POTS caused by cfs leak?

[jayut]

Had anyone out there with pots later discovered they have a cfs leak?

What happened? Did the cfs leak get treated? Did pots symptoms resolve?

 

[bombsh3ll]

I recently got checked for this as I have splitting orthostatic headaches, EDS and my illness was triggered by a forceful valsalva. I was so hopeful to have a treatable cause, but my MRI with gadolinium was negative. 

I am so glad I excluded it though. Obviously no test is 100% but it has pretty high sensitivity which is good enough for me.

If you think this could be you, I would really encourage seeing a neurologist knowledgeable in CSF leaks. 

B xxx

[GasconAlex]

CFS leaks are tricky, and the MRI is no way definitive on this, except for huge leaks. Smaller and intermittent leaks are missed.

The headache neurologist I saw was 100% confident in the MRI, even though I had a confirmed CSF leak (by surgery) and the MRI 3 days before suggested there were no problems. She dismissed any possibility of a leak because of the new MRI.

The only headache relief that worked was a blood patch, this worked for 36 hours before stopping abruptly. During that time I was still orthostatically intolerent with some bp issues - but no headache.

The ENT who is now treating me believes that I have another CSF leak from a different skull base defect and will (hopefully) be operating in July. They however said that 20-30% of nasal CSF leaks do not show up on the MRI. They are also not considering any neuro or pots symptoms for their diagnosis only ent ones.

I (and some of my doctors) are considering that there may well be an underlying problem, probably eds that is causing repeated problems. So even if the next leak gets fixed the pots might well still be there. But if fixing the leak fixes the headaches that would be great.

There is a huge disagreement on this within the professions, the neuro surgeon believed the symptoms justify a blood patch (hurts but not hugely risky) and if that makes things better then his view was that is confirmation. Some neurologists say only a spinal leak can cause the headaches, some believe cranial leaks cause problems but not by low pressure. There are many opinions so look at your case carefully and don't necessarily accept the first assurance you come across - it depends on your particular symptoms.

If you can get an opening pressure reading from a lumbar puncture / blood patch that can help. I've never had this despite numerous lumbar punctures and blood patches, but some doctors say it is useful in CSF leak diagnosis.

[GasconAlex]

Neurology Reviews. 2017 March;25(3):23-24

Imaging of patients with CSF leaks often initially is read as normal, and MRI is not an adequate evaluation in the high clinical suspicion of a leak, Dr. Carroll said. “It is a good place to start, because if you see a leak on your MRI, maybe you do not have to get a CT myelogram,” he said. “But if you have a clinical suspicion of a leak … you should pursue that in the face of your radiologist telling you that there is nothing.”

[jayut]

It is my understanding that MRI for cfs leak is very specific but has incredibly poor sensitivity.

https://spinalcsfleak.org/about-spinal-csf-leaks/diagnosis/

[jayut]

Fresh off the presses. Dated May 22, 2019.

https://www.ncbi.nlm.nih.gov/m/pubmed/31118385/

[jayut]

Also according to Dr. Kranz video. Imaging becomes less reliable the longer the leak had existed.

https://spinalcsfleak.org/symposia/symposium-2018/

 

Upon reflection, I first started having orthostatic headaches in 1997 after injuring my lower back while working out. It became full blown disabling POTS after another back injury in 2014. Could I have had some low level leak since 1997?

[jayut]

SIH and cfs myths.

https://www.ncbi.nlm.nih.gov/m/pubmed/29797515/

[jayut]

Cfs leak Q and A with Dr. Schievink.

https://www.csfleak.info/what-is-a-cerebrospinal-fluid-csf-leak/csf-leak-questions-and-answers-schievink/

 

[jayut]

Another study linking cfs leak and POTS. 

https://journals.sagepub.com/doi/full/10.1177/2515816318773774

[JimL]

the DINET website has a video on this. 

