieri Stefania, Ori Carlo
Abstract BACKGROUND
Spontaneous intracranial hypotension (SIH) results from leaks developing in the dura mater. The major symptom is orthostatic headache which gradually disappears after lying down. Lumbar epidural blood patches (EBPs) can be effective in relieving headaches, however, thoracic and cervical EBPs have also been applied to alleviate the symptoms.

OBJECTIVE AND METHODS
Retrospective collection of the main characteristics of SIH, site and amount of blood injection, and clinical outcomes of 18 patients who underwent thoracic EBPs for intractable SIH.

STUDY DESIGN
Retrospective case series

RESULTS
All thoracic autologous EBPs except 3 were performed in the sitting position. Patients undergoing epidural puncture at lower thoracic levels (T10-T12) received 25 mL of autologous blood, 15 mL and 18 mL were injected at spinal segments T5-T7 (mid-thoracic) and T2-T4 (upper- thoracic), respectively. Thoracic EBPs did not lead to immediate resolution of symptoms in 3 of 18 patients; one of them underwent early repetition with complete headache relief, one refused a second EBP, and one experienced partial resolution, followed by a recurrence, and then satisfactory improvement with a second high thoracic EBP. In long-term follow-up only 2 patients complained of symptoms or relapses.

LIMITATIONS
Retrospective nature of the case series, single center experience.

CONCLUSIONS
Performing thoracic-targeted EBPs as the preferred approach theoretically improves results with respect to those observed with lumbar EBPs. The immediate response was comparable with that of other reports, but the long-term success rate (90%) turned out to be very effective in terms of both quality of headache relief and very low incidence of recurrence.
Article ID(s) 26218937 (PubMed)
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Article Not in Favourites List Article not in Must-Read List  Treatment of spontaneous intracranial hypotension with intravenous Factor XIII administration: initial clinical experience.
Publication Turkish neurosurgery 2015; 25(1) (Publication year: 2015)
Author(s) Nagatani Kimihiro, Takeuchi Satoru, Wada Kojiro, Mori Kentaro, Shima Katsuji
Abstract AIM
Coagulation Factor XIII plays an important role in wound healing by stabilizing the fibrin clot. We hypothesized that Factor XIII administration might promote the repair of cerebrospinal fluid leak sites and lead to resolution of the orthostatic headache in patients with spontaneous intracranial hypotension (SIH). The aim of this study was to investigate the efficacy of intravenous Factor XIII administration in SIH patients.

MATERIAL AND METHODS
A retrospective review of nine patients (four men, five women; mean age 42.3 yr) with SIH resistant to conservative treatment (bed rest, hydration and analgesics) was performed. All patients had an orthostatic headache. Intravenous administration of Factor XIII (1200 units per day for at least five days) was additionally performed on all patients.

RESULTS
The orthostatic headache completely resolved and never reoccurred in six patients (67%), and partially resolved in two patients (22%). One patient (11%) had no change in headache activity. No complications occurred in any patients treated with Factor XIII.

CONCLUSION
This study may suggest that intravenous administration of Factor XIII is useful for treating SIH, even if the patients are resistant to conservative treatment.
Article ID(s) 25640548 (PubMed)
10.5137/1019-5149.JTN.9849-13.1 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Ultrafast dynamic computed tomography myelography for the precise identification of high-flow cerebrospinal fluid leaks caused by spiculated spinal osteophytes.
Publication Journal of neurosurgery. Spine 2015 Mar; 22(3) (Publication year: 2015)
Author(s) Thielen Kent R, Sillery John C, Morris Jonathan M, Hoxworth Joseph M, Diehn Felix E, Wald John T, Rosebrock Richard E, Yu Lifeng, Luetmer Patrick H
Abstract OBJECT
Precise localization and understanding of the origin of spontaneous high-flow spinal CSF leaks is required prior to targeted treatment. This study demonstrates the utility of ultrafast dynamic CT myelography for the precise localization of high-flow CSF leaks caused by spiculated spinal osteophytes.

METHODS
This study reports a series of 14 patients with high-flow CSF leaks caused by spiculated spinal osteophytes who underwent ultrafast dynamic CT myelography between March 2009 and December 2010. There were 10 male and 4 female patients, with an average age of 49 years (range 37-74 years). The value of ultrafast dynamic CT myelography in depicting the CSF leak site was qualitatively assessed.

RESULTS
In all 14 patients, ultrafast dynamic CT myelography was technically successful at precisely demonstrating the site of the CSF leak, the causative spiculated osteophyte piercing the dura, and the relationship of the implicated osteophyte to adjacent structures. Leak sites included 3 cervical, 11 thoracic, and 0 lumbar levels, with 86% of the leaks occurring from C-5 to T-7. Information obtained from the ultrafast dynamic CT myelogram was considered useful in all treated CSF leaks.

CONCLUSIONS
Spinal osteophytes piercing the dura are a more frequent cause of high-flow CSF leaks than previously recognized. Ultrafast dynamic CT myelography adds value beyond standard dynamic myelography or digital subtraction myelography in the diagnosis and anatomical characterization of high-flow spinal CSF leaks caused by these osteophytes. This information allows for appropriate planning for percutaneous or surgical treatment.
Article ID(s) 25555057 (PubMed)
10.3171/2014.10.SPINE14209 (DOI)
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2014



Article Not in Favourites List Article not in Must-Read List  Bariatric surgery as a possible risk factor for spontaneous intracranial hypotension.
Publication Neurology 2014 Nov 11; 83(20) (Publication year: 2014)
Author(s) Schievink Wouter I, Goseland Aimee, Cunneen Scott
Abstract OBJECTIVE
To evaluate a possible link between bariatric surgery and spontaneous intracranial hypotension.

METHODS
The frequency of bariatric surgery was examined in a group of 338 patients with spontaneous intracranial hypotension and compared with a group of 245 patients with unruptured intracranial aneurysms.

RESULTS
Eleven (3.3%) of the 338 patients with spontaneous intracranial hypotension had a history of bariatric surgery compared with 2 (0.8%) of the 245 patients with intracranial aneurysms (p = 0.02). Among the 11 patients with spontaneous intracranial hypotension after bariatric surgery, the mean age at the time of bariatric surgery was 40.8 years (range, 26-53 years) and the mean age at the time of onset of spontaneous intracranial hypotension was 45.6 years (range, 31-59 years). Weight at the time of bariatric surgery ranged from 95 to 166 kg (mean, 130 kg) (body mass index range: 34.9-60.1 kg/m(2); mean: 44.6). Weight at the time of onset of symptoms of spontaneous intracranial hypotension ranged from 52 to 106 kg (mean, 77.5 kg) (body mass index range: 19.2-32.1 kg/m(2); mean: 26.4). The mean weight loss from bariatric surgery to onset of spontaneous intracranial hypotension was 52.5 kg (range, 25-98 kg). Time interval from bariatric surgery to onset of symptoms of spontaneous intracranial hypotension ranged from 3 to 241 months (mean, 56.5 months).

CONCLUSIONS
This case-control study shows that bariatric surgery is a potential risk factor for spontaneous intracranial hypotension.
Article ID(s) 25339216 (PubMed)
10.1212/WNL.0000000000000985 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Cerebral venous thrombosis in two patients with spontaneous intracranial hypotension.
Publication Case reports in neurological medicine 2014; 2014 (Publication year: 2014)
Author(s) Garcia-Carreira M C, Vergé D Cánovas, Branera J, Zauner M, Herrero J Estela, Tió E, Perpinyà G Ribera
Abstract Although few patients with spontaneous intracranial hypotension develop cerebral venous thrombosis, the association between these two entities seems too common to be simply a coincidental finding. We describe two cases of spontaneous intracranial hypotension associated with cerebral venous thrombosis. In one case, extensive cerebral venous thrombosis involved the superior sagittal sinus and multiple cortical cerebral veins. In the other case, only a right frontoparietal cortical vein was involved. Several mechanisms could contribute to the development of cerebral venous thrombosis in spontaneous intracranial hypotension. When spontaneous intracranial hypotension and cerebral venous thrombosis occur together, it raises difficult practical questions about the treatment of these two conditions. In most reported cases, spontaneous intracranial hypotension was treated conservatively and cerebral venous thrombosis was treated with anticoagulation. However, we advocate aggressive treatment of the underlying cerebrospinal fluid leak.
Article ID(s) 25525533 (PubMed)
10.1155/2014/528268 (DOI)
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Article in Favourites List Article not in Must-Read List  CSF-venous fistula in spontaneous intracranial hypotension.
Publication Neurology 2014 Jul 29; 83(5) (Publication year: 2014)
Author(s) Schievink Wouter I, Moser Franklin G, Maya M Marcel
Abstract No abstract available.
Article ID(s) 24951475 (PubMed)
10.1212/WNL.0000000000000639 (DOI)
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Article in Favourites List Article not in Must-Read List  Echocardiographic findings in patients with spontaneous CSF leak.
Publication Journal of neurology 2014 Oct; 261(10) (Publication year: 2014)
Author(s) Pimienta Allen L, Rimoin David L, Pariani Mitchel, Schievink Wouter I, Reinstein Eyal
Abstract The presence of cardiovascular abnormalities in patients with spontaneous cerebrospinal fluid (CSF) leaks are not well-documented in the literature, as cardiovascular evaluation is not generally pursued if a patient does not exhibit additional clinical features suggesting an inherited connective tissue disorder. We aimed to assess this association, enrolling a consecutive group of 50 patients referred for spinal CSF leak consultation. Through echocardiographic evaluation and detailed medical history, we estimate that up to 20% of patients presenting with a spontaneous CSF leak may have some type of cardiovascular abnormality. Further, the increase in prevalence of aortic dilatation in our cohort was statistically significant in comparison to the estimated population prevalence. This supports a clinical basis for echocardiographic screening of these individuals for cardiovascular manifestations that may have otherwise gone unnoticed or evolved into a more severe manifestation.
Article ID(s) 25059392 (PubMed)
10.1007/s00415-014-7438-0 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Fibrin glue to treat spinal fluid leaks associated with intrathecal drug systems.
Publication Pain practice : the official journal of World Institute of Pain 2014 Jul; 14(6) (Publication year: 2014)
Author(s) Freeman Eric D, Hoelzer Bryan C, Eldrige Jason S, Moeschler Susan M
Abstract Intrathecal drug delivery systems (IDDSs) are used to treat resistant pain states as well as intractable spasticity via medication delivery into the spinal fluid. Risks associated with implantation of these devices include infection, bleeding, intrathecal granuloma formation, and neurologic sequelae similar to other neuraxial procedures. Intrathecal catheter placement creates the additional risk of persistent spinal fluid leak, which can lead to postdural puncture headaches as well as seroma formation and may require subsequent surgical exploration or explantation. This retrospective case series examines 3 patients at a single institution with persistent spinal fluid leak after IDDS placement (and explantation in one case) resulting in headache and/or seroma formation that were treated with epidural fibrin glue. Three patients underwent IDDS implantation with baclofen for spasticity. In 1 patient, a cerebral spinal fluid leak developed at 1-week postoperatively. After several unsuccessful epidural blood patches and surgical exploration with a catheter revision, she was ultimately treated successfully with a fibrin glue patch. The second patient received an IDDS and did well until a seroma developed 1 year later. He was likewise treated with an epidural fibrin glue patch after 2 failed blood patches. In a third patient, a spinal fluid leak developed after explantation of an IDDS and was treated with an epidural fibrin glue patch as initial therapy.
Article ID(s) 24256213 (PubMed)
10.1111/papr.12151 (DOI)
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Article in Favourites List Article not in Must-Read List  Headache secondary to intracranial hypotension.
Publication Current pain and headache reports 2014 Nov; 18(11) (Publication year: 2014)
Author(s) Schievink Wouter I, Deline Constance R
Abstract Intracranial hypotension is known to occur as a result of spinal cerebrospinal fluid (CSF) leaking, which may be iatrogenic, traumatic, or spontaneous. Headache is usually, but not always, orthostatic. Spontaneous cases are recognized more readily than in previous decades as a result of a greater awareness of clinical presentations and typical cranial magnetic resonance imaging findings. An underlying disorder of connective tissue that predisposes to weakness of the dura is implicated in spontaneous spinal CSF leaks. CT, MR, and digital subtraction myelography are the imaging modalities of choice to identify spinal CSF leakage. Spinal imaging protocols continue to evolve with improved diagnostic sensitivity. Epidural blood patching is the most common initial intervention for those seeking medical attention, and may be repeated several times. Surgery is reserved for cases that fail to respond or relapse after simpler measures. While the prognosis is generally good with intervention, serious complications do occur. More research is needed to better understand the genetics and pathophysiology of dural weakness as well as physiologic compensatory mechanisms, to continue to refine imaging modalities and treatment approaches, and to evaluate short- and long-term clinical outcomes.
Article ID(s) 25255993 (PubMed)
10.1007/s11916-014-0457-9 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Injecting under pressure: the pain of low CSF pressure headache responsive to botulinum toxin injections.
Publication Current neurology and neuroscience reports 2014 Sep; 14(9) (Publication year: 2014)
Author(s) Mathew Paul G, Cutrer F Michael
Abstract Low intracranial pressure headaches can, at times, be refractory to treatment including multiple blood patches and preventative medications. Imaging studies are often unable to demonstrate a cerebrospinal fluid leak that is causing headache and other associated symptoms. Onabotulinum toxin A (BTX) injection is a treatment that has proven efficacy for the treatment of chronic migraine and potentially other headache disorders. We report a patient with a long standing history of refractory low pressure headaches with brain imaging that demonstrated brain sag, and no CSF leak could be identified. She received no sustained benefit from numerous blood patches, and was unresponsive or intolerant to multiple preventative medications. With BTX treatment, the patient continued to have daily headaches, but her pain intensity improved from an average 7/10 to 3/10. This benefit has been sustained over 7 years. This case suggests that BTX may be an effective treatment for headaches due to low intracranial pressure. It also suggests that the beneficial effects of BTX in the treatment of headaches occur through a direct modulation of the nociceptive system rather than merely induction of pericranial muscle relaxation.
Article ID(s) 25027263 (PubMed)
10.1007/s11910-014-0477-1 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Movement disorders associated with spontaneous CSF leaks: a case series.
Publication Cephalalgia : an international journal of headache 2014 Dec; 34(14) (Publication year: 2014)
Author(s) Mokri Bahram
Abstract IMPORTANCE AND OBJECTIVE
Headache is the most common symptom in spontaneous CSF leaks, frequently associated with additional manifestations. Herein, attention is drawn to movement disorder as a notable manifestation of spontaneous CSF leaks.

DESIGN
Four women and one man (ages 51-78 years) with spontaneous CSF leaks and movement disorders were evaluated clinically and by pertinent neuroimaging studies with follow-up of one to seven years (mean 3.2 years).

RESULTS
The movement disorder consisted of choreiform movements in two patients, torticollis in one, mixed tremor in one, and parkinsonism in one. All except the last patient had headaches (orthostatic in one, Valsalva maneuver-induced in one, both orthostatic and Valsalva-induced in two, lingering low-grade headache in one). Diffuse pachymeningeal enhancement and sinking of the brain was noted in all. CT-myelography showed definite CSF leak in three and equivocal leak in one, while no leak could be located in the fifth patient. Two patients improved over time with complete resolution of the movement disorder. One responded to epidural blood patch with complete resolution of his choreiform movements. Two patients required surgery and epidural blood patches. Results were drastic but nondurable in one, while complete recovery was achieved in the other.

CONCLUSION
Movement disorders are uncommon in spontaneous CSF leaks but occasionally can be one of the major components of the clinical presentation.
Article ID(s) 24728303 (PubMed)
10.1177/0333102414531154 (DOI)
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Article Not in Favourites List Article not in Must-Read List  MR myelography for identification of spinal CSF leak in spontaneous intracranial hypotension.
Publication AJNR. American journal of neuroradiology 2014 Oct; 35(10) (Publication year: 2014)
Author(s) Chazen J L, Talbott J F, Lantos J E, Dillon W P
Abstract BACKGROUND AND PURPOSE
CT myelography has historically been the test of choice for localization of CSF fistula in patients with spontaneous intracranial hypotension. This study evaluates the additional benefits of intrathecal gadolinium MR myelography in the detection of CSF leak.

MATERIALS AND METHODS
We performed a retrospective review of patients with spontaneous intracranial hypotension who underwent CT myelography followed by intrathecal gadolinium MR myelography. All patients received intrathecal iodine and off-label gadolinium-based contrast followed by immediate CT myelography and subsequent intrathecal gadolinium MR myelography with multiplanar T1 fat-suppressed sequences. CT myelography and intrathecal gadolinium MR myelography images were reviewed by an experienced neuroradiologist to determine the presence of CSF leak. Patient records were reviewed for demographic data and adverse events following the procedure.

RESULTS
Twenty-four patients met both imaging and clinical criteria for spontaneous intracranial hypotension and underwent CT myelography followed by intrathecal gadolinium MR myelography. In 3/24 patients (13%), a CSF leak was demonstrated on both CT myelography and intrathecal gadolinium MR myelography, and in 9/24 patients (38%), a CSF leak was seen on intrathecal gadolinium MR myelography (P = .011). Four of 6 leaks identified independently by intrathecal gadolinium MR myelography related to meningeal diverticula. CT myelography did not identify any leaks independently. There were no reported adverse events.

