Alphabetical Guide to CSF Leak Terminology

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Struggling with phrases and acronyms?

Health-related terminology can be confusing at times, particularly to those without a medical background, with limited experience dealing with doctors or hospitals or who are new to a particular condition or illness.

In order to assist, we have prepared an alphabetical list of some of the most commonly used CSF leak terminology.

Check out our new alphabetical guide to CSF leak terminology.

Living with Intracranial Hypotension: Claire Hubbard’s Story

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Before becoming poorly in Dec 2009, I was a happily married, fit and healthy 43 year old with 2 teenage daughters. I collapsed one day for no reason and had not been right since. I would blank out and collapse in a headache attack after being upright too long, resulting in many visits to A&E. After suffering for nearly four years and almost every test you can imagine showing nothing, last July 2013 we decided that enough was enough and to take the bull by the horns and do something once and for all. Final decision. It turned out that was the best decision we had made.

I had been diagnosed with POTs in 2012 but felt it was not quite the right diagnosis for the sort of symptoms I was feeling. I had not got the tachycardia or dizziness many people with POTs have and my heart rate only just increased to the POTs threshold of 30 beats per minute when I stood up. I did have low blood pressure and had spent most of 2010 lying flat, which could have prompted the POTs like symptoms. I also had a daily headache, but mine was ongoing – 24 hours a day, every day. It was lessened in the morning but gradually got worse as the day progressed.

Every day I spasmed or jerked if I was upright too long or did too much. I would have to sleep every day for an hour at lunchtime in a completely flat position just to be able to continue with anything in the afternoon. If I did not, my head pain would simply increase till I had to lie down and spasm or jerk in pain.

My turning point was to pay privately for one consultation with a neurologist at my local hospital. We had to persuade my GP for the referral, as I knew it was my last chance and he said I had seen enough people. But, my new neurologist listened…

He was very interested in my symptoms and sent me for a series of tests – some of which I’d had before. All came back negative for a CSF leak. I would tell anyone who thinks they are leaking to persevere with doctors, as, if they can’t see a leak, they will say you haven’t got one, even if it is a slow leak and not likely to be seen.

I did my research. I read Dr. Schievink’s Question and Answers and highlighted anything that referred to myself. I even went on a POTs medication trial for a fortnight whilst in hospital to see if that would help. Once we had exhausted all the doctors’ ideas, including many of the anti-seizure medications, (all of which made my symptoms worse).

In February 2014, I lifted a heavy weight and carried it some distance, feeling that something had gone wrong as the pressure in my head suddenly grew. I went home and 36 hours later I could not get up at all without severe pain. It took 2 days to get hold of anyone who would help me and I was prescribed phyllocontin as it is a form of theophylline that increases csf. I was up again in 36 hours, but after taking the medication for 3 weeks and got gradually worse and worse until I was having repeated painful non-epileptic seizures – as in 8-11 in a row. I ended up in hospital where they did not know what to do with me. I was seizing at first 3-5 times a day, reducing to three times a day later on. Every day was the same and it was awful. I persuaded the UK doctors to try a scan that I hadn’t had after emailing Dr Linda Gray in North Carolina, USA. I had already had contact with her as I had asked for a quote to see how much money I needed to raise in order to get a second opinion. I asked her what she would do for me that the UK doctors may be able to do.

She said she would scan me and look for any potential leak sites. The scanner she uses is a DSM scanner and my hospital had not got one, but as it is a fairly newly built hospital building, they had the latest MRI scanners up to 3 tesla. Many MRI scanners are 1 – 2 tesla so I knew this scanner would be as good as a DSM scanner (hopefully). The test she recommended was a fat saturated MRI myelogram, using intrathecal gadolinium. My neurologist immediately said it must be done and went to organise it. And this is where it gets tricky. It was proposing to use gadolinium off license and so had to go through 3 weeks of protocol and paper work. I was desperate for help.

Eventually the test came. The way it was organised was to do 3 MRIs, one before the gadolinium was introduced and two after. Each MRI lasted 70 minutes. We split it up so the first one was done on one day and the next two on the next day, as it was a long time to stay absolutely still. This was completed Friday afternoon about 6pm. I would be lying if I said it was not hard work. To stay still in the noisy MRI for that long is difficult, but I knew I must do it if I wanted any chance of any leaks to be found.

The next day I was told the test showed nothing. I was surprised as it was the morning and I was trying to work out who had looked at them in so much detail to ascertain that information in such a short space of time.

So, the next step for me was to get a copy of the scans, to get a second opinion. Again, this isn’t easy. You have to apply to the ministries department at our hospital and pay £50 for your written records and scans. As an inpatient, the scans were not allowed to be released. I had to fight for this by asking the Chief Executive of the hospital to intervene, as my neurologist was more than happy to get the second opinion from the States. This took a while but they did arrive.

