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I recently had to put together a collection of my medical info for disability, and I was reading over my hospital letters, did a bit of thinking and online reading and came across a very interesting presentation by Dr Ian Carroll who spoke at the dysautonomia international conference on CSF leaks. 

I have EDS (only diagnosed after POTS, lifelong weak collagen/weird surgical issues but nothing disabling so wouldn't have seen geneticist otherwise), and was COMPLETELY well until a very forceful Valsalva (blowing up a water bomb I thought was a balloon) at which point life as I knew it ended instantly, and I could no longer stand or walk without passing out. Along with this came severe nausea and a splitting headache and neck pain whenever I was upright, yet lie me down and my old personality, IQ & light behind my eyes are back within minutes. 

Like everyone on here, I would so love to find a treatable cause for my POTS. I was tested for Sjogrens due to dry eyes and mouth but my lip biopsy was normal. I have seen 2 neurologists and a neurosurgeon and repeatedly expressed concern that the Valsalva could have caused HIGH intracranial pressure, but none of them (nor I to be fair - I am a GP) ever considered the opposite possibility, that I could have triggered a CSF leak with LOW intracranial pressure. 

Dr Carroll's talk went into detail about a number of POTS patients actually having this, especially people with connective tissue disorders like EDS. I know that EDS is associated with OI generally, attributed to lax vessels that don't constrict, but surely that would come on gradually, not instantaneously with one event that is a recognized trigger for spontaneous CSF leaks. 

There is another video on Youtube by a young lady called Kristin Ann who had POTS and turned out to have a leak and was fixed by this guy. Her symptoms sound like me to a T. None of the standard POTS medications have helped me either. 

I know this is probably clutching at straws, but has anyone else here had, considered or been tested/treated for a CSF leak?

I would love to hear your experiences if you have or know somebody that has.

B x

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Very difficult getting a leak confirmed. Looking towards my 10th MRI and still no general signs visible. The problem being that I was operated on 3 days after an MRI, which they were sure meninges were still intact- they weren't- and I had a leak confirmed surgically (along with some brain herniation) this meant that operation wasn't successful.

After meningitis still have problems, including postural headaches etc. However now have disagreements about causes. Neurosurgeon convinced is still leak, some neurologists think maybe, some no. The Neurotosurgeon thinks not because other than the tegmen tympani being mostly missing nothing else shows. The ENT has just run a new cone beam but that shows the ears fine but large bony bits missing in the brain / sinus border. This is with a very slow nose drip that increases when the hr goes into overdrive by standing up.

Have had a few blood patches that work for a day or two (during which time my nose runs more) then stop.

My gp is convinced its organic, some of the local neuros think might be related to eds, some say psychological - psychiatrist says organic.

So my case is a little complex, I'm leaning towards the problems of pots and eds always being there and I thought this was normal (physio says partial dislocation of jaw if do move too far is not normal) but the other problems and leak just put it over the edge.

If you have visible signs on the MRI it's easy to show a leak, if not you run around the houses again and again. The pots (from ttt) was easy to show, although the cardiologist here think pots should be an instant acceleration on standing not gradual over 2 or 3 minutes.

Good luck on trying, I'm hoping at least some of my problems are sortable.

 

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The confirmed leak was spontaneous, but linked to an ear tumor. Since then the family has confirmed eds with pots which means the rest of us have looked at the symptoms. I'm fairly certain eds is there but the doctor wants to sort out the neuro deficits and severe headaches before looking into this - which might be the underlying cause.

The otologist who examined me last was not sure where the fluid he found by rhinoscope was coming from, so needed to check. That is when the bony defects in the sinus were found. I have the next MRI in December to check, but the imaging is often not conclusive in leaks. He strongly suspects another (different) spontaneous cranial leak.

The blood patches were tried because of the lumber punctures I've had, these were fairly difficult due to low bp. The neuro who did them is now chief neuro at the local hospital, he didn't want to do the patches and only did when told to do so by his now retired boss. This makes having another try difficult to say the least.

My point is that even with issues and specialists thinking there might well be a leak, because the MRI's are not clear it is difficult to progress.

I'm doing better this year, still have lots of problems but no inpatient stays so far this year. That's a lot better than the 25 ish days last year and 142 in 2016. It's certainly easier for my kids.

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Yes it's hard having kids as a non-upright person! How frustrating that your specialists don't agree. Have you ever had a CT myelogram to look for spinal leaks? This seems to be the test favoured by Dr Carroll. 

I've been referred to Dr Tyagi, an NHS neurologist in Glasgow who has at least heard of CSF leaks/spontaneous intracranial hypotension, but I am not sure if the CT myelogram is available in the UK. If I don't get anywhere on the NHS I plan to try and get to Stanford to see Dr Carroll. 