CONCLUSIONS
Present data demonstrate a higher rate of leak detection with intrathecal gadolinium MR myelography when investigating CSF leaks in our cohort of patients with spontaneous intracranial hypotension. Although intrathecal gadolinium is an FDA off-label use, all patients tolerated the medication without evidence of complications. Our data suggest that intrathecal gadolinium MR myelography is a well-tolerated examination with significant benefit in the evaluation of CSF leak, particularly for patients with leak related to meningeal diverticula.
Article ID(s) 24852289 (PubMed)
10.3174/ajnr.A3975 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Orgasmic dural tear: an unusual delayed presentation of postural headache following lumbar discectomy
Publication BMJ Case Reports bcr-2014-208071 2014 (Publication year: 2014)
Author(s) Dannawi Zaher, Lennon Shirley Evelyn, Zaidan Ammar, Khazim Rabi
Abstract No abstract available.
Article ID(s) 25432914 (PubMed)
PMC4248107 (PMC)
10.1136/bcr-2014-208071 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Radioisotope cisternography in spontaneous CSF leaks: interpretations and misinterpretations.
Publication Headache 2014 Sep; 54(8) (Publication year: 2014)
Author(s) Mokri Bahram
Abstract A broadening of the clinical and imaging features of the spontaneous cerebrospinal fluid (CSF) leaks is now well recognized, far beyond what was thought only two decades ago. This has resulted in increasing number of patients with atypical and unusual features who, not unexpectedly, are directed to headache specialists and tertiary referral centers. In many cases, obviously the fundamental question of presence or absence of CSF leak will need to be addressed prior to proceeding with further and often more involved, more invasive, and more costly diagnostic and therapeutic considerations. Radioisotope cisternography often proves to be very helpful in these situations by demonstrating reliable, although indirect, evidences of CSF leak while it is less helpful in directly identifying the exact site of the CSF leakage. In this overview article, the expectations from and the limitations of this diagnostic method are described along with some personal observations in the past 25 years.
Article ID(s) 25041119 (PubMed)
10.1111/head.12421 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Rapid resolution of subdural hematoma after targeted epidural blood patch treatment in patients with spontaneous intracranial hypotension.
Publication Chinese medical journal 2014; 127(11) (Publication year: 2014)
Author(s) Wang Jin, Zhang Dan, Gong Xiangyang, Ding Meiping
Abstract BACKGROUND
Subdural hematoma (SDH) is a common complication of spontaneous intracranial hypotension (SIH). To date, the management of SDH caused by SIH remains controversial. In this paper, we reviewed the clinical course of SDH in patients with SIH, and discuss the underlying mechanism and attributing factors for rapid resolution of subdural hematomas after epidural blood patch (EBP) surgery.

METHODS
We retrospectively reviewed a cohort of seventy-eight SIH patients diagnosed and treated with targeted EBP in our neurology center. Patients who received early CT/MRI follow-up after EBP operation were included.

RESULTS
A series of four cases of SIH complicated with SDHs were evaluated. Early follow-up neuroimages of these patients revealed that SDHs could be partially or totally absorbed just two to four days after targeted epidural blood patch treatment.

CONCLUSION
Targeted epidural blood patch can result in rapid hematoma regression and good recovery in some patients with a combination of SDH and SIH.
Article ID(s) 24890153 (PubMed)
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Article in Favourites List Article is Must-Read  Rebound intracranial hypertension: a complication of epidural blood patching for intracranial hypotension.
Publication AJNR. American journal of neuroradiology 2014 Jun; 35(6) (Publication year: 2014)
Author(s) Kranz P G, Amrhein T J, Gray L
Abstract Rebound intracranial hypertension is a complication of epidural blood patching for treatment of intracranial hypotension characterized by increased intracranial pressure, resulting in potentially severe headache, nausea, and vomiting. Because the symptoms of rebound intracranial hypertension may bear some similarity to those of intracranial hypotension and literature reports of rebound intracranial hypertension are limited, it may be mistaken for refractory intracranial hypotension, leading to inappropriate management. This clinical report of 9 patients with confirmed rebound intracranial hypertension reviews the clinical characteristics of patients with this condition, emphasizing factors that can be helpful in discriminating rebound intracranial hypertension from refractory spontaneous intracranial hypotension, and discusses treatment.
Article ID(s) 24407273 (PubMed)
10.3174/ajnr.A3841 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spinal cerebrospinal fluid leak as the cause of chronic subdural hematomas in nongeriatric patients.
Publication Journal of neurosurgery 2014 Dec; 121(6) (Publication year: 2014)
Author(s) Beck Jürgen, Gralla Jan, Fung Christian, Ulrich Christian T, Schucht Philippe, Fichtner Jens, Andereggen Lukas, Gosau Martin, Hattingen Elke, Gutbrod Klemens, Z'Graggen Werner J, Reinert Michael, Hüsler Jürg, Ozdoba Christoph, Raabe Andreas
Abstract OBJECT
The etiology of chronic subdural hematoma (CSDH) in nongeriatric patients (≤ 60 years old) often remains unclear. The primary objective of this study was to identify spinal CSF leaks in young patients, after formulating the hypothesis that spinal CSF leaks are causally related to CSDH.

METHODS
All consecutive patients 60 years of age or younger who underwent operations for CSDH between September 2009 and April 2011 at Bern University Hospital were included in this prospective cohort study. The patient workup included an extended search for a spinal CSF leak using a systematic algorithm: MRI of the spinal axis with or without intrathecal contrast application, myelography/fluoroscopy, and postmyelography CT. Spinal pathologies were classified according to direct proof of CSF outflow from the intrathecal to the extrathecal space, presence of extrathecal fluid accumulation, presence of spinal meningeal cysts, or no pathological findings. The primary outcome was proof of a CSF leak.

RESULTS
Twenty-seven patients, with a mean age of 49.6 ± 9.2 years, underwent operations for CSDH. Hematomas were unilateral in 20 patients and bilateral in 7 patients. In 7 (25.9%) of 27 patients, spinal CSF leakage was proven, in 9 patients (33.3%) spinal meningeal cysts in the cervicothoracic region were found, and 3 patients (11.1%) had spinal cysts in the sacral region. The remaining 8 patients (29.6%) showed no pathological findings.

CONCLUSIONS
The direct proof of spinal CSF leakage in 25.9% of patients suggests that spinal CSF leaks may be a frequent cause of nongeriatric CSDH.
Article ID(s) 25036203 (PubMed)
10.3171/2014.6.JNS14550 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous cerebrospinal fluid leak following a pilates class: a case report
Publication Journal of Medical Case Reports 10.1186/1752-1947-8-456 2014 (Publication year: 2014)
Author(s) Davis James, Yanny Irini, Chatu Sukhdev, Dubois Patrick, Hayee Bu, Moran Nick
Abstract Introduction
A spinal cerebrospinal fluid leak is the most common cause of spontaneous intracranial hypotension which is an uncommon but increasingly recognized cause of headache. This article describes the first reported case of pilates being associated with a spontaneous spinal cerebrospinal fluid leak whilst also highlighting the key information about spontaneous cerebrospinal fluid leaks that will be useful to the general clinician.

Case presentation
We present the case of a 42-year-old Caucasian woman who developed a low-pressure headache following a pilates class. A computed tomography scan of her head demonstrated bilateral chronic subdural hematomas and cerebellar descent. Magnetic resonance imaging of her spine revealed the presence of extensive extradural cerebrospinal fluid collections. She responded to conservative management and repeat neuroimaging after symptom resolution revealed no abnormalities.

Conclusions
Awareness and early recognition of spontaneous intracranial hypotension is important to prevent unnecessary investigations and delay in treatment. Pilates may be a risk factor for the development of a spontaneous cerebrospinal fluid leak.
Article ID(s) PMC4308014 (PMC)
10.1186/1752-1947-8-456 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous CSF leaks: low CSF volume syndromes.
Publication Neurologic clinics 2014 May; 32(2) (Publication year: 2014)
Author(s) Mokri Bahram
Abstract Practically all cases of spontaneous intracranial hypotension results from spontaneous cerebral spinal fluid (CSF) leaks, often at the level of the spine and only rarely from the skull base. The triad of orthostatic headaches, diffuse pachymeningeal enhancement on head imaging and low CSF opening pressure is considered the hallmark of these leaks but substantial variability is noted in most aspects of this disorder including in features of the headaches, imaging and CSF findings, response to treatment and outcome.
Article ID(s) 24703536 (PubMed)
10.1016/j.ncl.2013.11.002 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous intracranial hypotension: improving recognition and treatment strategies in the local setting.
Publication Hong Kong medical journal = Xianggang yi xue za zhi 2014 Dec; 20(6) (Publication year: 2014)
Author(s) Lee Gregory K Y, Abrigo Jill M, Cheung Tom C Y, Siu Deyond Y W, Chan Danny T M
Abstract We report a case of spontaneous intracranial hypotension with classic symptoms of orthostatic headache and acute presentation of subdural haematoma on computed tomographic scan. Conventional approach with conservative treatment was initially adopted. The patient's condition, however, deteriorated after 2 weeks, requiring surgical evacuation of the intracranial haemorrhage. We reviewed the clinical features of this disease and the correlated magnetic resonance imaging findings with the pathophysiological mechanisms, and described treatment strategies in the local setting. Subtle findings on initial computed tomographic scan are also reported which might improve pathology recognition. Spontaneous intracranial hypotension is not uncommonly encountered in Hong Kong, and physicians must adopt a high level of clinical suspicion to facilitate early diagnosis and appropriate management. In addition, novel therapeutic approaches may be required in those with recurrent symptoms or who are refractory to current treatment strategies.
Article ID(s) 25488033 (PubMed)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous intracranial hypotension: presentation, diagnosis, and treatment.
Publication Anesthesiology 2014 Dec; 121(6) (Publication year: 2014)
Author(s) Williams Elizabeth Cox, Buchbinder Bradley R, Ahmed Shihab, Alston Theodore A, Rathmell James P, Wang Jingping
Abstract No abstract available.
Article ID(s) 25118954 (PubMed)
10.1097/ALN.0000000000000410 (DOI)
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2013



Article Not in Favourites List Article not in Must-Read List  A review article on the diagnosis and treatment of cerebrospinal fluid fistulas and dural tears occurring during spinal surgery
Publication Surgical Neurology International 10.4103/2152-7806.111427 2013 (Publication year: 2013)
Author(s) Epstein Nancy E.
Abstract Background
In spinal surgery, cerebrospinal fluid (CSF) fistulas attributed to deliberate dural opening (e.g., for tumors, shunts, marsupialization of cysts) or inadvertent/traumatic dural tears (DTs) need to be readily recognized, and appropriately treated.

Methods
During spinal surgery, the dura may be deliberately opened to resect intradural lesions/tumors, to perform shunts, or to open/marsupialize cysts. DTs, however, may inadvertently occur during primary, but are seen more frequently during revision spinal surgery often attributed to epidural scarring. Other etiologies of CSF fistulas/DTs include; epidural steroid injections, and resection of ossification of the posterior longitudinal ligament (OPLL) or ossification of the yellow ligament (OYL). Whatever the etiology of CSF fistulas or DTs, they must be diagnosed utilizing radioisotope cisternography (RIC), magnetic resonance imaging (MRI), computed axial tomography (CT) studies, and expeditiously repaired.

Results
DTs should be repaired utilizing interrupted 7-0 Gore-Tex (W.L. Gore and Associates Inc., Elkton, MD, USA) sutures, as the suture itself is larger than the needle; the larger suture occludes the dural puncture site. Closure may also include muscle patch grafts, dural patches/substitutes (bovine pericardium), microfibrillar collagen (Duragen: Integra Life Sciences Holdings Corporation, Plainsboro, NJ), and fibrin glues or dural sealants (Tisseel: Baxter Healthcare Corporation, Deerfield, IL, USA). Only rarely are lumbar drains and wound-peritoneal and/or lumboperitoneal shunts warranted.

Conclusion
DTs or CSF fistulas attributed to primary/secondary spinal surgery, trauma, epidural injections, OPLL, OYL, and other factors, require timely diagnosis (MRI/CT/Cisternography), and appropriate reconstruction.
Article ID(s) PMC3801173 (PMC)
10.4103/2152-7806.111427 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Brain herniation induced by drainage of subdural hematoma in spontaneous intracranial hypotension.
Publication Asian journal of neurosurgery 2013 Apr; 8(2) (Publication year: 2013)
Author(s) Chotai Silky, Kim Jong-Hyun, Kim Joo-Han, Kwon Taek-Hyun
Abstract Spontaneous intracranial hypotension (SIH), typically presents with orthostatic headache, low pressure on lumbar tapping, and diffuse pachymeningeal enhancement on magnetic resonance imaging. SIH is often accompanied by subdural fluid collections, which in most cases responds to conservative treatment or spinal epidural blood patch. Several authors advocate that large subdural hematoma with acute deterioration merits surgical drainage; however, few have reported complications following craniotomy. We describe a complicated case of SIH, which was initially diagnosed as acute subarachnoid hemorrhage with bilateral chronic subdural hematoma (SDH), due to unusual presentation. Burr hole drainage of subdural hematoma was performed due to progressive decrease of consciousness, which then resulted in a huge postoperative epidural hematoma collection. Prompt hematoma evacuation did not restore the patient's consciousness but aggravated downward brain herniation. Trendelenburg position and spinal epidural blood patch achieved a rapid improvement in patient's consciousness. This case indicates that the surgical drainage for chronic SDH in SIH can lead to serious complications and it should be cautiously considered.
Article ID(s) 24049555 (PubMed)
10.4103/1793-5482.116390 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Cerebrospinal fluid volume-depletion headaches in patients with traumatic brachial plexus injury.
Publication Journal of neurosurgery 2013 Jan; 118(1) (Publication year: 2013)
Author(s) Hébert-Blouin Marie-Noëlle, Mokri Bahram, Shin Alexander Y, Bishop Allen T, Spinner Robert J
Abstract OBJECT
Patients with brachial plexus injury (BPI) present with a combination of motor weakness/paralysis, sensory deficits, and pain. Brachial plexus injury is generally not believed to be associated with headaches. However, CSF leaks may be associated with CSF volume-depletion (low-pressure) headaches and can occur in BPI secondary to nerve root avulsion. Only a few cases of headaches associated with BPI have been reported. It is unknown if headaches in patients with BPI occur so rarely, or if they are just unrecognized by physicians and/or patients in which the focus of attention is the affected limb. The aim of this study was to determine the prevalence of CSF volume-depletion headaches in patients with BPI.

METHODS
All adult patients presenting at the Mayo brachial plexus clinic with traumatic BPI were asked to complete a questionnaire addressing the presence and quality of headaches following their injury. The patients' clinical, injury, and imaging characteristics were subsequently reviewed.

RESULTS
Between December 2008 and July 2010, 145 patients completed the questionnaire. Twenty-two patients reported new onset headaches occurring after their BPI. Eight of these patients experienced positional headaches, suggestive of CSF volume depletion. One of the patients with orthostatic headaches was excluded because the headaches immediately followed a lumbar puncture for a myelogram. Six of the other 7 patients with positional headaches had a clear preganglionic BPI. The available imaging studies in these 6 patients revealed evidence of CSF leaks: pseudomeningoceles (n = 5), CSF tracking into soft tissues (n = 3), CSF tracking into the intraspinal compartment (n = 3), CSF tracking into the pleural space (n = 2), and low-positioned cerebellar tonsils (n = 2).

CONCLUSIONS
In this retrospective study, 15.2% of patients (22 of 145 patients) with traumatic BPI suffered from a new-onset headache. Seven of these patients (4.8%) experienced postural headaches clearly suggestive of CSF volume depletion likely secondary to a CSF leak associated with the BPI, whereas the other 15 patients (10.3%) suffered headaches that may have represented a variant of CSF depletion headaches without a postural characteristic or a headache from another cause. These data suggest that CSF volume-depletion headaches occur in a significant proportion of patients with BPI and have been underrecognized and underreported.
Article ID(s) 23082886 (PubMed)
10.3171/2012.9.JNS112368 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Connective tissue spectrum abnormalities associated with spontaneous cerebrospinal fluid leaks: a prospective study.
Publication European journal of human genetics : EJHG 2013 Apr; 21(4) (Publication year: 2013)
Author(s) Reinstein Eyal, Pariani Mitchel, Bannykh Serguei, Rimoin David L, Schievink Wouter I
Abstract We aimed to assess the frequency of connective tissue abnormalities among patients with cerebrospinal fluid (CSF) leaks in a prospective study using a large cohort of patients. We enrolled a consecutive group of 50 patients, referred for consultation because of CSF leak. All patients have been carefully examined for the presence of connective tissue abnormalities, and based on findings, patients underwent genetic testing. Ancillary diagnostic studies included echocardiography, eye exam, and histopathological examinations of skin and dura biopsies in selected patients. We identified nine patients with heritable connective tissue disorders, including Marfan syndrome, Ehlers-Danlos syndrome and other unclassified forms. In seven patients, spontaneous CSF leak was the first noted manifestation of the genetic disorder. We conclude that spontaneous CSF leaks are associated with a spectrum of connective tissue abnormalities and may be the first noted clinical presentation of the genetic disorder. We propose that there is a clinical basis for considering spontaneous CSF leak as a clinical manifestation of heritable connective tissue disorders, and we suggest that patients with CSF leaks should be screened for connective tissue and vascular abnormalities.
Article ID(s) 22929030 (PubMed)
10.1038/ejhg.2012.191 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Evaluation on a clinical course of subdural hematoma in patients undergoing epidural blood patch for spontaneous cerebrospinal fluid leak.
Publication Clinical neurology and neurosurgery 2013 Aug; 115(8) (Publication year: 2013)
Author(s) Hashizume Keiji, Watanabe Keisuke, Kawaguchi Masahiko, Fujiwara Aki, Furuya Hitoshi
Abstract OBJECTIVE
Subdural hematoma (SDH) is a frequent complication of spontaneous intracranial hypotension (SIH), in which epidural blood patch (EBP) may be applied as a treatment to stop cerebrospinal fluid (CSF) leak. However, a clinical course of SDH in SIH patients has not been sufficiently evaluated. We retrospectively evaluated the temporal relationships between EBP and SDH in the patients with SIH.