 

[jayut]

Does anyone get teeth pain, facial pain, facial tingling, scalp tingling, pressure or fullness in ear(s) after standing for a period of time?

If so, do all these symptoms subside after laying down, completely flat, for 30 minutes?

[Pistol]

@jayut - the only time I experienced scalp tingling ws when I took Midodrine - are you on that by any chance?

[jayut]

2 hours ago, Pistol said:

@jayut - the only time I experienced scalp tingling ws when I took Midodrine - are you on that by any chance?

I trialed Midodrine and it didn't do anything positive or negative.

[bombsh3ll]

On 5/27/2019 at 8:50 PM, jayut said:

Fresh off the presses. Dated May 22, 2019.

https://www.ncbi.nlm.nih.gov/m/pubmed/31118385/

 

On 5/27/2019 at 1:47 PM, GasconAlex said:

Imaging of patients with CSF leaks often initially is read as normal, and MRI is not an adequate evaluation in the high clinical suspicion of a leak, Dr. Carroll said. “It is a good place to start, because if you see a leak on your MRI, maybe you do not have to get a CT myelogram,” he said. “But if you have a clinical suspicion of a leak … you should pursue that in the face of your radiologist telling you that there is nothing.”

Thanks for the shares guys.

I've come back around to this after further research & consulting with another couple of specialists.

Alex I know you've had successful treatment but what about you Jayut, have you pursued this line further?

My MRI contrast was normal but I am not letting go now without further imaging & preferably a couple of blood patches, given I have such classic symptoms, a clear precipitating event known to cause spinal leaks, before which I was completely normal, and a connective tissue disorder. 

B xxx

 

[GasconAlex]

@bombsh3ll the treatment helped, but never touched the POTS. I think I always had this but was never symptomatic, again a history of a connective tissue disorder but I have it mildly (my boys are showing much more in the way of symptoms)

After some success now I am more vertical I am leaking again, but from a different point. I do have a little nasal discharge but mostly only after I've done way too much. I have a metallic taste issue, orthostatic headaches and muscular rigidity and tremors. The skull base surgeon is leaving it 3 months, then he'll do an MRI, then try to fix this new different leak. The MRI is expected to show nothing as the leak is intermittent and much better when lying down. So lying down in the MRI means the leak is very unlikely to be active.

Looking around there is quite a bit of stuff that shows cranial leaks can go unremarked with no exterior signs or images (like my first CSF leak) and suffer from the same headaches etc. as spinal leaks.

 

 

[bombsh3ll]

5 minutes ago, GasconAlex said:

the treatment helped, but never touched the POTS. I think I always had this but was never symptomatic, again a history of a connective tissue disorder but I have it mildly (my boys are showing much more in the way of symptoms)

Sorry your boys are dealing with this too. I think watching your children suffer must be harder than having it yourself. When you say treatment never touched the POTS, are you referring to numerical heart rate changes or how unwell you feel upright? I am only concerned with the latter (& my HR is normal anyway). 

How frustrating that you have started to leak again, still at least you should be able to obtain treatment more quickly now that the surgical findings were proven last time.  How can you tell it is from a different site without it showing on imaging?

For me, nothing leaks from my nose, I believe a spinal leak is likely. You are right most of the literature doesn't describe cranial leaks as causing the splitting orthostatic headaches, but I believe textbook descriptions, particularly concerning rarer conditions, originate from an era where patients' symptoms were not listened to much, or discounted if they didn't fit an expected pattern. 

I think a similar thing happens with spinal leaks in that the lightheadedness & fainting is often not described in medical literature, yet having reached out on CSF leak forums to people with proven leaks, it appears to be fairly common. My guess is if you go to a headache clinic, syncopal complaints are ignored, & if you go to a syncope clinic, headaches are ignored, because they only look at their own specialty not the whole picture. Also in my case nobody listened to the fact that I was completely normal prior to a forceful valsalva. 

