Pots or leak?

[Amarilis]

Dear Doctor Diana,
I’m housebound due to a series of symptoms which at the end have been labelled as a csf leak with negative mri. But i read that pots have very similar symptoms. I have a story of neurological problems so a leak is possible, but how can you differentiate them? Do they both give autonomic issues?thank you

[Dr. Diana]

Hi Amarillis, How interesting — I don’t know that I’ve heard of anyone else having a diagnosis of a CSF leak (spinal) without any evidence of it. I believe that certainly occurs (leaks can be occult or intermittent), but how would someone make that ‘diagnosis’? Perhaps it is considered “presumed”? I’m eager to hear what others may think. As far as symptoms, the number one symptom found in spinal leaks is the WORST HEADACHE EVER when vertical. The headache is relieved when lying down. Does that happen to you?

[spdsk8trgrl]

So I have a very similar problem. I have symptoms of low CSF pressure/volume that have been chronic for at least 5 years and are getting much worse recently. I have constant numbness and tingling in my forehead, worsens to pain and stiffness in my neck, pain at the back of my head, tingling and pain in the top of my head, and numbness and tingling in my hands, legs, and feet as soon as I stand up in the morning. The symptoms increase over the day, and I can notice some relief of symptoms by lying down. Exercise makes things much much worse, and will generally trigger a “worst headache ever” migraine within a few hours. Also triggered by weather, altitude, and hormones. As the symptoms worsen, I get a distinct feeling of suction inside my head basically behind my sinuses. When it is really bad, I can sometimes feel the “bubbles” when I lie down and some of the areas equalize in pressure. It is just like when you clear your ears for altitude, but behind the sinuses.

At first I was unsure if my symptoms were more indicative of low or high CSF pressure. I have distinct worsening of symptoms with bending over and with any Valsalva maneuvers, which is supposed to be more for high pressure. I got my PA at my neurologist to let me test Diamox, and I got distinctly worse feelings of suction, so I’m fairly sure I have low CSF pressure, possibly with a leak. I have quite a few instances of trauma and car accidents over the years starting with passing out on a driveway chin first at age 13, and headaches first started in my teens, so there is the possibility of a trauma induced CSF leak. I have also had tinnitus that worsens when the headaches get bad as long as I can remember.

I have done extensive research on CSF leaks and low pressure symptoms, and it is interesting that the longer the symptoms have been chronic the less likely it is to be associated with positional worsening, meaning that the distinct worsening to very severe symptoms when standing is lost. I was able to reduce the daily symptoms by strictly limiting exertion for a few months, but they returned full force the first day I added any exercise back in, and have remained back at the severe daily level since, which to me also indicates a possible leak. I had a MRI with contrast done again in December 2014 with nothing notable for CSF pressure low or high, but according to my research there are a significant percentage of patients that show nothing on MRI especially when the leak is chronic for extended times. There is a study out there that actually shows that the imaging is distinctly different depending on the time since the leak began, and shows how easy it is to miss the “window” when you might see something on imaging. I also had a lumbar puncture to test pressure in January 2015 with no notable results. This is also known to be possible to be in normal range but still have a leak or low pressure/volume.

I have been diagnosed with MCAS, mostly evidenced by a sensitivity to many medications like opiates and NSAIDs as well as insect venom. I started daily antihistamine treatment summer of 2013, winding up on a particularly high dose of hydroxyzine daily and noticing an improvement in the daily facial numbness and headaches. I started taking the mast cell stabilizer cromolyn sodium August 2014, and had an additional improvement in numbness and headache control. I have no real idea of how these medications are impacting my low CSF symptoms other than possibly lessening the nerve reactions?

I recently found out that EDS likely runs in my family. I probably had enough signs as a kid to be diagnosed, but due to limited activity over the past number of years I’ve become quite stiff. My mom and daughter have pretty classic signs of EDS, though. This is important because connective tissue disorders are implicated in increasing possibility of CSF leaks through tears in a weakened dura. Also important because “brain sagging” is more likely from the reduced pressure/volume with EDS, putting more pressure on cranial nerves.

I also now have several endocrine issues, which tracks with CSF pressure issues causing pituitary dysfunction. Of course, when I consult with “real” endocrinologists I get laughed out of the office because my test results just don’t indicate things like full adrenal failure. I am fortunate to have an integrative doc who is willing to treat my hypothyroid with compounded t4 (I react to synthroid, armor, and tyrosint). He also diagnosed my critically low cortisol via saliva tests and I’ve at least improved the deathly fatigue with cortisone replacement treatment. I have had a high result on prolactin, which I’ve seen mentioned in research on CSF leaks/low CSF pressure. I also had a very low in normal range ADH (anti-diuretic hormone) which could be increasing the problems by keeping me more dehydrated because I immediately pee out everything I drink. I even have times where my skin dents from just momentary pressure. I’ve been diagnosed with Hashimoto’s hypothyroid for 15 yrs.