I have a friend who was able to take the discs and link them as a hyperlink to his Drop box account. This enabled me to simply email Dr Gray in USA with the link and password. I was the go between for my neurologist and Dr Gray for many days. At first she couldn’t open them, then she could. Then she sent the email that made me take a huge sigh – she could see potential leak sites and could name some of them. She wanted to see the most recent scan too as she couldn’t open them. This took a while with instructions and print screen images of how to find what she wanted. She advised my neurologist to do a full volume patch at the cervico-lumbar and then mid-thoracic region.

When I discussed this with my radiologist, he said the areas were too wide for him to think I could get any gain. I asked Dr Gray for yet more information. I offered to pay for her services all along, yet she would not accept anything. She replied with a comment of doing the same as before but adding a lumbar region patch too, as the UK would probably not be able to do CT fluoroscopic targeted blood patches at any diverticula that could be a problem and that she would look at me when I could get over there.

My radiologist took my scans home for the weekend and then on Monday, he gave me the choice. Three large blood patches or many targeted ones – he said that they could do that but with blood, not fibrin glue. The latter would be more painful and take longer. I decided to go for the targeted ones, as that is what Dr Gray would do in the USA.

It was not an easy procedure. I was asked if I would be able to lie absolutely still for it. As it was in the morning I felt confident I wouldn’t seizure as any seizure activity had started developing in the afternoon. After the lumbar puncture and injection of dye, I was asked to roll 15 times lengthways on the bed to mix the dye with the csf fluid. This is easier said than done. After that I lay on my front on the CT scanner and was asked not to move at all whilst being scanned and whilst the needles and then blood was inserted.

This is really important. If you move, the blood patches will not go to the targeted areas and will be useless. I had numerous needles put all down my back, from my shoulder blades to my lower back. Most of these were in pairs but some were single. The needles were inserted after an initial local anaesthetic on each one. My radiologist was meticulous to keep them in the exact right place. Sometimes a nerve was touched or tickled, but I still was not allowed to move. When all the needles were in place, the blood was syringed out of my arm and into the needles, about 5cc at a time. A lot of that ran out and down my sides! Some of the inserted blood was quite painful.

After each needle was filled, they were all removed and it was over. I lay head down in a gentle tilt for 2-3 hours before I sat up and ate my tea, feeling much better. I did not spasm, jerk or seizure from that moment onwards.

I am now at home, at the moment, 2 weeks in and symptom free. Totally. I’m no longer noise, light and smell hypersensitive. I can stay upright all day if I want to and can drive. I feel free and released. I know I’m not healed yet and so need to take it easy. I am planning to see Dr Gray in North Carolina as my neurologist thinks it would be a good idea to get a second opinion and maybe fibrin glue patching. I’m planning to go out in about a month’s time.

If you want any recent updates, please see my blog on: www.lucyhubblog.wordpress.com

Claire Hubbard

Anonymous donor gives £10k to fund Eccleshall woman Claire Hubbard’s vital brain scan

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SERIOUSLY ill Claire Hubbard could soon fly to the U.S. for pioneering treatment on her brain – after receiving a £10,000 anonymous donation.

Mother-of-two Claire, from Eccleshall, has been diagnosed with intracranial hypotension, which causes brain or spinal fluid to leak. It means she suffers from constant headaches as well as daily seizures.

The 47-year-old’s only hope is to travel to America to meet a specialist and undergo a scan to pinpoint the leak.

Her friends and family have been trying to raise at least £13,000 to help meet the costs.

Read the full story here: http://www.stokesentinel.co.uk/Anonymous-donor-gives-pound-10k-fund-Claire-s/story-20963083-detail/story.html#ixzz2zMG8QJaq

CSF Leak Questions Answered by Dr Schievink

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We’re pleased to announce that with the kind permission of the CSFleak.org Forum, we are able to publish a recent Questions and Answers session with leading CSF leak expert, Dr Wouter Schievink. The questions were posted by members of the CSFleak.org forum during January 2014 and answers were provided on 4 April 2014. We are deeply grateful to the administrators of the forum for arranging and managing the Q&A session and for making the information available.

Dr Schievink is one of the world’s foremost experts on CSF leaks and Spontaneous Hypertension. Originally from The Netherlands, but now practicing Cedars-Sinai Hospital in California, he has conducted a number of studies into the diagnosis and treatment of CSF leaks and many specialists in the UK base their approach on his work.

This valuable information sheds light onto many of the questions and issues faced by leakers across the UK and Ireland. Dr Schievink’s advice will also be of relevance to GPs and other medical professionals dealing with patients presenting with persistent daily headaches and other symptoms of intracranial hypotension.

You can view the Q&As online by clicking here or download them by clicking here.

Cedars-Sinai neurosurgeon successfully performs CSF repair surgery on patient

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When Elizabeth “Beth” Johnson stood up from working at her computer Oct. 28, she noticed “a kind of funny feeling” in the back of her neck. By Nov. 2, the sensation had become an excruciating headache that came on whenever she tried to stand or sit upright.

Her symptoms and MRIs suggested that a hole had developed in the dura, the lining around her spinal cord. With the loss of cerebrospinal fluid – which circulates around the brain and spinal cord – her brain shifted and sagged away from her skull when she tried to stand, causing severe positional headaches and eventually leading to periods of confusion.