METHODS
Twenty-nine consecutive patients, diagnosed as SIH, were studied. Clinical records and images were retrospectively evaluated. When orthostatic headache continued for 2 weeks regardless of conservative treatment, EBP was performed under fluoroscopy.

RESULTS
We detected 13(45%) cases of SDH (mean age 44 years, 8 males and 5 females). In 6 patients, SDHs disappeared after effective EBP, i.e., after the disappearance of orthostatic headache. In 3 patients, SDHs were enlarged or recurred after effective EBP, and in 4 patients, SDHs were first detected after effective EBP.

CONCLUSION
The knowledge of the presence of these types of SDH (enlarged or recurred or detected after EBP) may deserve clinical attention.
Article ID(s) 23462186 (PubMed)
10.1016/j.clineuro.2013.01.022 (DOI)
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Article in Favourites List Article not in Must-Read List  Novel neuroimaging modalities in the evaluation of spontaneous cerebrospinal fluid leaks.
Publication Current neurology and neuroscience reports 2013 Jul; 13(7) (Publication year: 2013)
Author(s) Schievink Wouter I
Abstract Although for the vast majority of patients with spontaneous intracranial hypotension knowledge of the exact site of the underlying spinal CSF leak is not necessary, it is for patients with recalcitrant symptoms. Such patients may require directed treatments such as percutaneous fibrin glue injections or surgery. A variety of MRI techniques have been shown to be able to detect CSF leaks as well and sometimes better than the "gold standard" - CT-myelography. For unusually rapid CSF leaks - particularly those ventral to the spinal cord - digital subtraction myelography or dynamic CT-myelography are indicated. Some patients with spontaneous intracranial hypotension verified by intracranial MRI are never found to have a spinal CSF leak using current techniques.
Article ID(s) 23703239 (PubMed)
10.1007/s11910-013-0358-z (DOI)
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Article Not in Favourites List Article not in Must-Read List  Occipital neuralgia secondary to CerebroSpinal fluid leak
Publication The Journal of Headache and Pain 10.1186/1129-2377-14-S1-P151 2013 (Publication year: 2013)
Author(s) Ansari H
Abstract No abstract available.
Article ID(s) PMC3620309 (PMC)
10.1186/1129-2377-14-S1-P151 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Primary spontaneous cerebrospinal fluid leaks and idiopathic intracranial hypertension.
Publication Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society 2013 Dec; 33(4) (Publication year: 2013)
Author(s) Pérez Mario A, Bialer Omer Y, Bruce Beau B, Newman Nancy J, Biousse Valérie
Abstract INTRODUCTION
Idiopathic intracranial hypertension (IIH) is increasingly recognized as a cause of spontaneous cerebrospinal fluid (CSF) leak in the otolarnygological and neurosurgical literature. The diagnosis of IIH in patients with spontaneous CSF leaks typically is made a few weeks after surgical repair of the leak when symptoms and signs of elevated intracranial pressure (ICP) appear.

METHODS
Case reports and literature review. Two young obese women developed spontaneous CSF rhinorrhea related to an empty sella in one and a cribriform plate encephalocele in the other. Both patients underwent surgical repair of the CSF leak. A few weeks later, they developed chronic headaches and bilateral papilledema. Lumbar punctures showed elevated CSF opening pressures with normal CSF contents, with temporary improvement of headaches. A man with a 3-year history of untreated IIH developed spontaneous CSF rhinorrhea. He experienced improvement of his headaches and papilledema after a CSF shunting procedure, and the rhinorrhea resolved after endoscopic repair of the leak.

RESULTS
These cases and the literature review confirm a definite association between IIH and spontaneous CSF leak based on: 1) similar demographics; 2) increased ICP in some patients with spontaneous CSF leak after leak repair; 3) higher rate of leak recurrence in patients with raised ICP; 4) patients with intracranial hypertension secondary to tumors may develop CSF leak, confirming that raised ICP from other causes than IIH can cause CSF leak.

CONCLUSIONS
CSF leak occasionally may keep IIH patients symptom-free; however, classic symptoms and signs of intracranial hypertension may develop after a CSF leak is repaired, exposing these patients to a high risk of recurrence of the leak unless an ICP-lowering intervention is performed.
Article ID(s) 24042170 (PubMed)
10.1097/WNO.0b013e318299c292 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Sensitivity of MRI of the spine compared with CT myelography in orthostatic headache with CSF leak.
Publication Neurology 2013 Nov 12; 81(20) (Publication year: 2013)
Author(s) Starling Amaal, Hernandez Fatima, Hoxworth Joseph M, Trentman Terrence, Halker Rashmi, Vargas Bert B, Hastriter Eric, Dodick David
Abstract OBJECTIVE
To investigate the sensitivity of MRI of the spine compared with CT myelography (CTM) in detecting CSF leaks.

METHODS
Between July 1998 and October 2010, 12 patients with orthostatic headache and a CTM-confirmed spinal CSF leak underwent an MRI of the spine with and without contrast. Using CTM as the gold standard, we retrospectively investigated the sensitivity of spinal MRI in detecting a CSF leak.

RESULTS
Eleven of 12 patients with a CSF leak documented by CTM also had extradural fluid collections on spinal MRI (sensitivity 91.7%). Six patients with extradural fluid collections on spinal MRI also had spinal dural enhancement.

CONCLUSION
When compared with the gold standard of CTM, MRI of the spine appears to be a sensitive and less invasive imaging modality for detecting a spinal CSF leak, suggesting that MRI of the spine should be the imaging modality of first choice for the detection of spinal CSF leaks.
Article ID(s) 24107860 (PubMed)
10.1212/01.wnl.0000435555.13695.22 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spinal manifestations of spontaneous intracranial hypotension.
Publication Journal of neurosurgery. Spine 2013 Jan; 18(1) (Publication year: 2013)
Author(s) Schievink Wouter I, Chu Ray M, Maya M Marcel, Johnson J Patrick, Cohen Hart C M
Abstract OBJECT
The goal of the study was to elucidate the spinal manifestations of spontaneous intracranial hypotension.

METHODS
The authors reviewed the medical records and imaging studies of 338 consecutive patients with spontaneous intracranial hypotension who were evaluated at their institution between 2001 and 2010.

RESULTS
Twenty patients (6%; mean age 35.8 [range 16 to 60 years]; 5 males and 15 females) had convincing signs or symptoms referable to the spinal cord or spinal nerve roots. The spinal manifestations consisted of radiculopathy in 11 patients (unilateral in 8 and bilateral in 3), myelopathy in 8 patients, and bibrachial amyotrophy in 1 patient. The cervical spine was involved in 12 patients, the thoracic spine in 5, and the lumbosacral spine in 3. The spinal symptoms were positional in only 3 patients. The spinal manifestations occurred around the time of the headache onset in 16 patients, and months to years after the positional headache had resolved in 4 patients. A large extrathecal CSF collection causing compression of the spinal cord or nerve root was responsible for the spinal manifestations in the majority of patients. Treatment of the spinal CSF leak resulted in resolution of the spinal manifestations along with the headache, except for those in the patient with bibrachial amyotrophy.

CONCLUSIONS
Spinal manifestations are uncommon in cases of spontaneous intracranial hypotension, occurring in about 6% of patients, but myelopathy and radiculopathy involving all spinal segments do occur. Unlike the headache, the spinal manifestations usually are not positional and are caused by mass effect from an extradural CSF collection.
Article ID(s) 23121651 (PubMed)
10.3171/2012.10.SPINE12469 (DOI)
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Article in Favourites List Article is Must-Read  Spinal meningeal diverticula in spontaneous intracranial hypotension: analysis of prevalence and myelographic appearance.
Publication AJNR. American journal of neuroradiology 2013 Jun-Jul; 34(6) (Publication year: 2013)
Author(s) Kranz P G, Stinnett S S, Huang K T, Gray L
Abstract BACKGROUND AND PURPOSE
Spinal meningeal diverticula have been implicated in the pathogenesis of spontaneous intracranial hypotension and have been proposed as both diagnostic features of and therapeutic targets for the condition. We compared the prevalence and myelographic appearance of spinal diverticula in patients with SIH and healthy controls.

MATERIALS AND METHODS
Patients satisfying the ICHD-2 criteria for SIH were retrospectively identified. CT myelograms of 19 patients with SIH were compared with CT myelograms of 18 control patients. Images were reviewed by 2 blinded neuroradiologists. The prevalence, morphology (round versus multilobulated), size, and location (cervical, upper thoracic, lower thoracic, or lumbar) of spinal meningeal diverticula were analyzed.

RESULTS
There was no difference in the proportion of patients with diverticula in the SIH group compared with the control group (68% versus 44%, P = .14) or in the mean number of diverticula per patient (6.3 versus 2.2, P = .099). No difference was seen in the morphology (P = .95) or size (P = .71) of diverticula between groups. There was a difference between groups that just reached statistical significance (P = .050) in the location of the diverticula along the spinal axis, but substantial overlap was seen between groups for all spinal locations.

CONCLUSIONS
Despite the well-established association between spinal meningeal diverticula and SIH, we found no difference in the prevalence or myelographic appearance of diverticula in patients with SIH compared with controls. Further investigation into the role of diverticula in the diagnosis and treatment of SIH is necessary.
Article ID(s) 23221945 (PubMed)
10.3174/ajnr.A3359 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous intracranial hypotension in childhood and adolescence.
Publication The Journal of pediatrics 2013 Aug; 163(2) (Publication year: 2013)
Author(s) Schievink Wouter I, Maya M Marcel, Louy Charles, Moser Franklin G, Sloninsky Liliana
Abstract OBJECTIVES
To describe the clinical and radiographic manifestations of spontaneous intracranial hypotension, a rarely diagnosed cause of headache in children.

STUDY DESIGN
This study included patients 19 years of age or younger evaluated between January 1, 2001, and June 30, 2012, for spontaneous intracranial hypotension.

RESULTS
We evaluated 24 children (18 girls and 6 boys) with spontaneous intracranial hypotension (age at onset of symptoms: 2-19 years, mean 14.3 years). Twenty-three patients presented with orthostatic headaches and 1 presented with a nonpositional headache. A generalized connective tissue disorder was diagnosed in 54% of patients. Magnetic resonance imaging showed the typical changes of spontaneous intracranial hypotension in most patients (79%). Spinal imaging demonstrated a cerebrospinal fluid (CSF) leak with or without an associated meningeal diverticulum in 12 patients (50%) and with dural ectasia or meningeal diverticula in 10 patients (42%), and it was normal in 2 patients (8%). Twenty-three patients initially underwent epidural blood patching, but 8 patients also were treated with percutaneous injections of fibrin glue and 11 patients eventually required surgical correction of the underlying CSF leak. There was no morbidity or mortality associated with any of the treatments, but 5 patients required acetazolamide for rebound high intracranial pressure headache. Overall, outcome was good in 22 patients (92%) and poor in 2 patients (8%).

CONCLUSIONS
Spontaneous intracranial hypotension in childhood is rare. Most patients can be treated effectively using a combination of epidural blood patching and percutaneous injections of fibrin glue or surgical CSF leak repair in refractory cases.
Article ID(s) 23453548 (PubMed)
10.1016/j.jpeds.2013.01.055 (DOI)
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Article in Favourites List Article is Must-Read  Spontaneous intracranial hypotension: recommendations for management.
Publication The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques 2013 Mar; 40(2) (Publication year: 2013)
Author(s) Amoozegar Farnaz, Guglielmin Darryl, Hu William, Chan Denise, Becker Werner J
Abstract A literature search found no clinical trials or guidelines addressing the management of spontaneous intracranial hypotension (SIH). Based on the available literature and expert opinion, we have developed recommendations for the diagnosis and management of SIH. For typical cases, we recommend brain magnetic resonance (MR) imaging with gadolinium to confirm the diagnosis, and conservative measures for up to two weeks. If the patient remains symptomatic, up to three non-directed lumbar epidural blood patches (EBPs) should be considered. If these are unsuccessful, non-invasive MR myelography, radionuclide cisternography, MR myelography with intrathecal gadolinium, or computed tomography with myelography should be used to localize the leak. If the leak is localized, directed EPBs should be considered, followed by fibrin sealant or neurosurgery if necessary. Clinically atypical cases with normal brain MR imaging should be investigated to localize the leak. Directed EBPs can be used if the leak is localized; non-directed EBPs should be used only if there are indirect signs of SIH.
Article ID(s) 23419561 (PubMed)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous low pressure, low CSF volume headaches: spontaneous CSF leaks.
Publication Headache 2013 Jul-Aug; 53(7) (Publication year: 2013)
Author(s) Mokri Bahram
Abstract Spontaneous intracranial hypotension typically results from spontaneous cerebrospinal fluid (CSF) leak, often at spine level and only rarely from skull base. Once considered rare, it is now diagnosed far more commonly than before and is recognized as an important cause of headaches. CSF leak leads to loss of CSF volume. Considering that the skull is a rigid noncollapsible container, loss of CSF volume is typically compensated by subdural fluid collections and by increase in intracranial venous blood which, in turn, causes pachymeningeal thickening, enlarged pituitary, and engorgement of cerebral venous sinuses on magnetic resonance imaging (MRI). Another consequence of CSF hypovolemia is sinking of the brain, with descent of the cerebellar tonsils and brainstem as well as crowding of the posterior fossa noted on head MRI. The clinical consequences of these changes include headaches that are often but not always orthostatic, nausea, occasional emesis, neck and interscapular pain, cochleovestibular manifestations, cranial nerve palsies, and several other manifestations attributed to pressure upon or stretching of the cranial nerves or brain or brainstem structures. CSF lymphocytic pleocytosis or increase in CSF protein concentration is not uncommon. CSF opening pressure is often low but can be within normal limits. Stigmata of disorders of connective tissue matrix are seen in some of the patients. An epidural blood patch, once or more, targeted or distant, at one site or bilevel, has emerged as the treatment of choice for those who have failed the conservative measures. Epidural injection of fibrin glue of both blood and fibrin glue can be considered in selected cases. Surgery to stop the leak is considered when the exact site of the leak has been determined by neurodiagnostic studies and when less invasive measures have failed. Subdural hematomas sometimes complicate the CSF leaks; a rebound intracranial hypertension after successful treatment of a leak is not rare. Cerebral venous sinus thrombosis as a complication is fortunately less common, and superficial siderosis and bibrachial amyotrophy are rare. Short-term recurrences are not uncommon, and long-term recurrences are not rare.
Article ID(s) 23808630 (PubMed)
10.1111/head.12149 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Stroke and death due to spontaneous intracranial hypotension.
Publication Neurocritical care 2013 Apr; 18(2) (Publication year: 2013)
Author(s) Schievink Wouter I
Abstract BACKGROUND
Spontaneous intracranial hypotension has become a well-recognized cause of headaches and a wide variety of other manifestations have been reported. Recently, several patients with asymptomatic spontaneous intracranial hypotension were reported. I now report two patients with spontaneous intracranial hypotension who developed multiple arterial strokes associated with death in one patient, illustrating the spectrum of disease severity in spontaneous intracranial hypotension.

METHODS
Medical records and radiologic imaging of the two patients were reviewed.

RESULTS
Case 1. A 45-year-old man presented with an orthostatic headache. Neurologic examination was normal. MRI showed bilateral subdural fluid collections, brain sagging, and pachymeningeal enhancement. At lumbar puncture, the opening pressure was too low to record. He underwent two epidural blood patches with transient improvement of symptoms. His headaches progressed and a CT-myelogram showed a lower cervical CSF leak. Subsequently, periodic lethargy and confusion was noted and he then rapidly deteriorated. Examination showed coma (GCS: 4 [E1, M2, V1]), a fixed and dilated right pupil, and decerebrate posturing. Bilateral craniotomies were performed for the evacuation of chronic subdural hematomas. Immediate postoperative CT showed bilateral posterior cerebral artery infarcts and a recurrent right subdural hematoma, requiring re-evacuation. Postoperative examination was consistent with brain death and support was withdrawn.  Case 2. A 42-year-old man presented with a non-positional headache. Neurologic examination was normal. CT showed bilateral acute on chronic subdural hematomas and effacement of the basilar cisterns. MRI showed brain sagging, bilateral subdural hematomas, and pachymeningeal enhancement. Bilateral craniotomies were performed and subdural hematomas were evacuated. Postoperatively, the patient became progressively lethargic (GCS: 8 [E2, M4, V2]) and variable degrees of pupillary asymmetry and quadriparesis were noted. MRI now also showed multiple areas of restricted diffusion in the pons and midbrain, consistent with multiple infarcts. CT showed worsening subdural fluid collections with midline shift and increased effacement of the basilar cisterns. Repeat bilateral craniotomies were performed for evacuation of the subdural fluid collections. Neurologic examination was then noted to be fluctuating but clearly improved when lying flat (GCS: 10T [E4, M6, VT]). CT-myelography demonstrated an extensive cervico-thoracic CSF leak. An epidural blood patch was performed. The patient made a good, but incomplete, recovery with residual quadriparesis and dysphagia.