I am getting my neurologist to order a full spine and cervical myelogram, which is supposed to be the best chance of seeing a leak on imaging. If nothing is visible from that, I plan to have my imaging records sent to Dr. Linda Gray-Leithe at Duke who is a neuroradiologist that specializes in detecting CSF leaks. Apparently she is able to see things on imaging that are missed by most everyone else. She performs mainly epidural blood patches for repairs I think. Another option is Dr. Schievink at Cedars Sinai, who is also very experienced with leaks and does surgical repairs.

I am basically driving this bus myself to try to get a definite diagnosis and possibly attempt a repair. It is exhausting and frustrating, and really difficult to constantly go up against disbelieving doctors to get what is necessary. I am putting this info up here hoping that my research and experience might help others.

Leslie

[spdsk8trgrl]

I also get small amounts of clear nose discharge when leaning forward or exercising. It is not enough to properly test for CSF fluid, though. I attempted to collect enough over a week, and results were negative.

Leslie

[Dr. Diana]

Hi Leslie, Sadly, I think you are in good company here, as many of us can relate to your journey! I just wanted to throw out something for you and your doctors to consider, OK? Many of us appear to be having leaks that are secondary to the combination of HIGH ICP and weak dura. What can happen is that the leak is found, repaired, and the high ICP comes bounding back. As you know, the symptoms of high and low ICP can be difficult to distinguish, and the presentation can be easily missed. Many of these patients do best if the leak is repaired, then Diamox is immediately started to prevent the rebound ICP. Does that make sense? Hang in, my friend, and please keep us posted! 😉

[Dr. Diana]

Amarillis, Just to clarify — I hear about patients suffering from “presumed” leaks all the time, but never a “diagnosis” of a leak without any confirmation on imaging. Does that sound like it is splitting hairs? 🙂 It may be important because if it can’t be confirmed with imaging, we need to figure out if it is occult (perhaps leaking from the tail bone and can’t be seen on imaging), intermittent (perhaps only occurring with Valsalva, for example), or is it secondary to HIGH intracranial pressure? High intracranial pressure (especially when combined with weak connective tissue) can cause a leak, but if the underlying cause of HIGH ICP is not treated properly, the leak will return after repair. Ugh. What a journey, right?

[susan_sk]

I have both POTS and a CSF leak. The CSF leak made POTS much worse. I can distinguish between the two because my heart rate only goes way up when I stand but is pretty much OK when I sit. When I sit I got a progressively worse headache. The limited sitting time was the csf leak. The feeling icky and faint when I stand is POTS. I had the leak surgically repaired. POTS improved substantially but it is still there. My doctor (Dr. Schievink at Cedars Sinai) says many small leaks do not show up on imaging, but a positional headache that comes on slowly over time is likely a leak. A headache that you get whenever you stand up is more likely POTS.

[Dr. Diana]

I’m so sorry you are having to deal with this, too! 🙁 Can I ask you if your doctor has any plan for treatment for your small leaks, or do they “let ’em ride”? 🙂

[miagracie]

Dr. Driscoll,

First of all, THANK you for all you do for us and the info. It is making a huge difference in my quality of life.

Wearing a soft collar to bed and elevating my head (or adjusting it more in the night if I feel pressure) as well as the zyrtec/zantac combo is giving my a pretty good amount of relief. Not total, of course, but more than I’ve had in many years.

In this post on CSF leaks I have a few questions…

I’m pending my EDS Classical/1 diagnosis (awaiting final confirmation and exam)w/a Beighton score of 6…

anyway…

I spontaneously get clear fluid unrelated to infection or bathing leak out my ears. It doesn’t seem related to the headaches necessarily.

The other day I over did it and I was rubbing my neck muscles to relax them and suddenly fluid started coming out of my ear.

My eardrum is intact. I do get earaches but they look perfect. No fluid bubbles behind the drum.

We purchased an otoscope and my husband can watch the fluid leak in my ear, the eardrum is intact and dry, but about ½ way into my ear canal he can see a bubble drop of liquid forming and seemingly come through my skin.

I’ve tried to test it with a glucose meter, but am never able to collect enough, even though it sometimes runs out of my ear.

It’s not a lot, but enough to feel weird. I do occasionally get a very minor clear fluid from my nose, but am unsure if it’s allergies or not but it does seem clearer and thinner than my sinus drainage down the back of my throat which is pretty constant.

I tend to think I have CSF buildup as stated in the Driscoll theory. With all the signs and issues. Unconfirmed, but nothing else fits the symptoms.

Once after having a headache for a week, I felt pressure and fluid leaking inside me, down the back of my neck and spine with a whoosh and a rush and the pain felt much better, I know it sounds nuts though. It felt very much like the fluid flowing out of my ears but in my spine. I’ve never felt anything like that before or since.

My headaches either start as overall pressure or in my neck at c1-c2 and then into my head. Valsalva maneuvers hurt severely: laughing, crying, singing, blowing my nose, leaning forward, etc…)

I have not yet been evaluated for possible Chiari. I think it would be Chiari zero, since it does sometimes get better and I don’t have significant neuro deficits. (Can still heel walk, heel toe/drunk test quickly, the hand shift test…)

Anyway, any thoughts on the fluid coming out of my ears? Is there a test that you know of I could obtain that was a swab or something that only took a little of the fluid?