I hope you can either reseal spontaneously or get a successful repair more quickly this time. Again thanks for the info. Whilst I don't yet know conclusively if I have a leak at this point, I would put money on it that others on here do, & may not realize, particularly if they were fine until some trauma like me or childbirth with epidural etc. then suddenly couldn't be upright. 

B xxx

[GasconAlex]

@bombsh3ll the pots symptoms are just ridiculous heart rate changes. I feel pretty awful being vertical and it gets worse the longer I'm up. After the operation (5 days absolutely flat including eating, toilet) felt much better. Then had a month mostly flat no effort. When I started being up more it hurt more, but I don't want to be horizontal all day so take pain pills for the headaches and when too much lie on floor / bench or recline wheelchair. ( I have balance issues and mobility issues).

The leak was right sided, now left sided so is new. The surgeon has little expectation that the patch will be lifelong as 'I have very little bone there', if / when it leaks again on that side I can look at other options but in absence of definitive imagery he operated to look and fix rather than having all the repair options available.

Neurologists in particular seem to be very compartmentalized, the headache neuro looked at only the headaches, the movement ones ignored the headaches, the dysautonomic one showed the mild dysautonomic dysfunction but  that's all. The local general neurologists are approaching retirement and not interested in 'new' stuff. I found a goodish one but since he cannot see the link between leak fixed - rapid improvements, leaking again problems restarting (he see's only the improvement so carry on.....) and why should a CSF leak cause odd neuro dysfunction? At that point gave up as a bad day and will speak to my nice gp.

The great thing about it being leak related as it isn't neuro degenerative - which when I cannot think clearly is a great help!

Hope you find a solution but it's taken 3 years for the leak diagnosis even when I'd already had a CSF leak surgically proven. The difficulty in my view is that with better imagery many doctors look at the pictures and not the patient.

 

[bombsh3ll]

56 minutes ago, GasconAlex said:

Neurologists in particular seem to be very compartmentalized, the headache neuro looked at only the headaches, the movement ones ignored the headaches, the dysautonomic one showed the mild dysautonomic dysfunction but  that's all. The local general neurologists are approaching retirement and not interested in 'new' stuff. I found a goodish one but since he cannot see the link between leak fixed - rapid improvements, leaking again problems restarting (he see's only the improvement so carry on.....) and why should a CSF leak cause odd neuro dysfunction?

How frustrating! I was quite familiar with the game of medical "hot potato" during my career, when multiple specialists would each argue that a complex patient didn't quite fall within their remit, or were only interested in addressing one piece of a larger puzzle, and now experience the same as a patient  

1 hour ago, GasconAlex said:

The great thing about it being leak related as it isn't neuro degenerative - which when I cannot think clearly is a great help!

Yes, I just threw an opened but full sachet of trioral in the bin! I just hope it hasn't caused any lasting damage being like this for so long. People with diagnosed leaks are advised to lie flat in order to prevent excess traction on the delicate structures in the absence of a fluid cushion, but people with POTS are advised to force themselves upright as much as possible, & I have been doing the latter for 5+ years (without it becoming one ounce more comfortable or tolerable).

1 hour ago, GasconAlex said:

The difficulty in my view is that with better imagery many doctors look at the pictures and not the patient.

That's true right across the board sadly. When I was at medical school there was a saying that 80% of the diagnosis comes from the history taking, the other 20% from examination & investigations. I think that has been forgotten now. The guy I am going to see later this month though, Dr Simon Ellis, wrote the UK CSF leak association booklet in which it states MRI misses a proportion, so I am hopeful he will be worth the money! I spoke to another lady here (also diagnosed with POTS) with classic upright headaches of sudden onset & borderline HR changes whose imaging was all reported negative & he patched after collaborating with Dr Gray at Duke in the US, & she is now upright & working again.

Hopefully in time the arena of synthetic/cadaver/animal derived tissues will develop so you may have more options for repairing the thinned area of bone. 

B xxx