CONCLUSIONS
Arterial cerebral infarcts are rare, but potentially life-threatening complications of spontaneous intracranial hypotension. The strokes are due to downward displacement of the brain and can be precipitated by craniotomy for evacuation of associated subdural hematomas.
Article ID(s) 23196352 (PubMed)
10.1007/s12028-012-9800-3 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Ventral spinal cerebrospinal fluid leak as the cause of persistent post-dural puncture headache in children.
Publication Journal of neurosurgery. Pediatrics 2013 Jan; 11(1) (Publication year: 2013)
Author(s) Schievink Wouter I, Maya M Marcel
Abstract Headache occurs after dural puncture in about 1%-25% of children who undergo the procedure-a rate similar to that seen in adults. Persistence of post-dural puncture headache in spite of bed rest, increased fluid intake, and epidural blood patch treatment, however, is rare. The authors reviewed the medical records and imaging studies of all patients 19 years of age or younger who they evaluated between 2001 and 2010 for intracranial hypotension, and they identified 8 children who had persistent post-dural puncture headache despite maximal medical treatment and placement of epidural blood patches. A CSF leak could be demonstrated radiologically and treated surgically in 3 of these patients, and the authors report these 3 cases. The patients were 2 girls (ages 14 and 16 years) who had undergone lumbar puncture for evaluation of headache and fever and 1 boy (age 13 years) who had undergone placement of a lumboperitoneal shunt using a Tuohy needle for treatment of pseudotumor cerebri. The boy also had undergone a laminectomy and exploration of the posterior dural sac, but no CSF leak could be identified. All 3 patients presented with new-onset orthostatic headaches, and in all 3 cases MRI demonstrated a large ventral lumbar or thoracolumbar CSF collection. Conventional myelography or digital subtraction myelography revealed a ventral dural defect at L2-3 requiring surgical repair. Through a posterior transdural approach, the dural defect was repaired using 6-0 Prolene sutures and a dural substitute. Postoperative recovery was uneventful, with complete resolution of orthostatic headache and of the ventral cerebrospinal fluid leak on MRI. The authors conclude that persistent postdural puncture headache requiring surgical repair is rare in children. They note that the CSF leak may be located ventrally and may require conventional or digital subtraction myelography for exact localization and that transdural repair is safe and effective in eliminating the headaches.
Article ID(s) 23140214 (PubMed)
10.3171/2012.10.PEDS12353 (DOI)
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2012



Article in Favourites List Article not in Must-Read List  CT myelography for the planning and guidance of targeted epidural blood patches in patients with persistent spinal CSF leakage.
Publication AJNR. American journal of neuroradiology 2012 Mar; 33(3) (Publication year: 2012)
Author(s) Wendl C M, Schambach F, Zimmer C, Förschler A
Abstract Intracranial hypotension is a rare cause of persistent headache mostly originating from a dural CSF leak. If a conservative treatment fails, a minimally invasive EBP can lead to a successful sealing of such a leak. Independent of the leakage site, an EBP is usually applied at the lumbar level with varying success. We used CT myelography to detect the site of the dural leakage, then immediately applied a targeted EBP at the corresponding level to patch the leak. Seven patients from our clinic were treated with a single targeted EBP in the lumbar or cervical spine. Within 24 hours, 6 patients experienced a considerable relief of symptoms; 1 patient went into remission after a repeat procedure. Our preliminary data suggest that a CT-guided, CT myelography-assisted targeted EBP is a safe and effective treatment for persistent spinal CSF leaks.
Article ID(s) 22194376 (PubMed)
10.3174/ajnr.A2808 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Intracranial hypotension producing reversible coma: a systematic review, including three new cases.
Publication Journal of neurosurgery 2012 Sep; 117(3) (Publication year: 2012)
Author(s) Loya Joshua J, Mindea Stefan A, Yu Hong, Venkatasubramanian Chitra, Chang Steven D, Burns Terry C
Abstract Intracranial hypotension is a disorder of CSF hypovolemia due to iatrogenic or spontaneous spinal CSF leakage. Rarely, positional headaches may progress to coma, with frequent misdiagnosis. The authors review reported cases of verified intracranial hypotension-associated coma, including 3 previously unpublished cases, totaling 29. Most patients presented with headache prior to neurological deterioration, with positional symptoms elicited in almost half. Eight patients had recently undergone a spinal procedure such as lumbar drainage. Diagnostic workup almost always began with a head CT scan. Subdural collections were present in 86%; however, intracranial hypotension was frequently unrecognized as the underlying cause. Twelve patients underwent one or more procedures to evacuate the collections, sometimes with transiently improved mental status. However, no patient experienced lasting neurological improvement after subdural fluid evacuation alone, and some deteriorated further. Intracranial hypotension was diagnosed in most patients via MRI studies, which were often obtained due to failure to improve after subdural hematoma (SDH) evacuation. Once the diagnosis of intracranial hypotension was made, placement of epidural blood patches was curative in 85% of patients. Twenty-seven patients (93%) experienced favorable outcomes after diagnosis and treatment; 1 patient died, and 1 patient had a morbid outcome secondary to duret hemorrhages. The literature review revealed that numerous additional patients with clinical histories consistent with intracranial hypotension but no radiological confirmation developed SDH following a spinal procedure. Several such patients experienced poor outcomes, and there were multiple deaths. To facilitate recognition of this treatable but potentially life-threatening condition, the authors propose criteria that should prompt intracranial hypotension workup in the comatose patient and present a stepwise management algorithm to guide the appropriate diagnosis and treatment of these patients.
Article ID(s) 22725982 (PubMed)
10.3171/2012.4.JNS112030 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Lack of causal association between spontaneous intracranial hypotension and cranial cerebrospinal fluid leaks.
Publication Journal of neurosurgery 2012 Apr; 116(4) (Publication year: 2012)
Author(s) Schievink Wouter I, Schwartz Marc S, Maya M Marcel, Moser Franklin G, Rozen Todd D
Abstract OBJECT
Spontaneous intracranial hypotension is an important cause of headaches and an underlying spinal CSF leak can be demonstrated in most patients. Whether CSF leaks at the level of the skull base can cause spontaneous intracranial hypotension remains a matter of controversy. The authors' aim was to examine the frequency of skull base CSF leaks as the cause of spontaneous intracranial hypotension.

METHODS
Demographic, clinical, and radiological data were collected from a consecutive group of patients evaluated for spontaneous intracranial hypotension during a 9-year period.

RESULTS
Among 273 patients who met the diagnostic criteria for spontaneous intracranial hypotension and 42 who did not, not a single instance of CSF leak at the skull base was encountered. Clear nasal drainage was reported by 41 patients, but a diagnosis of CSF rhinorrhea could not be established. Four patients underwent exploratory surgery for presumed CSF rhinorrhea. In addition, the authors treated 3 patients who had a postoperative CSF leak at the skull base following the resection of a cerebellopontine angle tumor and developed orthostatic headaches; spinal imaging, however, demonstrated the presence of a spinal source of CSF leakage in all 3 patients.

CONCLUSIONS
There is no evidence for an association between spontaneous intracranial hypotension and CSF leaks at the level of the skull base. Moreover, the authors' study suggests that a spinal source for CSF leakage should even be suspected in patients with orthostatic headaches who have a documented skull base CSF leak.
Article ID(s) 22264184 (PubMed)
10.3171/2011.12.JNS111474 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Neuroimaging and other investigations in patients presenting with headache.
Publication Annals of Indian Academy of Neurology 2012 Aug; 15(Suppl 1) (Publication year: 2012)
Author(s) Duncan Callum W
Abstract Headache is very common. In the United Kingdom, it accounts for 4.4% of primary care consultations, 30% of referrals to neurology services and 0.5-0.8% of alert patients presenting to emergency departments. Primary headache disorders account for the majority of patients and most patients do not require investigation. Warning features (red flags) in the history and on examination help target those who need investigation and what investigations are required. This article summarizes the typical presentations of the common secondary headaches and what neuroimaging and other investigations are appropriate for each headache type.
Article ID(s) 23024561 (PubMed)
10.4103/0972-2327.99995 (DOI)
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Article in Favourites List Article not in Must-Read List  Second-half-of-the-day headache as a manifestation of spontaneous CSF leak.
Publication Journal of neurology 2012 Feb; 259(2) (Publication year: 2012)
Author(s) Leep Hunderfund Andrea N, Mokri Bahram
Abstract Orthostatic headache related to spontaneous cerebrospinal fluid leak (CSF) appears within 2 h of sitting or standing in most patients. However, longer delays to headache onset have been observed, including some patients who have headaches only in the afternoon. The objective of this study is to describe second-half-of-the-day headache as a manifestation of spontaneous CSF leak and propose potential mechanisms. From 142 patients evaluated by one of us (B.M.) during a 10-year period for spontaneous intracranial hypotension, those describing headache occurring exclusively in the afternoon accompanied by typical changes of intracranial hypotension on head MRI were retrospectively identified and their medical records reviewed. Five patients met our pre-defined inclusion criteria (5/142, 3.5%; three women; mean age 50 years). Second-half-of-the-day headache was an initial symptom of intracranial hypotension in one patient, spontaneously evolved from prior all-day orthostatic headache in one patient, and was a residual or recurrent symptom after epidural blood patch in three patients. Head MRI changes due to intracranial hypotension were decreased during second-half-of-the-day-headache compared to typical all-day orthostatic headache in three out of four patients. The timing of second-half-of-the-day headache and orthostatic headache in the clinical course of patients with spontaneous CSF leaks and related MRI findings suggest that second-half-of-the-day headache is likely a manifestation of a slowed or slow-flow CSF leak.
Article ID(s) 21811806 (PubMed)
10.1007/s00415-011-6181-z (DOI)
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Article Not in Favourites List Article not in Must-Read List  Supine digital subtraction myelography for the demonstration of a dorsal cerebrospinal fluid leak in a patient with spontaneous intracranial hypotension: a technical note.
Publication Journal of radiology case reports 2012 Sep; 6(9) (Publication year: 2012)
Author(s) Carstensen Michael, Chaudhary Navjot, Leung Andrew, Ng Wai
Abstract A patient with spontaneous intracranial hypotension due to a spinal cerebrospinal fluid (CSF) leak required localization of the leakage site prior to surgical management. Conventional, computed tomography and prone digital subtraction myelography failed to localize the dural tear, which was postulated to be dorsally located. We present here a digital subtraction myelographic approach to accurately localize a dorsal site of CSF leakage by injecting iodinated contrast via a lumbar drain with the patient in the supine position.
Article ID(s) 23378882 (PubMed)
10.3941/jrcr.v6i9.1002 (DOI)
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Article in Favourites List Article is Must-Read  The role of digital subtraction myelography in the diagnosis and localization of spontaneous spinal CSF leaks.
Publication AJR. American journal of roentgenology 2012 Sep; 199(3) (Publication year: 2012)
Author(s) Hoxworth Joseph M, Trentman Terrence L, Kotsenas Amy L, Thielen Kent R, Nelson Kent D, Dodick David W
Abstract OBJECTIVE
The objective of our study was to review the clinical utility of digital subtraction myelography for the diagnosis of spinal CSF leaks in patients with spontaneous intracranial hypotension (SIH) and those with superficial siderosis.

MATERIALS AND METHODS
Procedure logs from 2007 to 2011 were reviewed to identify cases in which digital subtraction myelography was performed to diagnose spinal CSF leaks. Electronic medical records were reviewed to obtain information regarding diagnosis and outcome. For patients to be included in the study, preprocedural spinal MRI had to show an extradural fluid collection spanning more than one vertebral level and postmyelographic CT had to confirm the presence of an active CSF leak. If digital subtraction myelography successfully showed the site of the CSF leak, the location was documented.

RESULTS
Eleven patients (seven men and four women; mean age, 49.0 years) underwent digital subtraction myelography during the study period. Six patients had SIH and five patients had superficial siderosis. The extradural fluid collection on spinal MRI averaged a length of 15.5 vertebral levels. Digital subtraction myelography successfully showed the site of the CSF leak in nine of the 11 patients, and all of the dural tears were located in the thoracic spine between T3 and T11.

CONCLUSION
Digital subtraction myelography is a valuable diagnostic tool for the localization of rapid spinal CSF leaks and should be considered in patients who are clinically suspected to have a dural tear that is accompanied by a longitudinally extensive extradural fluid collection on spinal MRI.
Article ID(s) 22915407 (PubMed)
10.2214/AJR.11.8238 (DOI)
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Article in Favourites List Article not in Must-Read List  The role of MR myelography with intrathecal gadolinium in localization of spinal CSF leaks in patients with spontaneous intracranial hypotension.
Publication AJNR. American journal of neuroradiology 2012 Mar; 33(3) (Publication year: 2012)
Author(s) Akbar J J, Luetmer P H, Schwartz K M, Hunt C H, Diehn F E, Eckel L J
Abstract BACKGROUND AND PURPOSE
Localization of spinal CSF leaks in CSF hypovolemia is critical in directing focal therapy. In this retrospective review, our aim was to determine whether GdM was helpful in confirming and localizing spinal CSF leaks in patients in whom no leak was identified on a prior CTM.

MATERIALS AND METHODS
Forty-one symptomatic patients with clinical suspicion of SIH were referred for GdM after undergoing at least 1 CTM between February 2002 and August 2010. A retrospective review of the imaging and electronic medical records was performed on each patient.

RESULTS
In 17 of the 41 patients (41%), GdM was performed for follow-up of a previously documented leak at CTM. In the remaining 24 patients (59%), in whom GdM was performed for a suspected CSF leak, which was not identified on CTM, GdM localized the CSF leak in 5 of 24 patients (21%). In 1 of these 5 patients, GdM detected the site of leak despite negative findings on brain MR imaging, spine MR imaging, and CTM of the entire spine. Sixteen of 17 patients with previously identified leaks underwent interval treatment, and leaks were again identified in 12 of 17 (71%).

CONCLUSIONS
GdM is a useful technique in the highly select group of patients who have debilitating symptoms of SIH, a high clinical index of suspicion of spinal CSF leak, and no demonstrated leak on conventional CTM. Intrathecal injection of gadolinium contrast remains an off-label use and should be reserved for those patients who fail conventional CTM.
Article ID(s) 22173753 (PubMed)
10.3174/ajnr.A2815 (DOI)
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Article Not in Favourites List Article not in Must-Read List  The Value of Changing Position in the Detection of CSF Leakage in Spontaneous Intracranial Hypotension Using Tc-99m DTPA Scintigraphy: Two Case Reports.
Publication Iranian journal of radiology : a quarterly journal published by the Iranian Radiological Society 2012 Sep; 9(3) (Publication year: 2012)
Author(s) Lu Yu Yu, Wang Hsin Yi, Lin Ying, Lin Wan Yu
Abstract Radionuclide Cisternography (RNC) is of potential value in pointing out the sites of cerebrospinal fluid (CSF) leakage in patients with spontaneous intracranial hypotension (SIH). In the current report, we present two patients who underwent RNC for suspected CSF leakage. Both patients underwent magnetic resonance imaging (MRI) and RNC for evaluation. We describe a simple method to increase the detection ability of RNC for CSF leakage in patients with SIH.
Article ID(s) 23329981 (PubMed)
10.5812/iranjradiol.7956 (DOI)
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Article Not in Favourites List Article not in Must-Read List  When should I do dynamic CT myelography? Predicting fast spinal CSF leaks in patients with spontaneous intracranial hypotension.
Publication AJNR. American journal of neuroradiology 2012 Apr; 33(4) (Publication year: 2012)
Author(s) Luetmer P H, Schwartz K M, Eckel L J, Hunt C H, Carter R E, Diehn F E
Abstract BACKGROUND AND PURPOSE
Some patients with SIH have fast CSF leaks requiring dynamic CTM for localization; however, patients generally undergo conventional CTM before a dynamic study. Our aim was to determine whether findings on head MR imaging, spine MR imaging, or opening pressure measurements can predict fast spinal CSF leaks.

MATERIALS AND METHODS
A retrospective review was performed on 151 consecutive patients referred for CTM to evaluate for spinal CSF leak. Head MR imaging was evaluated for diffuse dural enhancement and "brain sag," and spine MR imaging for presence of an extradural fluid collection. The opening pressure was recorded. The CTM was scored as no leak, slow leak localized on conventional CTM, or fast leak that required dynamic CTM.

RESULTS
Fast CSF leaks were identified in 32 (21%), slow leaks in 36 (24%), and no leak in 83 (55%) of 151 patients on initial CTM. There was significant association between spinal extra-arachnoid fluid on MR imaging and the presence of a fast leak (sensitivity 85%, specificity 79%, P < .0001). There was not significant association between fast leak and findings on head MR imaging (P = .27) or opening pressure (P = .30).