What type of EDS do your family members have? I am diagnosed as hypermobile type based on Beighton score, but have never had genetic testing. I understand the other types have genetic tests but there isn't currently one for HEDS. 

B x

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I don't have a CSF leak that I know of. I just wanted to pipe in that I remember reading an article a while back that a woman had a slow leak that caused havoc. They kept diagnosing her with allergies because it was leaking out of her nose. They eventually found the leak, fixed it, and she was her old self again. A CSF leak can possibly cause our issues but I would think it would be fairly easy diagnosis with an MRI if the right person looked at it. 

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It's great hearing about people who were finally cured of a debilitating illness when they just found the right diagnosis!

I could be clutching at straws- don't we all long for a treatable cause to be found - but I am determined now to be fully evaluated for this. I know it probably isn't relevant to a large number of people but having EDS, onset triggered by forceful valsalva, splitting headaches when upright relieved on lying flat - I emailed Dr Carroll & he thought I had a good history for a spinal leak. 

Problem is very specific myelography imaging, read by someone experienced in this field is needed to accurately diagnose it, & even then it can be missed. 

I'll have to wait a while to see the neurologist I've been referred to re this, & I'm gathering info to argue my case for the right investigation.

This study is interesting https://journals.sagepub.com/doi/10.1177/2515816318773774

B x

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@bombsh3ll never had a myelogram, did have a full spinal MRI looking for leaks. This took over 50minutes in the MRI (from image timestamps) nothing relevant found. 

Family has HEDS, but no genetic testing done. Some suggestion of Marfan's, from a range of doctors for different family members but consensus moving towards eds. (Last year I had an angio ct looking for aortic dilation the neuro came back with 'sorry signs of dilation' cardio said ' yes but 'just' within normal so not worrisome')  I have quite a lot of dystonia, so no longer showing flexibility! 

@StayAtHomeMom depends on the leak. Constant high volume leaks are more obvious, intermittent lower volume leaks are much more difficult. 

http://dizziness-and-balance.com/disorders/central/csf-leak.html

Has some interesting stuff but also quotes that no imaging is particularly useful. They look for diffuse symptoms but these are not definite. To really look you have to examine each area closely. 

I remember the neuro intern saying ' ah we'll need an MRI'

Head neuro saying 'of what?'

Intern - 'the head'

HN - 'you can't say that, you need to look for something'

@bombsh3ll if some of your stuff gets fixed, you can probably live with the rest. Its what I'm hoping for.

Sorry this is a bit disjointed, I broke a rib on Monday and this has made life even more difficult.

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On 11/15/2018 at 5:10 PM, bombsh3ll said:

I recently had to put together a collection of my medical info for disability, and I was reading over my hospital letters, did a bit of thinking and online reading and came across a very interesting presentation by Dr Ian Carroll who spoke at the dysautonomia international conference on CSF leaks. 

I have EDS (only diagnosed after POTS, lifelong weak collagen/weird surgical issues but nothing disabling so wouldn't have seen geneticist otherwise), and was COMPLETELY well until a very forceful Valsalva (blowing up a water bomb I thought was a balloon) at which point life as I knew it ended instantly, and I could no longer stand or walk without passing out. Along with this came severe nausea and a splitting headache and neck pain whenever I was upright, yet lie me down and my old personality, IQ & light behind my eyes are back within minutes. 

Like everyone on here, I would so love to find a treatable cause for my POTS. I was tested for Sjogrens due to dry eyes and mouth but my lip biopsy was normal. I have seen 2 neurologists and a neurosurgeon and repeatedly expressed concern that the Valsalva could have caused HIGH intracranial pressure, but none of them (nor I to be fair - I am a GP) ever considered the opposite possibility, that I could have triggered a CSF leak with LOW intracranial pressure. 

Dr Carroll's talk went into detail about a number of POTS patients actually having this, especially people with connective tissue disorders like EDS. I know that EDS is associated with OI generally, attributed to lax vessels that don't constrict, but surely that would come on gradually, not instantaneously with one event that is a recognized trigger for spontaneous CSF leaks. 

There is another video on Youtube by a young lady called Kristin Ann who had POTS and turned out to have a leak and was fixed by this guy. Her symptoms sound like me to a T. None of the standard POTS medications have helped me either. 

I know this is probably clutching at straws, but has anyone else here had, considered or been tested/treated for a CSF leak?

I would love to hear your experiences if you have or know somebody that has.

B x

I am curious of something like this going on, I always get a pressure at the top and center of my brain and it feels like it moves slightly like its getting tighter and lightheaded that isnt relieved by laying down! I myself didnt know i had EDS until the POTS my EDS symptoms are nothing hardly luckily lol. 