After two attempts at another neurosurgical center failed to patch the hole, Johnson, who lives 13 miles from Santa Fe, N.M., came to Cedars-Sinai, where Wouter Schievink, MD, director of the Microvascular Neurosurgery Program in the Department of Neurosurgery, surgically repaired the tear.

Schievink, one of the world’s most experienced neurosurgeons for this diagnosis, said Johnson’s tear was extensive but no larger than many he has treated. It was, however, located on the front side of her spinal cord, making surgical access more challenging and the repair procedure more delicate.

When Johnson – who describes herself as a fast-moving, quick-thinking Type A personality – felt the strange sensation in her neck, she didn’t slow down at first. But as symptoms got worse, she wondered if she should go to an urgent care center. Friends surmised she was just stressed out and recommended therapeutic massage. But by Nov. 5, no longer able to sit or stand without pain, she saw her primary care physician, who immediately sent her for MRIs that showed she had spontaneous intracranial hypotension – a defect in the dura had opened, allowing spinal fluid to leak.

Two days later, Johnson was flown by air ambulance to another neurosurgical center, where doctors used injections of her own blood to try to seal the tear – the first line of treatment for most leaks. Although she was discharged to go home Nov. 15, Johnson and her partner of 14 years, Charlotte “Char” Schnepf, quickly realized the blood patches hadn’t held.

“When I called the doctors, they said to come back and have a third blood patch. They said sometimes people need 10 of them. But this didn’t make sense to me,” said Johnson, manager of a family compound and a life transitions coach.

At the suggestion of a client and friend who had found Schievink’s name online, Johnson called Cedars-Sinai; Schnepf wrote a letter, collected copies of the original MRIs and shipped them overnight to Schievink.

“He looked at my MRIs and called me the next day,” recalled Johnson, who again was flown by air ambulance, but this time to Los Angeles Monday, Dec. 2. She met Schievink that afternoon and began undergoing a series of diagnostic procedures. They confirmed the defect’s location at the front of the spine and showed a calcium deposit on a disc, which may have contributed to the tear.

Read the full article here: http://www.news-medical.net/news/20140228/Cedars-Sinai-neurosurgeon-successfully-performs-CSF-repair-surgery-on-patient.aspx

New Factsheet – What is a CSF Leak?

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We’re pleased to announce the publication of the first in a series of CSFleak.info factsheets – What is a CSF Leak? – which can be downloaded via our downloads section or by clicking on the button below.

Our factsheets provide easy access to key facts and information; they are printable and can be taken with you when you meet with your GP. They are provided in good faith and following careful research, however they cannot be guaranteed error-free and are not a substitute for professional healthcare advice.

Diagnostic Criteria for Spontaneous Spinal CSF Leaks and Intracranial Hypotension

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Authors: W.I. Schievinka, M.M. Mayab, C. Louyc,  F.G. Moserb and J. Tourjeb

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.

Copyright © American Society of Neuroradiology

Read more here: http://www.ajnr.org/content/29/5/853

Role of MR Myelography with Intrathecal Gadolinium in Localization of Spinal CSF Leaks

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mri-gadolinium-contrastA, Pre- and post-intrathecal gadolinium fat-suppressed T1 images demonstrate typical artifacts, which may simulate a leak at C1-C2 seen in 6 of 41 of our patients. Note the inhomogeneous fat saturation on this precontrast sagittal T1 image at C1-2 (arrow). B, Recognizing this artifact on precontrast imaging is important because with the addition of intrathecal gadolinium, this inhomogeneous fat saturation can potentially mimic a leak (arrow).

Full article and images available here: http://www.ajnr.org/content/33/3/535/F2.expansion.html

Detection of CSF Leak in Spinal CSF Leak Syndrome Using MR Myelography

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cisternography-results-slide

Schematic drawing of 4-point-scale grading system of CSF leak on MRM (A) and RIC (B). A, Our grading scale on MRM is depicted as follows: grade zero, no leak (normal findings on MR myelogram); grade 1, possible leak (expansion of the CSF space column around the nerve root sleeve); grade 2, probable leak (streaky hyperintensity lateral to the nerve root sleeves but with length less than the transverse diameter of the thecal sac); and grade 3, definite leak (lateral extension greater than the transverse diameter of the thecal sac). However, actual grading of the case is determined on the basis of the highest grade in each level of the spine. B, Grade of CSF leak on RIC is depicted as follows: grade zero, no paraspinal activity; grade 1, possible leak (faint paraspinal activity with length under the transverse diameter of spinal canal activity); grade 2, probable leak (hot paraspinal activity with the length under the transverse diameter of spinal canal activity); and grade 3, definite leak (hot paraspinal activity with the length over the transverse diameter of spinal canal activity)‏

Full article and PowerPoint presentation available here: http://www.ajnr.org/content/29/4/649/F1.expansion.html

The Value of Changing Position in the Detection of CSF Leakage

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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.