CONCLUSIONS
If all patients with spinal extra-arachnoid CSF on MR imaging had been sent directly to dynamic CTM, repeat myelography would have been avoided in most patients with fast leaks (23 of 27; 85%). However, a minority of patients with slow or no leaks would have been converted from conventional to dynamic CTM (16 of 77; 21%). Spinal MR imaging is helpful in premyelographic evaluation of SIH.
Article ID(s) 22194380 (PubMed)
10.3174/ajnr.A2849 (DOI)
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2011



Article Not in Favourites List Article not in Must-Read List  Cerebrospinal fluid leakage--reliable diagnostic methods.
Publication Clinica chimica acta; international journal of clinical chemistry 2011 May 12; 412(11-12) (Publication year: 2011)
Author(s) Mantur Maria, Łukaszewicz-Zając Marta, Mroczko Barbara, Kułakowska Alina, Ganslandt Oliver, Kemona Halina, Szmitkowski Maciej, Drozdowski Wiesław, Zimmermann Rüdiger, Kornhuber Johannes, Lewczuk Piotr
Abstract Prompt diagnosis and early treatment of cerebrospinal fluid (CSF) leakage minimizes the risk of severe complications. In patients presenting with clear fluid nasal discharge it is important to identify the nature of the rhinorrhea. The CSF leakage may occur as post-traumatic, iatrogenic, spontaneous or idiopathic rhinorrhea. The differential diagnosis of CSF rhinorrhea often presents a challenging problem. The confirmation of CSF rhinorrhea and localization of the leakage may be diagnosed by CT, MRI cisternography and MRI cisternography in combination with single photon emission tomography or radioisotopic imaging. Although these methods allow estimation of the CSF leakage with high accuracy, they are expensive and invasive procedures. Therefore, biochemical methods are still used in the differentiation. Although the most common diagnostic method for screening CSF leakage is glucose oxidase, its diagnostic sensitivity and specificity is generally unsatisfactory. False negative results may occur with bacterial contamination and false positive results are common in diabetic patients. Glucose detection is not recommended as a confirmatory test. As such, other biomarkers of the CSF leakage, such as beta-2-transferrin (beta-2 trf) and beta-trace protein (betaTP) are necessary to identify and confirm of this condition.
Article ID(s) 21334321 (PubMed)
10.1016/j.cca.2011.02.017 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Chronic cerebellar hemorrhage in spontaneous intracranial hypotension: association with ventral spinal cerebrospinal fluid leaks: clinical article.
Publication Journal of neurosurgery. Spine 2011 Oct; 15(4) (Publication year: 2011)
Author(s) Schievink Wouter I, Maya M Marcel, Nuño Miriam
Abstract OBJECT
Spontaneous intracranial hypotension is an important cause of new-onset daily persistent headache. Cerebellar hemorrhage has been identified as a possible feature of spontaneous intracranial hypotension. The authors reviewed the MR imaging studies from a group of patients with spontaneous intracranial hypotension to assess the presence of cerebellar hemorrhage.

METHODS
Medical records and radiological images were reviewed in 262 cases involving patients with spontaneous intracranial hypotension who had undergone MR imaging of the brain as well as spinal imaging.

RESULTS
Chronic cerebellar hemorrhages were found in 7 (2.7%) of the 262 patients with spontaneous intracranial hypotension. These hemorrhages were found in 7 (19.4%) of the 36 patients with a ventral spinal CSF leak and in none of the 226 patients who did not have such a CSF leak (p < 0.0001). The degree of hemosiderin deposits was variable, ranging from mild involvement of the cerebellar folia to widespread superficial siderosis. Only the 1 patient with superficial siderosis had symptoms due to the hemorrhages. The time period between the onset of symptoms due to spontaneous intracranial hypotension and MR imaging examination was significantly longer in those patients with cerebellar hemorrhage than in those with a ventral spinal CSF leak and no evidence for cerebellar hemorrhage (mean 19.6 years vs 2.3 months, p < 0.0001).

CONCLUSIONS
Chronic cerebellar hemorrhage should be included among the manifestations of spontaneous intracranial hypotension. The severity is variable, but the hemorrhage generally is asymptomatic. The underlying spinal CSF leak is ventral and mostly of long duration.
Article ID(s) 21740128 (PubMed)
10.3171/2011.5.SPINE10890 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Connective tissue disorders in patients with spontaneous intracranial hypotension.
Publication Cephalalgia : an international journal of headache 2011 Apr; 31(6) (Publication year: 2011)
Author(s) Liu Fang-Chun, Fuh Jong-Ling, Wang Yen-Feng, Wang Shuu-Jiun
Abstract OBJECTIVE
Spontaneous intracranial hypotension (SIH) is caused by spinal cerebrospinal fluid (CSF) leakage. An underlying connective tissue disorder has been hypothesized to cause dural weakness and predisposition to CSF leak. We conducted a case-controlled study to investigate the role of connective tissue disorders in SIH patients.

METHODS
We recruited 55 consecutive SIH patients (38 F, 17 M; mean age, 40.8 ± 9.8 years) and 55 age- and sex-matched control individuals (mean age, 38.0 ± 8.9 years) for this study. The connective tissue disorders were evaluated by: (i) Beighton hypermobility scores and revised diagnostic criteria for benign joint hypermobility syndrome; (ii) skin and skeletal manifestations of Ehlers-Danlos syndrome (EDS); and (iii) skeletal features of Marfan syndrome.

RESULTS
The frequencies of joint hypermobility according to Beighton scores >4/9 (SIH 23.6% vs controls 16.4%, P = 0.48) and revised benign joint hypermobility syndrome criteria (SIH 23.6% vs controls 34.5%, P = 0.29) did not differ between SIH patients and controls. Sixteen patients and 16 controls had one or more skin features of EDS (P = 1.0). Nine SIH patients (16.4%) demonstrated the skeletal features of Marfan syndrome; this frequency did not differ from that of the control group (9.1%; P = 0.262). Only dolichostenomelia (disproportionately long limbs) was more prominent in SIH patients than in controls (34.5% vs 9.1%; P = 0.002).

CONCLUSION
Compared with Western studies, the frequencies of connective tissue disorders were higher in our SIH patients. However, these frequencies did not differ between SIH patients and control individuals, except for dolichostenomelia.
Article ID(s) 21220378 (PubMed)
10.1177/0333102410394676 (DOI)
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Article in Favourites List Article is Must-Read  CT-guided epidural blood patching of directly observed or potential leak sites for the targeted treatment of spontaneous intracranial hypotension.
Publication AJNR. American journal of neuroradiology 2011 May; 32(5) (Publication year: 2011)
Author(s) Kranz P G, Gray L, Taylor J N
Abstract BACKGROUND AND PURPOSE
Optimal diagnosis and management of spontaneous intracranial hypotension remains uncertain. CT-guided blood patching has been described but has not been evaluated in larger case series. We sought to evaluate the efficacy of CT-guided blood patching of observed or potential CSF leaks in spontaneous intracranial hypotension.

MATERIALS AND METHODS
Patients referred for evaluation of spontaneous intracranial hypotension were retrospectively reviewed. Inclusion criteria were findings of intracranial hypotension on pretreatment brain MR imaging, evaluation and treatment with CT-guided myelography and blood patching, and availability of posttreatment brain MR images. Eight patients met inclusion criteria. Imaging findings, treatment details, and clinical outcomes were assessed.

RESULTS
Pretreatment imaging findings included dural enhancement, tonsillar ectopia, subdural collections, and syrinx. All findings resolved or significantly improved on posttreatment imaging. Presenting clinical symptoms included positional headache, neck/interscapular/shoulder pain, and tinnitus. Headaches and neck/interscapular/shoulder pain improved in all patients; tinnitus improved in 1 of 2 patients. CSF leak sites were directly visualized in 37% of patients and were targets for patching when seen. When no direct visualization of leaks was seen, irregular spinal nerve root diverticula were targeted as potential leak sites. The average number of blood patching sessions was 3 (range, 1-6) and the average number of individual sites patched per session was 5 (range, 1-10).

CONCLUSIONS
Our results suggest that CT-guided blood patching targeting observed or potential leak sites can be effective in the treatment of intracranial hypotension. Prospective controlled studies are needed to confirm efficacy and compare outcomes with other treatment options.
Article ID(s) 21349964 (PubMed)
10.3174/ajnr.A2384 (DOI)
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Article in Favourites List Article is Must-Read  Diagnostic criteria for headache due to spontaneous intracranial hypotension: a perspective.
Publication Headache 2011 Oct; 51(9) (Publication year: 2011)
Author(s) Schievink Wouter I, Dodick David W, Mokri Bahram, Silberstein Stephen, Bousser Marie-Germaine, Goadsby Peter J
Abstract The clinical and radiographic manifestations of spontaneous intracranial hypotension are highly variable and many patients do not satisfy the 2004 International Classification of Headache Disorders criteria. We developed new diagnostic criteria for spontaneous intracranial hypotension based on cases we have seen reflecting the variable manifestations of the disorder. These criteria provide a basis for change when the classification criteria are next revised. The diagnostic criteria consist of A, orthostatic headache; B, the presence of at least one of the following: low opening pressure (≤ 60 mm H(2) O), sustained improvement of symptoms after epidural blood patching, demonstration of an active spinal cerebrospinal fluid leak, cranial magnetic resonance imaging changes of intracranial hypotension (eg, brain sagging or pachymeningeal enhancement); C, no recent history of dural puncture; and D, not attributable to another disorder.
Article ID(s) 21658029 (PubMed)
10.1111/j.1526-4610.2011.01911.x (DOI)
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Article Not in Favourites List Article not in Must-Read List  Frequency of intracranial aneurysms in patients with spontaneous intracranial hypotension.
Publication Journal of neurosurgery 2011 Jul; 115(1) (Publication year: 2011)
Author(s) Schievink Wouter I, Maya M Marcel
Abstract OBJECT
Spontaneous intracranial hypotension (SIH) is a significant cause of new-onset daily persistent headache. A generalized connective tissue disorder also involving the intracranial arteries has been suspected in the population with SIH. Therefore, the authors reviewed angiographic studies for the presence of intracranial aneurysms in a group of patients with SIH.

METHODS
Magnetic resonance angiography studies of the brain were performed in 93 patients with SIH (mean age 43 years, range 14-86 years) and in 291 controls (mean age 56 years, range 28-78 years).

RESULTS
Intracranial aneurysms were detected in 8 (8.6%) of the 93 patients with SIH (95% CI 2.9%-14.3%). This incidence was higher than in the control population (3 (1.0%) of 291 (95% CI 0%-2.2%; p = 0.0007). In 7 patients the aneurysms were incidental, and in 1 patient SIH developed 5 weeks after an aneurysmal subarachnoid hemorrhage.

CONCLUSIONS
In this retrospective case-control study, the frequency of intracranial aneurysms among patients with SIH was significantly higher than in the control population.
Article ID(s) 21395391 (PubMed)
10.3171/2011.2.JNS101805 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Frontotemporal brain sagging syndrome: an SIH-like presentation mimicking FTD.
Publication Neurology 2011 Apr 19; 76(16) (Publication year: 2011)
Author(s) Wicklund M R, Mokri B, Drubach D A, Boeve B F, Parisi J E, Josephs K A
Abstract BACKGROUND
Behavioral variant frontotemporal dementia (bvFTD) is a relatively well-defined clinical syndrome. It is associated with frontal and temporal lobe structural/metabolic changes and pathologic findings of a neurodegenerative disease. We have been evaluating patients with clinical and imaging features partially consistent with bvFTD but with evidence also suggestive of brain sagging, which we refer to as frontotemporal brain sagging syndrome (FBSS).

METHODS
Retrospective medical chart review to identify all patients seen at our institution between 1996 and 2010, who had a clinical diagnosis of FTD and imaging evidence of brain sag.

RESULTS
Eight patients, 7 male and 1 female, were diagnosed with FBSS. The median age at symptom onset was 53 years. All patients had insidious onset and slow progression of behavioral and cognitive dysfunction accompanied by daytime somnolence and headache. Of the 5 patients with functional imaging, all showed evidence of hypometabolism of the frontotemporal regions. On brain MRI, all patients had evidence of brain sagging with distortion of the brainstem; 3 patients had diffuse pachymeningeal enhancement. CSF opening pressure was varied and CSF protein was mildly elevated. A definite site of CSF leak was not identified by myelogram or cisternography, except in one patient with a site highly suggestive of leak who subsequently underwent surgery confirming a CSF leak. In 2 patients with a neuropathologic examination, there was no evidence of a neurodegenerative disease.

CONCLUSIONS
This case series demonstrates that FBSS may mimic typical bvFTD but should be recognized as an unusual presentation that is potentially treatable.
Article ID(s) 21502595 (PubMed)
10.1212/WNL.0b013e3182166e42 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous CSF Leaks.
Publication Otolaryngologic clinics of North America 2011 Aug; 44(4) (Publication year: 2011)
Author(s) Wang Eric W, Vandergrift William Alex, Schlosser Rodney J
Abstract Spontaneous cerebrospinal fluid rhinorrhea represents a distinct clinic entity that is likely a variant of idiopathic intracranial hypertension (IIH). Patients with spontaneous cerebrospinal fluid (CSF) leaks are generally middle-aged obese women with radiographic evidence of skull base defects, associated meningoencephaloceles, and empty sella syndrome, a common sign of increased intracranial pressure. Significant overlap exists in the characteristics of patients with spontaneous CSF leak and IIH. Endoscopic repair of the CSF fistula is the gold standard treatment for this condition, but emerging evidence supports the reduction of CSF pressure as an important adjuvant treatment in this patient population.
Article ID(s) 21819875 (PubMed)
10.1016/j.otc.2011.06.018 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Traumatic, iatrogenic, and spontaneous cerebrospinal fluid (CSF) leak: endoscopic repair.
Publication B-ENT 2011; 7 Suppl 17 (Publication year: 2011)
Author(s) Daele J J M, Goffart Y, Machiels S
Abstract Over the past two decades, Cerebrospinal Fluid (CSF) leak repair has advanced from open invasive intracranial approaches to transnasal endoscopic ones that avoid the traditional morbidities of frontal craniotomy approaches--such as anosmia, intracranial haemorrhage or oedema, seizures, memory deficiencies, and behaviour disorders--reducing morbidity, reducing hospitalisation times and accelerating return to work, and therefore cutting indirect costs. The diagnosis of CSF rhinorrhoea is both clinical and radiological. The presence of CSF in clear nasal drainage should be established by analysis for CSF markers. Localisation of the leak site involves radiological investigation, mainly Computerised Tomography (CT) and Magnetic Resonance Imaging (MRI). In addition to suppressing symptoms, the main goal of the closure of CSF rhinorrhoea is to prevent ascending meningitis. The operative management of cerebrospinal fluid leak is advised in the following circumstances: persistent, posttraumatic CSF leaks after 4 to 6 weeks of conservative treatment; all cases of spontaneous CSF fistulae; cases with intermittent leaks; delayed posttraumatic leaks; cases of CSF leak with a history of meningitis; false CSF rhinorrhoea coming from the petrous bone via the Eustachian tube. The graft material used depends mainly on the authors' experience and did not significantly influence the success rate. The main steps in the surgical procedures do not differ as much from one author to the other: accurate localisation of the defect; creation of a raw surface around the defect to accept the graft and to help in the formation of synechiae to support the seal later; plugging of the defect with fat covered with fascia lata supported by absorbable gelatin and Merocel. The differences between the authors relate to the use of fluorescein to locate the defect, the importance of prophylactic antibiotherapy, the plugging materials, the technique of underlay or overlay grafting, the use of fibrin glue and the need for lumbar drainage. The success rate for endoscopic repair of CSF rhinorrhoea is high: approximately 90% at the first attempt. Recent reports in the literature highlight the group of patients with spontaneous idiopathic CSF leak as a group with specific attributes and treatment challenges.
Article ID(s) 22338375 (PubMed)
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2010



Article in Favourites List Article not in Must-Read List  Epidural blood patch in Trendelenburg position pre-medicated with acetazolamide to treat spontaneous intracranial hypotension.
Publication European journal of neurology 2010 May; 17(5) (Publication year: 2010)
Author(s) Ferrante E, Arpino I, Citterio A, Wetzl R, Savino A
Abstract BACKGROUND
Spontaneous intracranial hypotension (SIH) is characterized by orthostatic headache, diffuse pachymeningeal enhancement on brain magnetic resonance imaging (MRI) and low cerebrospinal fluid (CSF) pressure. Treatment ranges from conservative management, such as bed rest, overhydration and caffeine, to invasive procedures, such as the autologous epidural blood patch (EBP), computed tomography (CT)-guided fibrin glue injection at the site of the leak and open surgical intervention. EBP has emerged as the treatment of choice for SIH when initial conservative measures fail to bring relief.

METHODS
Forty-two patients with SIH were treated with lumbar autologous EBP in Trendelenburg position preceded by pre-medication with acetazolamide.

RESULTS
A complete recovery was obtained in all patients after one (90%), two (5%) or three (5%) EBPs. After EBP, two patients (5%) also performed evacuation of bilateral chronic subdural hematoma with mass effect.