 

I don't agree with the lax blood vessels, it seems to simple and if this were the case laying down should essentially fix all problems as everything is working mostly, have you seen any studies on this at all? My skin isn't stretchy and joints not really bendy so I can only assume the vessels would be ok too. 

 

I wish to find a cause to my symptoms not relived by being supine too!

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I'm sure disorders of CSF pressure in either direction can create problems, in fact I repeatedly expressed concern about HIGH intracranial pressure. I have been for repeat eye exams to exclude any signs of this. I am angry that the neurologists and neurosurgeon didn't pick up on the fact that splitting headaches when upright relieved by lying down are characteristic of a spinal leak & consider this possibility, especially when the patient is mentioning intracranial pressure (albeit the opposite concern), and has known risk factors - EDS, precipitating Valsalva. 

Maybe I don't have it, but the diagnosis should be considered and excluded, rather than nobody having thought of it. It wouldn't be OK to go to hospital after a car accident with severe leg pain and unable to walk, & nobody thinks of or looks for a broken bone. Many people won't have one, but it should be ruled out. 

I also don't buy it about lax blood vessels - surely this would have come on gradually. I would have had lax vessels at 16.55 on the day I fatefully blew up that water bomb, not just since 17.00 when it happened. Also my dad who also has EDS is 78 and has awful varicose veins and an aortic aneurism, & he can stand up!

B x

 

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3 hours ago, bombsh3ll said:

I'm sure disorders of CSF pressure in either direction can create problems, in fact I repeatedly expressed concern about HIGH intracranial pressure. I have been for repeat eye exams to exclude any signs of this. I am angry that the neurologists and neurosurgeon didn't pick up on the fact that splitting headaches when upright relieved by lying down are characteristic of a spinal leak & consider this possibility, especially when the patient is mentioning intracranial pressure (albeit the opposite concern), and has known risk factors - EDS, precipitating Valsalva. 

Maybe I don't have it, but the diagnosis should be considered and excluded, rather than nobody having thought of it. It wouldn't be OK to go to hospital after a car accident with severe leg pain and unable to walk, & nobody thinks of or looks for a broken bone. Many people won't have one, but it should be ruled out. 

I also don't buy it about lax blood vessels - surely this would have come on gradually. I would have had lax vessels at 16.55 on the day I fatefully blew up that water bomb, not just since 17.00 when it happened. Also my dad who also has EDS is 78 and has awful varicose veins and an aortic aneurism, & he can stand up!

B x

 

I agree. It should be something that should be ruled out. Multiple articles talk about CSF leaks and it averages 5 years for diagnosis. Seems kind of silly that it should take that for a diagnosis. 

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  • 1 month later...

I’m pretty sure this is my issue...even though all my MRIs have yet to show a leak. Or at least none of the radiologists have found the leak on my MRIs.

They've found tarlov cysts in my cervical spine, mild dural ectasisa (thanks EDS!) and 2 herniations at C4 and C6. Oh- also my latest pituitary MRI in June showed *possibly* 2 microadenomas (they said have another pituitary study done in 6-12 months...).

But no leaks. Even though I have fluid in my ears and twice nasal discharge (never duplicated in dr’s office, of course.)

Crazy enough, I have terrible edema so one of my docs gave me Lasix and the most amazing thing happened. My daily headaches went away!! (Side note: I’m on topamax for my migraines and I’m wondering if it’s affecting my kidneys causing the edema.) For a while it was definitely helping all of my POTs symptoms- best couple of weeks I’ve had in a loooooong time! However, it doesn’t seem to be a long term solution since I’m in pretty bad shape all over again. That being said, still no daily headaches.

I’ve never had an upright MRI to see if I have Chiari whilst upright (my newest neurologist said chiari has already been ruled out. He’s also said my case is too difficult for him and should find another dr......soooo.......)

The first time I watched Kristen’s story (when she was talking with Dr. Carrol), my husband and I were floored at how similar our symptoms were. I just need to find a dr who believes me and who will help me find the leak(s). 😔

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My sister has POTS and EDS (type 3).  She started getting orthostatic headaches that got worse throughout the day unless she laid down for a bit many times during the day (which made it extremely hard to work).  Because of the EDS diagnosis, they checked for dural ectasias and found a few.  She got blood patches, but she keeps having to get some of them redone once in a while.  They do not take permanently for her, but she said that they are permanent in some people.

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I think I have CSF leak as well.....I had strained quite hard on the toilet the day before I woke up with POTS.....I have had fluid running out of my left ear repeatedly during my 6 month ordeal with this horrible illness......my question to anyone who may know is this.....won't a leak heal up on it's own after a period of time ????  some said it took 5 years for a diagnosis....I wonder why the leak won't heal up on its own ???   i have had an MRI of brain and spinal cord....and have many stenosis and Bone SPURS on all my vert...so wondering if they knicked the cord..??? it is possible......

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