CONCLUSIONS
Spontaneous intracranial hypotension can be effectively cured by lumbar autologous EBP in Trendelenburg position pre-medicated with acetazolamide.
Article ID(s) 20050898 (PubMed)
10.1111/j.1468-1331.2009.02913.x (DOI)
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Article Not in Favourites List Article not in Must-Read List  Postural tremor as a manifestation of spontaneous intracranial hypotension.
Publication Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2010 Feb; 17(2) (Publication year: 2010)
Author(s) Turgut Nilda, Unlü Ercüment, Hamamcioğlu Mustafa Kemal, Güldiken Babürhan, Albayram Sait
Abstract Spontaneous intracranial hypotension (SIH) is a syndrome caused by low cerebrospinal fluid (CSF) pressure due to leakage of CSF. Clinically, orthostatic headache, neck pain, nausea, emesis, interscapular pain, diplopia, dizziness, changes in hearing, visual blurring and radicular upper extremity symptoms are most frequently observed. We describe a 57-year-old man with SIH who presented with postural tremor. CSF leakage was revealed by cranial MRI. Lumbar puncture identified low CSF pressure and intrathecal gadolinium enhanced MR cisternography showed diffuse CSF leakage in the thoracolumbar region. The patient underwent epidural blood patching, which resulted in complete resolution of postural tremor within 2 months.
Article ID(s) 20036551 (PubMed)
10.1016/j.jocn.2009.05.024 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous spinal cerebrospinal fluid leaks as the cause of subdural hematomas in elderly patients on anticoagulation.
Publication Journal of neurosurgery 2010 Feb; 112(2) (Publication year: 2010)
Author(s) Schievink Wouter I, Maya M Marcel, Pikul Brian K, Louy Charles
Abstract Subdural hematoma is a relatively common complication of long-term anticoagulation, particularly in the elderly. The combination of anticoagulation and cerebral cortical atrophy is believed to be sufficient to explain the subdural bleeding. The authors report a series of elderly patients who were on a regimen of anticoagulation and developed chronic subdural hematomas (SDHs) due to a spontaneous spinal CSF leak. They reviewed the medical records and imaging studies of a consecutive group of patients with spontaneous intracranial hypotension who were evaluated at Cedars-Sinai Medical Center. Among 141 patients with spontaneous spinal CSF leaks and spontaneous intracranial hypotension, 3 (2%) were taking anticoagulants at the time of onset of symptoms. The mean age of the 3 patients (1 woman and 2 men) was 74 years (range 68-86 years). All 3 patients had chronic SDHs measuring between 12 and 23 mm in maximal diameter. The SDHs resolved after treatment of the underlying spontaneous spinal CSF leak, and there was no need for hematoma evacuation. Epidural blood patches were used in 2 patients, and percutaneous placement of a fibrin sealant was used in 1 patient. The presence of an underlying spontaneous spinal CSF leak should be considered in patients with chronic SDHs, even among the elderly taking anticoagulants.
Article ID(s) 19199465 (PubMed)
10.3171/2008.10.JNS08428 (DOI)
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2009



Article in Favourites List Article not in Must-Read List  A novel technique for treatment of intractable spontaneous intracranial hypotension: lumbar dural reduction surgery.
Publication Headache 2009 Jul; 49(7) (Publication year: 2009)
Author(s) Schievink Wouter I
Abstract BACKGROUND AND OBJECTIVE
Spontaneous intracranial hypotension has become a well-described cause of headache particularly among young and middle-aged individuals. Treatment of the underlying spinal cerebrospinal fluid (CSF) leak is effective in relieving symptoms in the vast majority of patients but symptoms may become refractory. The author describes a novel surgical technique to treat intractable spontaneous intracranial hypotension.

METHODS
A lumbar laminectomy is performed, a strip of dura is resected, and the dural defect is closed. The resulting decrease in lumbar CSF volume is believed to increase intracranial CSF volume and pressure.

RESULTS
The technique was utilized in a patient who suffered with intractable positional headaches because of a spinal CSF leak for 6 years in spite of numerous surgical and nonsurgical therapies. Significant improvement of symptoms was sustained during a 1-year period of follow-up.

CONCLUSION
Dural reduction surgery may be considered in carefully selected patients with intracranial hypotension.
Article ID(s) 19473279 (PubMed)
10.1111/j.1526-4610.2009.01450.x (DOI)
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Article Not in Favourites List Article not in Must-Read List  Clinical features and outcomes in spontaneous intracranial hypotension: a survey of 90 consecutive patients.
Publication Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2009 May; 30 Suppl 1 (Publication year: 2009)
Author(s) Mea E, Chiapparini L, Savoiardo M, Franzini A, Bussone G, Leone M
Abstract Spontaneous intracranial hypotension (SIH) is a rare disabling condition whose main clinical manifestation is orthostatic headache. We analysed clinical characteristics in relation to time to resolution in 90 consecutive patients diagnosed with SIH at our centre between 1993 and 2006. After excluding 7 patients lost to follow-up, the remaining 83 cases were divided into four groups: Group A (53 cases) with progressively worsening orthostatic headache; Group B (3 cases) with severe acute-onset orthostatic headache; Group C (9 cases) with fluctuating non-continuous headache, of mild severity, that, in 33% of cases, did not worsen on standing; Group D (18 cases), 5 with a previous history of headache, 14 with orthostatic headache, and 10 with altered neurological examination. Complete symptoms and neuroradiological resolution occurred during follow-up in Groups A, B and D, but was longer in Group D probably in relation to more severe clinical picture with altered neurological examination. However, after a mean of 52 months (range 24-108), none of the nine Group C patients had MRI indicating complete resolution. The main characteristic of Group C related to incomplete resolution was delayed diagnosis. These preliminary findings suggest that early diagnosis of SIH correlates with better outcome, further suggesting that patients with a new headache that may worsen on standing or sitting should undergo MRI with contrast to expedite a possible SIH diagnosis, even if the pain is relatively mild.
Article ID(s) 19415418 (PubMed)
10.1007/s10072-009-0060-8 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Diagnostic value of spinal MR imaging in spontaneous intracranial hypotension syndrome.
Publication AJNR. American journal of neuroradiology 2009 Jan; 30(1) (Publication year: 2009)
Author(s) Watanabe A, Horikoshi T, Uchida M, Koizumi H, Yagishita T, Kinouchi H
Abstract BACKGROUND AND PURPOSE
Spontaneous intracranial hypotension (SIH) presents with orthostatic headache, and the diagnosis is made on the basis of low CSF pressure and brain MR imaging findings characteristic of the disorder. However, a broad spectrum of symptoms and MR imaging findings of SIH is recognized, and some cases have no typical MR imaging abnormalities. SIH is believed to be caused by CSF leakage from the spinal dural sac, whereas the usefulness of MR imaging of the spine remains unclear. Our aim was to elucidate the diagnostic value of brain and spinal MR imaging

MATERIALS AND METHODS
The sensitivities for the detection of SIH were retrospectively evaluated in 18 patients with SIH treated in our institutions between January 1998 and August 2007.

RESULTS
Brain MR imaging detected abnormalities in 15 of the 18 patients (83%): diffuse pachymeningeal enhancement in 15 (83%), descent of the cerebellar tonsil in 13 (72%), brain stem sagging in 13 (72%), enlargement of the pituitary gland in 12 (67%), and subdural fluid collection in 13 (72%). Spinal MR imaging detected abnormalities in 17 of the 18 patients (94%): distention of the epidural veins in 14 (78%), epidural fluid collection on fat-saturated T2-weighted images in 16 (89%), and abnormal visualization of the nerve root sleeve in only 1 (6%). The sensitivity for SIH was 83% for brain MR imaging and 94% for spinal MR imaging.

CONCLUSIONS
Spinal MR imaging is useful for the diagnosis of SIH, especially in the early stage.
Article ID(s) 18768717 (PubMed)
10.3174/ajnr.A1277 (DOI)
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Article in Favourites List Article not in Must-Read List  Heavily T2-weighted MR myelography vs CT myelography in spontaneous intracranial hypotension.
Publication Neurology 2009 Dec 1; 73(22) (Publication year: 2009)
Author(s) Wang Y-F, Lirng J-F, Fuh J-L, Hseu S-S, Wang S-J
Abstract OBJECTIVE
To assess the diagnostic accuracy of heavily T2-weighted magnetic resonance myelography (MRM) in patients with spontaneous intracranial hypotension (SIH).

METHODS
Patients with SIH were recruited prospectively, and first underwent MRM and then computed tomographic myelography (CTM). The results of MRM were validated with the gold standard, CTM, focusing on 1) CSF leaks along the nerve roots, 2) epidural CSF collections, and 3) high-cervical (C1-3) retrospinal CSF collections. Comparisons of these 3 findings between the 2 studies were made by kappa statistics and agreement rates. Targeted epidural blood patches (EBPs) were placed at the levels of CSF leaks if supportive treatment failed.

RESULTS
Nineteen patients (6 men and 13 women, mean age 37.9 +/- 8.6 years) with SIH completed the study. MRM did not differ from CTM in the detection rates of CSF leaks along the nerve roots (84% vs 74%, p = 0.23), high-cervical retrospinal CSF collections (32% vs 16%, p = 0.13), and epidural CSF collections (89% vs 79%, p = 0.20). MRM demonstrated more spinal levels of CSF leaks (2.2 +/- 1.7 vs 1.5 +/- 1.5, p = 0.011) and epidural collections (12.2 +/- 5.9 vs 7.1 +/- 5.8, p < 0.001) than CTM. The overall level-by-level concordance was substantial for CSF leaks along the nerve roots (C1-L3) (kappa = 0.71, p < 0.001, agreement = 95%) and high-cervical retrospinal CSF collections (C1-3) (kappa = 0.73, p < 0.001, agreement = 92%), and moderate for epidural CSF collections (C1-L3) (kappa = 0.47, p < 0.001, agreement = 72%). Ten of the 14 patients (71%) receiving targeted EBPs experienced sustained symptomatic relief after a single attempt.

CONCLUSIONS
Heavily T2-weighted magnetic resonance myelography was accurate in localizing CSF leaks for patients with spontaneous intracranial hypotension. This noninvasive technique may be an alternative to computed tomographic myelography before targeted epidural blood patches.
Article ID(s) 19949036 (PubMed)
10.1212/WNL.0b013e3181c3fd99 (DOI)
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Article in Favourites List Article not in Must-Read List  Spontaneous intracranial hypotension.
Publication Developmental medicine and child neurology 2009 Dec; 51(12) (Publication year: 2009)
Author(s) Gordon Neil
Abstract Since the introduction of magnetic resonance imaging (MRI), spontaneous intracranial hypotension has been diagnosed much more frequently. The aim of this review is to discuss the symptoms and signs of the condition, in particular the characteristics of the associated headache, with sudden onset after sitting or standing, so that it can be included under the rubric of 'thunderclap headache'. This type of headache, like post lumbar puncture headaches, may be caused by cerebral vasodilatation and exacerbated by lowered pressure of the cerebrospinal fluid (CSF). Other symptoms include neck stiffness, nausea, vomiting, vertigo, tinnitus, deafness, and cognitive abnormalities. The clinical picture can sometimes mimic frontotemporal dementia, and the behaviour of some patients can sometimes be described as hypoactive-hypoalert, with somnolence, impaired attention, and stereotyped motor activity. Sagging of the brain, caused by leakeage of the CSF, can cause lesions in the brainstem with stupor, gaze palsies, and cranial nerve palsies. The condition can be a risk factor for cerebral venous thrombosis because of slowing of the blood flow and distortion of the blood vessels. The clinical picture may well suggest the diagnosis, but the headache may possibly indicate a subarachnoid haemorrhage. However, MRI will help to confirm the diagnosis and to localize the site of the CSF leak. MRI myelograms are of particular value, but if they are equivocal a computed tomography myelogram should be performed. The leakage of CSF is due to a tear in the dura, most frequently where the spinal roots leave the subarachnoid space. If this does not heal with bedrest, an epidural blood patch or a percutaneous injection of fibrin glue may be needed. More information is required on long-term follow-up.
Article ID(s) 19909307 (PubMed)
10.1111/j.1469-8749.2009.03514.x (DOI)
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2008



Article Not in Favourites List Article not in Must-Read List  Absence of TGFBR2 mutations in patients with spontaneous spinal CSF leaks and intracranial hypotension.
Publication The journal of headache and pain 2008 Apr; 9(2) (Publication year: 2008)
Author(s) Schievink Wouter I, Gordon Ora K, Hyland James C, Ala-Kokko Leena
Abstract A heritable connective-tissue-disorder often is suspected in patients with spontaneous spinal CSF leaks and intracranial hypotension, but the nature of the disorder remains unknown in most patients. The aim of this study was to assess the gene encoding TGF-beta receptor-2 (TGFBR2) as a candidate gene for spinal CSF leaks. We searched the TGFBR2 gene for mutations in eight patients with spontaneous spinal CSF leaks who also had other features associated with TGFBR2 mutations, i.e., skeletal features of Marfan syndrome, arterial tortuosity, and(or) thoracic aortic aneurysm. The mean age of these 7 women and 1 man was 38 years (range 14-60 years). We detected no TGFBR2 mutations and conclude that TGFBR2 mutations are not a major factor in spontaneous spinal CSF leaks.
Article ID(s) 18264665 (PubMed)
10.1007/s10194-008-0017-y (DOI)
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Article Not in Favourites List Article not in Must-Read List  Cerebral venous thrombosis in spontaneous intracranial hypotension.
Publication Headache 2008 Nov-Dec; 48(10) (Publication year: 2008)
Author(s) Schievink Wouter I, Maya Menahem Marcel
Abstract BACKGROUND AND OBJECTIVE
The occurrence of cerebral venous thrombosis has been reported among patients with spontaneous intracranial hypotension, but a causal relationship has not been clearly established. We reviewed our experience with spontaneous intracranial hypotension and cerebral venous thrombosis and we reviewed the relevant literature to evaluate the relationship between these 2 entities.

METHODS
We reviewed the medical records and imaging studies of a consecutive group of patients with spontaneous intracranial hypotension evaluated at a tertiary care center between 1/1/2001 and 12/31/2007. The main search strategy was a systemic review of journal articles in MEDLINE (1966 to January 2008).

RESULTS
Among 141 patients with spontaneous intracranial hypotension, 3 (2.1%) were also diagnosed with cerebral venous thrombosis. Among these 3 patients and the 17 reported in the literature there were 11 men and 9 women with a mean age of 39.5 years. Radiographic or clinical evidence for spontaneous intracranial hypotension preceding cerebral venous thrombosis was found in most patients, while there was no evidence for cerebral venous thrombosis preceding spontaneous intracranial hypotension in any patient. Eight (40%) of the 20 patients were found to have a change in their headache pattern believed to be due to the development of cerebral venous thrombosis. Complications of cerebral venous thrombosis, eg, cerebral venous infarction, occurred in 8 patients (40%).

CONCLUSIONS
Spontaneous intracranial hypotension is a risk factor for cerebral venous thrombosis, but cerebral venous thrombosis is found in only about 2% of patients with spontaneous intracranial hypotension. A change in headache pattern is not a reliable predictor of the development of cerebral venous thrombosis in patients with spontaneous intracranial hypotension.
Article ID(s) 19076649 (PubMed)
10.1111/j.1526-4610.2008.01251.x (DOI)
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Article in Favourites List Article is Must-Read  Detection of CSF leak in spinal CSF leak syndrome using MR myelography: correlation with radioisotope cisternography.
Publication AJNR. American journal of neuroradiology 2008 Apr; 29(4) (Publication year: 2008)
Author(s) Yoo H-M, Kim S J, Choi C G, Lee D H, Lee J H, Suh D C, Choi J W, Jeong K S, Chung S J, Kim J S, Yun S-C
Abstract BACKGROUND AND PURPOSE
Spinal CSF leak syndrome is a unique disorder caused by spinal CSF leak. In this study, we attempted to determine whether MR myelography (MRM) can detect the leakage site in the spine.

MATERIALS AND METHODS
We performed both MRM and radioisotope cisternography (RIC) in 15 patients with spinal CSF leak syndrome. Patients were included in this study if they had at least 2 of the following criteria: 1) orthostatic headache, 2) low CSF opening pressure, and 3) diffuse pachymeningeal enhancement on brain MR imaging. For comparison, we performed MRM in 15 subjects without symptoms of spinal CSF leak syndrome. MRM was performed with the 2D turbo spin-echo technique in the entire spine by using a 1.5T scanner. Two blinded radiologists evaluated the MRM findings in a total of 30 cases, composed of patient and control groups, with regard to the presence of leakage and the level of leakage if present. RIC was performed only in the patient group and was assessed by consensus among 3 physicians experienced in nuclear medicine. The diagnostic performance of MRM and RIC was evaluated on the basis of the clinical diagnosis of spinal CSF leak syndrome.

RESULTS
The sensitivity, specificity, and accuracy of MR myelography for detecting CSF leak was 86.7%, 86.7%, and 86.7% for reader 1, respectively, and 80.0%, 93.3%, and 86.7% for reader 2, respectively. The sensitivity of RIC was 93.3%. Agreement between the 2 techniques for the detection of CSF leak was substantial in reader 1 and moderate in reader 2 (kappa = 0.634 and 0.444, respectively).

CONCLUSION
MRM is an effective tool for detecting CSF leak in the spine in patients with spinal CSF leak syndrome.
Article ID(s) 18202233 (PubMed)
10.3174/ajnr.A0920 (DOI)
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Article in Favourites List Article is Must-Read  Diagnostic criteria for spontaneous spinal CSF leaks and intracranial hypotension.
Publication AJNR. American journal of neuroradiology 2008 May; 29(5) (Publication year: 2008)
Author(s) Schievink W I, Maya M M, Louy C, Moser F G, Tourje J
Abstract BACKGROUND AND PURPOSE
Comprehensive diagnostic criteria encompassing the varied clinical and radiographic manifestations of spontaneous intracranial hypotension are not available. Therefore, we propose a new set of diagnostic criteria.

MATERIALS AND METHODS
The diagnostic criteria are based on results of brain and spine imaging, clinical manifestations, results of lumbar puncture, and response to epidural blood patching. The diagnostic criteria include criterion A, the demonstration of extrathecal CSF on spinal imaging. If criterion A is not met, criterion B, which is cranial MR imaging findings of spontaneous intracranial hypotension, follows, with at least one of the following: 1) low opening pressure, 2) spinal meningeal diverticulum, or 3) improvement of symptoms after epidural blood patch. If criteria A and B are not met, there is criterion C, the presence of all of the following or at least 2 of the following if typical orthostatic headaches are present: 1) low opening pressure, 2) spinal meningeal diverticulum, and 3) improvement of symptoms after epidural blood patch. These criteria were applied to a group of 107 consecutive patients evaluated for spontaneous spinal CSF leaks and intracranial hypotension.

RESULTS
The diagnosis was confirmed in 94 patients, with use of criterion A in 78 patients, criterion B in 11 patients, and criterion C in 5 patients.

CONCLUSIONS
A new diagnostic scheme is presented reflecting the wide spectrum of clinical and radiographic manifestations of spontaneous spinal CSF leaks and intracranial hypotension.
Article ID(s) 18258706 (PubMed)
10.3174/ajnr.A0956 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Management of cerebrospinal fluid leakage following cervical spine surgery.
Publication Chinese medical sciences journal = Chung-kuo i hsueh k'o hsueh tsa chih 2008 Jun; 23(2) (Publication year: 2008)
Author(s) Tian Ye, Yu Ke-Yi, Wang Yi-Peng, Qian Jun, Qiu Gui-Xing
Abstract OBJECTIVE
To investigate the management and outcome of cerebrospinal fluid leakage (CSFL) after cervical surgery.

METHODS
Medical records of 642 patients who underwent cervical surgery between December 1999 and December 2005 at our hospital were retrospectively reviewed. Five patients complicated by CSFL after surgery were enrolled, of which 4 cases were complicated after ossified posterior longitudinal ligament or posterior vertebral osteophyte resection directly injuring the dura, and 1 case after posterior cervical double-door laminoplasty without observed dural injury during surgery. Of the 5 CSFL cases, 4 cases occurred at 1-3 days after operation and 1 case at 9 days after operation. All 5 postoperative CSFL cases were treated through wound drainage removal, wound sutures, prophylactic antibiotics, and continuous subarachnoid drainage in the elevated head position.

RESULTS
All 5 CSFL cases experienced leakage cessation within 1-3 days and wound healing within 4-8 days, and subarachnoid drainage lasted 11-16 days with an average volume of 320 mL (range, 150-410 mL). Four cases experienced headache, nausea and vomiting, 1 case suffered from somnolence and hyponatremia, and symptoms subsided after symptomatic treatment and intravenous fluid administration. All patients were followed up for an average of 32 months (range, 22-50 months). No occurrence of cerebrospinal fluid cyst or wound infection was observed. CSFL produced no significant negative effects upon neuromuscular function recovery.

CONCLUSION
Continuous subarachnoid cavity drainage in combination with elevated head position is a simple and safe non-surgical method in treatment of CSFL following cervical surgery.
Article ID(s) 18686633 (PubMed)
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Article in Favourites List Article not in Must-Read List  Spontaneous spinal cerebrospinal fluid leaks.
Publication Cephalalgia : an international journal of headache 2008 Dec; 28(12) (Publication year: 2008)
Author(s) Schievink W I
Abstract Spontaneous intracranial hypotension is an uncommon but not rare cause of new onset daily persistent headaches. A delay in diagnosis is the norm. Women are affected more commonly than men and most are in the fifth or sixth decade of life. The underlying cause is a spontaneous spinal cerebrospinal fluid (CSF) leak. Typically the headache is orthostatic in nature but other headache patterns occur as well. Associated symptoms are common and include neck pain, a change in hearing, diplopia, facial numbness, cognitive abnormalities and even coma. Typical imaging findings consist of subdural fluid collections, pachymeningeal enhancement, pituitary hyperaemia and brain sagging, but magnetic resonance imaging may be normal. Myelography is the study of choice to identify the CSF leak but is not always necessary to make the diagnosis. Treatment consists of bedrest, abdominal binder, epidural blood patching, percutaneous fibrin glue injection or surgical CSF leak repair. Outcomes have been poorly studied.
Article ID(s) 19037970 (PubMed)
10.1111/j.1468-2982.2008.01776.x (DOI)
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Article Not in Favourites List Article not in Must-Read List  The timing of MRI determines the presence or absence of diffuse pachymeningeal enhancement in patients with spontaneous intracranial hypotension.
Publication Cephalalgia : an international journal of headache 2008 Apr; 28(4) (Publication year: 2008)
Author(s) Fuh J-L, Wang S-J, Lai T-H, Hseu S-S
Abstract The timing and clinical relevance of diffuse pachymeningeal enhancement (DPE) in the magnetic resonance imaging (MRI) examination of patients with spontaneous intracranial hypotension (SIH) remain undetermined. We reviewed 53 consecutive SIH patients (30 F/23 M, mean age of onset 41.7 +/- 11.3 years) in a tertiary hospital. Thirteen (24.5%) patients did not have DPE on their initial cranial MRIs. They had significantly shorter latency between the time of MRI examinations and the time of headache onset compared with those with DPE (6.5 +/- 4.4 vs. 20.4 +/- 16.3 days, t-test, P < 0.001). Eight of these 13 patients received a follow-up MRI (mean duration 30.3 +/- 16.6 days, range 6-59 days) and six of them revealed DPE. Among patients with DPE, the enhancement disappeared as early as 25 days after headache onset. The outcome did not differ between patients with and without DPE. The presence of DPE was associated with the timing of the MRI examination.
Article ID(s) 18284422 (PubMed)
10.1111/j.1468-2982.2007.01498.x (DOI)
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2007



Article Not in Favourites List Article not in Must-Read List  Frequency of spontaneous intracranial hypotension in the emergency department.
Publication The journal of headache and pain 2007 Dec; 8(6) (Publication year: 2007)
Author(s) Schievink Wouter I, Maya M M, Moser Franklin, Tourje James, Torbati Sam
Abstract Spontaneous intracranial hypotension is considered a rare disorder. We conducted a study on the frequency of spontaneous intracranial hypotension in the emergency department (ED). We identified patients with spontaneous intracranial hypotension evaluated in the ED of a large urban hospital between 1 January 2003 and 31 December 2006. For comparison, we also identified all patients with spontaneous subarachnoid haemorrhage (SAH). Eleven patients with previously undiagnosed spontaneous intracranial hypotension were evaluated in the ED during the four-year time period. All patients presented with positional headaches and the duration of symptoms varied from one day to three months. None of the patients were correctly diagnosed with spontaneous intracranial hypotension in the ED. During the same time period, 23 patients with aneurysmal SAH were evaluated. Spontaneous intracranial hypotension is more common than previously appreciated and the diagnosis in the ED remains problematic.
Article ID(s) 18071632 (PubMed)
PMC3476164 (PMC)
10.1007/s10194-007-0421-8 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Heavily T2-weighted MR myelography in patients with spontaneous intracranial hypotension: a case-control study.
Publication Cephalalgia : an international journal of headache 2007 Aug; 27(8) (Publication year: 2007)
Author(s) Tsai P-H, Fuh J-L, Lirng J-F, Wang S-J
Abstract We performed whole-spine heavily T2-weighted magnetic resonance (MR) myelography using a single-shot fast spin-echo pulse sequence in 17 patients (8 M/9 F) with spontaneous intracranial hypotension (SIH) to detect abnormal cerebrospinal fluid (CSF) collections. In addition, a group of age- and sex-matched controls were recruited. Follow-up MR myelography was also done at 3 weeks. MR myelography showed three kinds of abnormal CSF collections in 15 patients with SIH (88%): epidural fluid collection (n = 15, 88%), C1-2 extraspinal collections (n = 6, 35%) and CSF collections along nerve roots in the lower cervical or upper thoracic spines (n = 6, 35%). One patient (6%) showed a meningeal diverticulum. In contrast, none of the controls showed these findings. Overall, MR myelography results helped in early diagnosis of SIH in four (24%) patients whose initial brain MRIs failed to show typical SIH findings. Follow-up MR myelography results were compatible with the clinical changes with kappa statistics of 0.52 and an agreement rate of 76%. Our study showed heavily T2-weighted MR myelography provided a rapid, non-invasive and high yield method to diagnose and follow-up patients with SIH. Whether the CSF collections along the nerve roots represent the ongoing leakage sites warrants further study.
Article ID(s) 17645756 (PubMed)
10.1111/j.1468-2982.2007.01376.x (DOI)
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Article Not in Favourites List Article not in Must-Read List  Precipitating factors of spontaneous spinal CSF leaks and intracranial hypotension.
Publication Neurology 2007 Aug 14; 69(7) (Publication year: 2007)
Author(s) Schievink Wouter I, Louy Charles
Abstract No abstract available.
Article ID(s) 17698794 (PubMed)
10.1212/01.wnl.0000267324.68013.8e (DOI)
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Article Not in Favourites List Article not in Must-Read List  Subdural haematoma in patients with spontaneous intracranial hypotension.
Publication Cephalalgia : an international journal of headache 2007 Feb; 27(2) (Publication year: 2007)
Author(s) Lai T H, Fuh J L, Lirng J F, Tsai P H, Wang S J
Abstract The incidence and clinical relevance of subdural haematoma (SDH) in patients with spontaneous intracranial hypotension (SIH) remain undetermined. We reviewed 40 consecutive SIH patients (18 female, 22 male) in a tertiary hospital. Eight (20%) of them had SDH and nine (23%), non-haemorrhagic subdural collections. The presence of SDH was associated with higher frequencies of male gender, recurrence of severe headache and neurological deficits. Outcomes were satisfactory after supportive care or epidural blood patches except for one SDH patient, who developed transtentorial herniation resulting in Duret haemorrhage and infarctions of bilateral posterior cerebral artery territories. In conclusion, subdural fluid collections were common in patients with SIH. SDH was associated with headache worsening or neurological deficits. Patients with SDH generally recovered well; however, serious sequela might occur.
Article ID(s) 17257233 (PubMed)
10.1111/j.1468-2982.2006.01249.x (DOI)
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2006



Article in Favourites List Article is Must-Read  Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension.
Publication JAMA 2006 May 17; 295(19) (Publication year: 2006)
Author(s) Schievink Wouter I
Abstract CONTEXT
Spontaneous intracranial hypotension is caused by spontaneous spinal cerebrospinal fluid (CSF) leaks and is known for causing orthostatic headaches. It is an important cause of new headaches in young and middle-aged individuals, but initial misdiagnosis is common.

OBJECTIVE
To summarize existing evidence regarding the epidemiology, pathophysiology, diagnosis, and management of spontaneous spinal CSF leaks and intracranial hypotension.

EVIDENCE ACQUISITION
MEDLINE (1966-2005) and OLDMEDLINE (1950-1965) were searched using the terms intracranial hypotension, CSF leak, low pressure headache, and CSF hypovolemia. Reference lists of these articles and ongoing investigations in this area were used as well.

EVIDENCE SYNTHESIS
Spontaneous intracranial hypotension is caused by single or multiple spinal CSF leaks. The incidence has been estimated at 5 per 100,000 per year, with a peak around age 40 years. Women are affected more commonly than men. Mechanical factors combine with an underlying connective tissue disorder to cause the CSF leaks. An orthostatic headache is the prototypical manifestation but other headache patterns occur as well, and associated symptoms are common. Typical magnetic resonance imaging findings include subdural fluid collections, enhancement of the pachymeninges, engorgement of venous structures, pituitary hyperemia, and sagging of the brain (mnemonic: SEEPS). Myelography is the study of choice to identify the spinal CSF leak. Treatments include bed rest, epidural blood patching, percutaneous placement of fibrin sealant, and surgical CSF leak repair, but outcomes have been poorly studied and no management strategies have been studied in properly controlled randomized trials.

CONCLUSIONS
Spontaneous intracranial hypotension is not rare but it remains underdiagnosed. The spectrum of clinical and radiographic manifestations is varied, with diagnosis largely based on clinical suspicion, cranial magnetic resonance imaging, and myelography. Numerous treatment options are available, but much remains to be learned about this disorder.
Article ID(s) 16705110 (PubMed)
10.1001/jama.295.19.2286 (DOI)
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2005



Article Not in Favourites List Article not in Must-Read List  Cranial MRI predicts outcome of spontaneous intracranial hypotension.
Publication Neurology 2005 Apr 12; 64(7) (Publication year: 2005)
Author(s) Schievink Wouter I, Maya M Marcel, Louy Charles
Abstract The outcome of spontaneous intracranial hypotension has been unpredictable. The results of initial MRI were correlated to outcome of treatment in 33 patients with spontaneous intracranial hypotension. A good outcome was obtained in 25 (97%) of 26 patients with an abnormal MRI vs only 1 (14%) of 7 patients with a normal MRI (p = 0.00004). These findings show that normal initial MRI is predictive of poor outcome in spontaneous intracranial hypotension.
Article ID(s) 15824366 (PubMed)
10.1212/01.WNL.0000156906.84165.C0 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spectrum of subdural fluid collections in spontaneous intracranial hypotension.
Publication Journal of neurosurgery 2005 Oct; 103(4) (Publication year: 2005)
Author(s) Schievink Wouter I, Maya M Marcel, Moser Franklin G, Tourje James
Abstract OBJECT
Spontaneous intracranial hypotension is a noteworthy but commonly misdiagnosed cause of new daily persistent headaches. Subdural fluid collections are frequent radiographic findings, but they can be interpreted as primary rather than secondary pathological entities, and uncertainties exist regarding their optimal management. The authors therefore reviewed their experience with subdural fluid collections in 40 consecutive patients with spontaneous spinal cerebrospinal fluid (CSF) leaks and intracranial hypotension.

METHODS
The mean age of the 26 female and 14 male patients was 43 years (range 13-72 years). Subdural fluid collections were present in 20 patients (50%); 12 of these patients (60%) had subdural hygromas alone, and eight (40%) had subacute to chronic subdural hematomas (SDHs) associated with significant mass effect. The subdural hygromas resolved within several days to weeks following treatment of the underlying CSF leak. Three patients with SDHs underwent evacuation of the hematoma prior to the establishment of the diagnosis of spontaneous intracranial hypotension, but the SDHs did not resolve until the underlying spinal CSF leak was treated. In the remaining five patients, the CSF leak was treated primarily and the SDHs resolved over a 1- to 3-month period without the need for evacuation.

CONCLUSIONS
Subdural fluid collections are common in spontaneous intracranial hypotension, varying in appearance from thin subdural hygromas to large SDHs associated with significant mass effect. These collections can be safely managed by directing treatment at the underlying CSF leak without the need for hematoma evacuation.
Article ID(s) 16266041 (PubMed)
10.3171/jns.2005.103.4.0608 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous intracranial hypotension presenting as mental deterioration.
Publication Headache 2005 Jan; 45(1) (Publication year: 2005)
Author(s) Tsai Ping-Huang, Wang Shuu-Jiun, Lirng Jiing-Feng, Fuh Jong-Ling
Abstract A 55-year-old woman had new onset of postural headache followed by change of mental status 3 weeks later. Magnetic resonance imaging (MRI) of the brain and whole spine showed typical spontaneous intracranial hypotension (SIH) findings, bilateral subdural hematoma, and cerebrospinal fluid leakage over the T7-T9. Her headache and mentality improved after epidural blood patches. Early recognition and correct diagnosis are crucial for successful treatment in patients with SIH presenting with mental confusion.
Article ID(s) 15663618 (PubMed)
10.1111/j.1526-4610.2005.t01-1-05013.x (DOI)
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2004



Article Not in Favourites List Article not in Must-Read List  Combined HRCT and MRI in the detection of CSF rhinorrhea.
Publication Skull base : official journal of North American Skull Base Society ... [et al.] 2004 Aug; 14(3) (Publication year: 2004)
Author(s) Mostafa Badr Eldin, Khafagi Ahmed
Abstract Cerebrospinal fluid (CSF) rhinorrhea is a potentially dangerous problem. Accurate preoperative localization of the site of leakage is mandatory. The standard diagnostic technique is computed tomography (CT) cisternography. Because of its related risks, however, various alternatives have been suggested. High-resolution CT (HRCT) provides good bony details, but fluid is poorly detected. In contrast, T2-weighted magnetic resonance imaging (MRI) shows CSF as a bright signal, but spatial resolution is poor as is the depiction of bony details. To overcome the shortcomings of both techniques, we superimposed the images obtained from each modality and used the result to plan surgical explorations. The sensitivity of HRCT was 88.25%. Fat-suppressed T2-weighted MRI detected a CSF-like density in 18 cases (90%) with a sensitivity of 88.88%. Superimposing the CTs and MRIs accurately localized the site of CSF leakage in 17 of 19 cases with a sensitivity of 89.74%. This finding compares favorably with the results of other techniques. We thus recommend this technique as the primary diagnostic method of choice for the investigation of patients with CSF rhinorrhea.
Article ID(s) 16145599 (PubMed)
10.1055/s-2004-832259 (DOI)
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Article in Favourites List Article not in Must-Read List  Connective tissue disorders with spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study.
Publication Neurosurgery 2004 Jan; 54(1) (Publication year: 2004)
Author(s) Schievink Wouter I, Gordon Ora Karp, Tourje James
Abstract OBJECTIVE
Intracranial hypotension attributable to a spontaneous spinal cerebrospinal fluid (CSF) leak is an increasingly recognized cause of postural headaches. The cause of these leaks is poorly understood, but it is likely multifactorial and may involve a primary connective tissue disorder. We undertook a study to estimate the contribution of systemic connective tissue disorders to the development of spontaneous spinal CSF leaks.

METHODS
We examined a group of 18 consecutive patients with spontaneous spinal CSF leaks for features of a connective tissue disorder.

RESULTS
The mean age of the 15 female patients and 3 male patients was 38 years (range, 22-55 yr). Seven patients (38%) demonstrated stigmata of a systemic connective tissue disorder, and three distinct types of disorders could be identified, as follows. 1) The association of spontaneous spinal CSF leaks and minor skeletal features of Marfan syndrome was noted for three patients. 2) Ehlers-Danlos syndrome Type II was noted for two patients. 3) Joint hypermobility associated with marked attenuation of the dorsal muscular fascia, precluding proper wound closure, was noted for two patients. In addition, isolated small-joint hypermobility was observed for five patients (28%). Slit-lamp ocular examinations, echocardiographic evaluations, histopathological examinations of skin biopsy specimens, and renal scanning did not reveal any other features of a systemic connective tissue disorder.

CONCLUSION
Findings suggesting connective tissue disorders are common among patients with spontaneous spinal CSF leaks, and manifestations may be subtle. A variety of disorders can be identified, probably reflecting genetic heterogeneity. Problems with wound healing may occur as a result of the systemic nature of the underlying connective tissue disorder.
Article ID(s) 14683542 (PubMed)
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Article in Favourites List Article not in Must-Read List  False localizing sign of C1-2 cerebrospinal fluid leak in spontaneous intracranial hypotension.
Publication Journal of neurosurgery 2004 Apr; 100(4) (Publication year: 2004)
Author(s) Schievink Wouter I, Maya M Marcel, Tourje James
Abstract OBJECT
Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid (CSF) leak is an important cause of new daily persistent headaches. Spinal neuroimaging is important in the treatment of these patients, particularly when direct repair of the CSF leak is contemplated. Retrospinal C1-2 fluid collections may be noted on spinal imaging and these are generally believed to correspond to the site of the CSF leak. The authors undertook a study to determine the significance of these C1-2 fluid collections.

METHODS
The patient population consisted of a consecutive group of 25 patients (18 female and seven male) who were evaluated for surgical repair of a spontaneous spinal CSF leak. The mean age of the 18 patients was 38 years (range 13-72 years). All patients underwent computerized tomography myelography. Three patients (12%) had extensive retrospinal C1-2 fluid collections; the mean age of this woman and these two men was 41 years (range 39-43 years). The actual site of the CSF leak was located at the lower cervical spine in these patients and did not correspond to the site of the retrospinal C1-2 fluid collection.

CONCLUSIONS
A retrospinal fluid collection at the C1-2 level does not necessarily indicate the site of the CSF leak in patients with spontaneous intracranial hypotension. This is an important consideration in the treatment of these patients because therapy may be inadvertently directed at this site.
Article ID(s) 15070118 (PubMed)
10.3171/jns.2004.100.4.0639 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Reversible coma: a rare presentation of spontaneous intracranial hypotension.
Publication The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques 2004 Nov; 31(4) (Publication year: 2004)
Author(s) Kashmere Jodi L, Jacka Michael J, Emery Derek, Gross Donald W
Abstract BACKGROUND
Spontaneous intracranial hypotension (SIH) is a well-recognized neurologic disorder that typically presents with orthostatic headaches, low cerebral spinal fluid pressures and distinct abnormalities on magnetic resonance imaging.

METHODS
We present a case of a rare presentation of SIH.

RESULTS
A 49-year-old man presented with a two week history of orthostatic headaches that rapidly progressed to encephalopathy and coma, requiring intubation. Neuroimaging revealed abnormalities typical of SIH; diffusely enhancing pachymeninges, subdural fluid collections, and descent of the brain. Treatment with an epidural blood patch reversed his coma within minutes. Following a second blood patch, the patient became asymptomatic. No cerebral spinal leak could be identified on magnetic resonance imaging or on a nuclear medicine technetium cerebral spinal fluid flow study. At six month follow-up, he remained symptom free.

CONCLUSION
The mechanism of coma in SIH is presumed to be compression of the diencephalon from downward displacement of the brain. Although it is very unusual for patients with SIH to present with coma, it is important to recognize since the coma may be reversible with epidural blood patches.
Article ID(s) 15595268 (PubMed)
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2003



Article in Favourites List Article not in Must-Read List  Dynamic CT myelography: a technique for localizing high-flow spinal cerebrospinal fluid leaks.
Publication AJNR. American journal of neuroradiology 2003 Sep; 24(8) (Publication year: 2003)
Author(s) Luetmer Patrick H, Mokri Bahram
Abstract In some patients with spontaneous spinal CSF leaks, leaks are numerous or tears are so large that extrathecal myelographic contrast material is seen at multiple levels during CT, making identification of their source impossible. This study introduces a dynamic CT myelographic technique that provides high temporal and spatial resolution. In this technical note, we describe the utility of this technique in four patients with challenging high-flow spinal CSF leaks.
Article ID(s) 13679297 (PubMed)
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Article Not in Favourites List Article not in Must-Read List  Orthostatic headaches without CSF leak in postural tachycardia syndrome.
Publication Neurology 2003 Oct 14; 61(7) (Publication year: 2003)
Author(s) Mokri Bahram, Low Phillip A
Abstract Four women age 17 to 28 years presented with orthostatic headaches as the most prominent feature of their symptom complex. None had CSF leak or intracranial hypotension. Autonomic studies showed evidence of orthostatic intolerance with tachycardia in all cases. Treatment of orthostatic intolerance, mainly with volume expansion, was only partially effective. Orthostatic headaches are not always caused by CSF leak or supine intracranial hypotension. Occasionally they may be the major clinical manifestation of postural tachycardia syndrome or orthostatic intolerance.
Article ID(s) 14557573 (PubMed)
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Article Not in Favourites List Article not in Must-Read List  Post-dural puncture headache: pathogenesis, prevention and treatment.
Publication British journal of anaesthesia 2003 Nov; 91(5) (Publication year: 2003)
Author(s) Turnbull D K, Shepherd D B
Abstract Spinal anaesthesia developed in the late 1800s with the work of Wynter, Quincke and Corning. However, it was the German surgeon, Karl August Bier in 1898, who probably gave the first spinal anaesthetic. Bier also gained first-hand experience of the disabling headache related to dural puncture. He correctly surmised that the headache was related to excessive loss of cerebrospinal fluid (CSF). In the last 50 yr, the development of fine-gauge spinal needles and needle tip modification, has enabled a significant reduction in the incidence of post-dural puncture headache. Though it is clear that reducing the size of the dural perforation reduces the loss of CSF, there are many areas regarding the pathogenesis, treatment and prevention of post-dural puncture headache that remain contentious. How does the microscopic pattern of collagen alignment in the spinal dura affect the dimensions of the dural perforation? How do needle design, size and orientation influence leakage of CSF through the dural perforation? Can pharmacological methods reduce the symptoms of post-dural puncture headache? By which mechanism does the epidural blood patch cure headache? Is there a role for the prophylactic epidural blood patch? Do epidural saline, dextran, opioids and tissue glues reduce the rate of CSF loss? This review considers these contentious aspects of post-dural puncture headache.
Article ID(s) 14570796 (PubMed)
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2002



Article Not in Favourites List Article not in Must-Read List  Intracranial hypertension after treatment of spontaneous cerebrospinal fluid leaks.
Publication Mayo Clinic proceedings 2002 Nov; 77(11) (Publication year: 2002)
Author(s) Mokri Bahram
Abstract Four patients, aged 10 to 44 years, with spontaneous cerebrospinal fluid (CSF) leaks and intracranial hypotension developed intracranial hypertension after treatment of their CSF leaks. The leak was at the spinal level in all patients (thoracic level, 2; lumbar level, 1; and undetermined, 1). One patient responded to an epidural blood patch. Three patients responded to surgery, of whom 2 had not responded to prior epidural blood patches. Treatment resulted in complete resolution of symptoms, including orthostatic headaches and disappearance of magnetic resonance imaging abnormalities. However, all patients later developed steady headaches different from their previous headaches. None had recurrence of magnetic resonance imaging abnormalities or any evidence of occlusion of cerebral venous sinuses. All had increased CSF opening pressures. One had bilateral papilledema, and another had no venous pulsations on examination of fundi. Follow-up was possible in 2 patients. One responded well to treatment with acetazolamide, and the other improved gradually and was asymptomatic within several months.
Article ID(s) 12440561 (PubMed)
10.4065/77.11.1241 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Intrathecal gadolinium (gadopentetate dimeglumine) enhanced magnetic resonance myelography and cisternography: results of a multicenter study.
Publication Investigative radiology 2002 Mar; 37(3) (Publication year: 2002)
Author(s) Tali E Turgut, Ercan Nil, Krumina Gaida, Rudwan Mohammed, Mironov Angel, Zeng Qing Yu, Jinkins J Randy
Abstract RATIONALE AND OBJECTIVES
This cooperative multicenter human study was designed to evaluate the safety, magnetic resonance (MR) imaging characteristics, and clinical response to a single gadolinium contrast agent: gadopentetate dimeglumine.

MATERIAL AND METHODS
Ninety-five patients (age range: 1 month to 78 years; sex: 50 males, 45 females) were included in this prospective study. The patients presented clinically with a variety of cranial or spinal signs and symptoms for which an intrathecal contrast myelogram or cisternogram was requested by clinical staff. Via lumbar puncture (20-25 g needle), 3 to 5 mL/ml of cerebrospinal fluid were withdrawn and mixed with a single volume of 0.5 (n = 63), 0.7 (n = 13), 0.8 (n = 12), or 1.0 (n = 7) cc/mL of gadopentetate dimeglumine (Magnevist; Schering, Berlin, Germany). This was then injected into the subarachnoid space, and the needle was removed. Immediate and delayed (up to 96 hours) T1- and T2-weighted MR imaging was performed on super conductive, high-field (1.0-1.5 tesla) imaging units in two or three planes. All patients were hospitalized for an observation period of 24 hours following the procedure, and follow-up neurologic examinations were performed serially for 6 to 12 months afterward.

RESULTS
No patient manifested gross behavioral changes, neurologic alterations, or seizure activity at any time following the procedure. Nineteen patients (20%) experienced postural postlumbar puncture headache, six patients had nausea (6%), and two patients had episodes of vomiting (2%), all which resolved within the first 24 hours of the lumbar puncture with conservative bed rest.

CONCLUSION
This cooperative study demonstrates the general safety and feasibility of low dose (0.5-1.0 mL/ml) intrathecal gadopentetate dimeglumine administration. The potential useful clinical applications include the evaluation of obstructions and communications of the various subarachnoid spaces, spontaneous or traumatic/postsurgical craniospinal cerebrospinal fluid leaks, and subarachnoid space CSF flow and parenchymal CNS interstitial diffusion dynamics. This worldwide cooperative study seeks to progressively perform human studies for further definitive evaluation of the practical clinical applications, of the relationship of this technique to other imaging studies and modalities, and the long-term safety of the procedure in a larger number of subjects.
Article ID(s) 11882795 (PubMed)
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Article Not in Favourites List Article not in Must-Read List  Myth: Fluids, bed rest, and caffeine are effective in preventing and treating patients with post-lumbar puncture headache
Publication Western Journal of Medicine (Publication year: 2002)
Author(s) Lin, W. and Geiderman, J.
Abstract Post-LP headache remains a vexing problem that is not well understood. In attempting to prevent or treat this phenomenon, physicians should be aware that there is no evidence to support treatment with fluids and bed rest and that the evidence supporting the use of caffeine is poor.
Article ID(s) PMC1071660 (PMC)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous CSF leaks: underlying disorder of connective tissue.
Publication Neurology 2002 Mar 12; 58(5) (Publication year: 2002)
Author(s) Mokri Bahram, Maher Cormac O, Sencakova Drahomira
Abstract Of 58 consecutive patients with spontaneous CSF leaks, nine exhibited features of connective tissue disorder. One had Marfan's syndrome. Five additional patients had hyperflexible joints, of whom four had arachnodactyly, four were tall and slender, two had hyperextensible skin, and one had a strong family history of abdominal aorta aneurysms. Retinal detachment at a young age was noted in two. One patient had bilateral carotid dissections. A dural weakness may predispose patients to spontaneous CSF leak.
Article ID(s) 11889250 (PubMed)
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Article Not in Favourites List Article not in Must-Read List  Spontaneous spinal cerebrospinal fluid leaks and minor skeletal features of Marfan syndrome: a microfibrillopathy.
Publication Journal of neurosurgery 2002 Mar; 96(3) (Publication year: 2002)
Author(s) Schrijver Iris, Schievink Wouter I, Godfrey Maurice, Meyer Fredric B, Francke Uta
Abstract OBJECT
Spontaneous spinal cerebrospinal fluid (CSF) leaks are increasingly recognized as a cause of postural headaches. The authors examined a group of patients suffering from spontaneous spinal CSF leaks who also had minor skeletal features of Marfan syndrome for abnormalities of fibrillin-containing microfibrils.

METHODS
Patients with spontaneous CSF leaks were evaluated for the clinical characteristics of connective tissue disorders. Skin biopsies were obtained in three patients with skeletal manifestations that constitute part of the Marfan syndrome phenotype. Cultured fibroblasts were studied for fibrillin-1 synthesis and incorporation into the extracellular matrix (ECM) by performing quantitative metabolic labeling and immunohistochemical analysis. Among 20 consecutive patients found to have spinal CSF leaks, four (20%) exhibited minor skeletal features of Marfan syndrome, but lacked any ocular or cardiovascular abnormalities. The mean age of these patients (30 years) was lower than that of the 16 patients without skeletal abnormalities (44 years; p = 0.01). Abnormalities in fibrillin-1 metabolism and immunostaining were detected in all three patients with the skeletal abnormalities who underwent examination, but not in a control patient without these skeletal manifestations.

CONCLUSIONS
Twenty percent of patients who experience spontaneous spinal CSF leaks have minor skeletal features of Marfan syndrome. The authors demonstrated abnormalities in fibrillin-1 protein deposition in all patients examined, but only one person was found to have a fibrillin-1 abnormality typically found in classic Marfan syndrome. The results indicate that there is a heterogeneous involvement of other components of ECM microfibrils at the basis of this cerebrospinal manifestation. In addition, the authors identified a connective-tissue etiological factor in a group of disorders not previously classified as such.
Article ID(s) 11883832 (PubMed)
10.3171/jns.2002.96.3.0483 (DOI)
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1998



Article Not in Favourites List Article not in Must-Read List  Cervical bone spur presenting with spontaneous intracranial hypotension. Case report.
Publication Journal of neurosurgery 1998 Sep; 89(3) (Publication year: 1998)
Author(s) Vishteh A G, Schievink W I, Baskin J J, Sonntag V K
Abstract Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid (CSF) leak is a rare but increasingly recognized cause of postural headaches. The exact cause of these CSF leaks often remains unknown. The authors treated a 32-year-old man with a unique cause of spontaneous intracranial hypotension. He suffered an excruciating headache that was exacerbated by his being in an upright position. The results of four-vessel cerebral angiography were negative; however, magnetic resonance (MR) imaging of the brain revealed pachymeningeal enhancement and hindbrain herniation. A presumptive diagnosis of spontaneous intracranial hypotension was made. Myelography revealed extrathecal contrast material ventral to the cervical spinal cord as well as an unusual midline bone spur at C5-6. The patient's symptoms did not resolve with the application of epidural blood patches, and he subsequently underwent an anterior approach to the C5-6 spur. After discectomy, a slender bone spur that had pierced the thecal sac was found. After its removal, the dural rent was closed using two interrupted prolene sutures. The patient was discharged home 2 days later. On follow up his symptoms had resolved, and on MR imaging the pachymeningeal enhancement had resolved and the cerebellar herniation had improved slightly.
Article ID(s) 9724127 (PubMed)
10.3171/jns.1998.89.3.0483 (DOI)
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Article Not in Favourites List Article not in Must-Read List  Surgical treatment of spontaneous spinal cerebrospinal fluid leaks.
Publication Journal of neurosurgery 1998 Feb; 88(2) (Publication year: 1998)
Author(s) Schievink W I, Morreale V M, Atkinson J L, Meyer F B, Piepgras D G, Ebersold M J
Abstract OBJECT
Spontaneous spinal cerebrospinal fluid (CSF) leaks are an increasingly recognized cause of intracranial hypotension and may require neurosurgical intervention. In the present report the authors review their experience with the surgical management of spontaneous spinal CSF leaks.

METHODS
Between 1992 and 1997, 10 patients with spontaneous spinal CSF leaks and intracranial hypotension were treated surgically. The mean age of the seven women and three men was 42.3 years (range 22-61 years). Preoperative imaging showed a single meningeal diverticulum in two patients, a complex of diverticula in one patient, and a focal CSF leak alone in seven patients. Surgical exploration in these seven patients demonstrated meningeal diverticula in one patient; no clear source of CSF leakage could be identified in the remaining six patients. Treatment consisted of ligation of the diverticula or packing of the epidural space with muscle or Gelfoam. Multiple simultaneous spinal CSF leaks were identified in three patients.

CONCLUSIONS
All patients experienced complete relief of their headaches postoperatively. There has been no recurrence of symptoms in any of the patients during a mean follow-up period of 19 months (range 3-58 months; 16 person-years of cumulative follow up). Complications consisted of transient intracranial hypertension in one patient and leg numbness in another patient. Although the disease is often self-limiting, surgical treatment has an important role in the management of spontaneous spinal CSF leaks. Surgery is effective in eliminating the headaches and the morbidity is generally low. Surgical exploration for a focal CSF leak, as demonstrated on radiographic studies, usually does not reveal a clear source of the leak. Some patients may have multiple simultaneous CSF leaks.
Article ID(s) 9452231 (PubMed)
10.3171/jns.1998.88.2.0243 (DOI)
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1995



Article Not in Favourites List Article not in Must-Read List  Spontaneous intracranial hypotension from a CSF leak in a patient with Marfan's syndrome.
Publication Journal of neurology, neurosurgery, and psychiatry 1995 Nov; 59(5) (Publication year: 1995)
Author(s) Davenport R J, Chataway S J, Warlow C P
Abstract A patient with Marfan's syndrome had spontaneous intracranial hypotension secondary to a proved CSF leak. It is postulated that the leak was caused by minor, unrecognised trauma rupturing spinal arachnoid diverticula. The diverticula were probably pre-existing abnormalities complicating the Marfan's syndrome. It is concluded that patients with spinal meningeal defects may be at increased risk of developing CSF leaks, possibly secondary to unrecognised trauma.
Article ID(s) 8530937 (PubMed)
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