Thank you and much gratitude!!!

[spdsk8trgrl]

Thanks for the reply Dr. D. I have, in fact, read that high ICP goes along with leaks. Sometimes the high pressure is though to be contributory to the leak, others it is thought to result after the leak as a compensatory reaction to the fluid leaking and the pressure going low. The thing that really makes me wonder is that my son has the (what we think of as) classic progression of large head until age 2 and then sudden drop off of his growth. His failure to grow was so severe he was eventually treated with growth hormone, was not producing any of his own (pituitary anyone?). We were fortunate to find a doctor to treat his growth at all, and we recently consulted with a premier pediatric endocrinologist who was surprised that an MRI was never done. Since he has normal growth hormone now for his age, the MRI is not indicated now. He has no headaches, but is irritable and has severe difficulty concentrating. Are there other symptoms we could look for that might validate that he really should have an MRI?

@susan_sk it is really good to hear that description of the headache from Dr. Schievink, mine started with progressively worsening numbness then went to getting a pressure related headache by late each afternoon. The really frustrating thing is that I did mention the postural component when the pattern first started, but was dismissed as “everyone feels better lying down”. Then the pattern went chronic and I was in so much pain all the time that there was no longer a postural component. It wasn’t until mast cell meds that I got a little control of the symptoms and noticed that I was worsening from being upright. Since I previously had more classic migraine with aura, all my symptoms were immediately diagnosed as just a different type of migraine. Sitting on the couch seems to be the worst, something about slouching and tipping the head forward.

So nice that Dr. D has the academic chops to take this issue on with her medical background and her willingness to be the guinea pig. For my part, I’m willing to share all of my remaining brain power and the knowledge I’ve gained about mast cell disorders. I’m also willing to share my medical records and imaging, and would volunteer for any research purposes. So if you need any of my records or need to examine me, Dr. D., I’m in!

Leslie

[miagracie]

Wow, Spdsk8trgl, you sound a lot like me.

The afternoon headaches used to be a daily issue, now they tend to be more morning or middle of the night. They did usually feel better lying down (no leakage from nose/ears before or during).

The pain being more constant and posture neutral fits my pattern too. I am more aware that now there is more of a posture element to my headaches than probably before. I too had migraines, and used to think these headaches were migranes that were “different.” Now I know they weren’t.

The mast cell (zyrtec/zantac) treatment is definitely helping me profoundly too.(Thanks Dr. Driscoll!!!)

My headaches are worse slouching or laying down a lot of the time. I did notice the couch thing from laying down, not supported properly under my neck/head. Reading, doing my nails, or any task head forward is very painful and can induce pressure and a headache. (Sometimes it goes away if I take steps such as adjusting position and/or putting heat on my neck.)

The worst thing for me for inducing a headache and/or neck pain is leaning forward. Loading/unloading the dishwasher is the worst. I’m learning to wear the cervical collar while I do it, and that helps. It’s definitely something I’m doing to my neck combined with leaning forward.

[Dr. Diana]

Thank you for your kind words, Miagracie. 😉 As far as wet ear canals, I can’t tell you how often I hear patients complain of this! If you really want to see if it is CSF, a capillary tube can be used to collect just a tiny amount (assuming you have a lab that will accommodate you!). I’d also consider you more likely to have a leak from your ears if you also have episodic vertigo. Boy, did I have that for a while!! Gads. I also had “squeaky noises” (don’t laugh) that went away when my pressure went down. If your hearing is OK, may I suggest that you focus on looking into high ICP in general (have you tried to reduce it with Diamox?), rather than battle with doctors and labs on testing your ear fluid (what a dead end that was for me!). It took a while, but when vertigo, “squeaky noises”, and ear-popping sort of pressure went away, I no longer worried about my ears. I can’t tell you if your fluid is CSF, but if all corrects with the reduction of CSF pressure, I suspect you were absolutely correct.

[spdsk8trgrl]

So true migracie! I was bedridden for much of the day for almost 2 years, just couldn’t make it through the day without lying down all afternoon. This was a result of the constant pain, but also crushing fatigue. I was being actively treated by my neurologist, trying pretty much every migraine treatment out there with very little effect and quite a few medication reactions. So glad you are getting some impact from Zyrtec/Zantac. I was able to try some different antihistamines with a cooperative doctor that didn’t see any harm, and I found that hydroxyzine is my best med. I take 50 mg 4x day, which would completely sedate most people (they use 100 mg pre surgery as a sedative). When it started taking away some of the numbness and tingling, I simply dosed based on when symptoms returned. Sadly the symptoms have exceeded the ability of the hydroxyzine to control them, but it still helps me be somewhat functional. I also took cyproheptadine (Periactin) daily for awhile, which helped a little, but could be contributing to my high prolactin levels so I’ve stopped for now. Cromolyn sodium was a huge help, also. Feel free to contact me for additional help with mast cell stuff. We also have some very active Facebook groups with many knowledgable members.

Leslie

[spdsk8trgrl]

Oh, and when I was trying to test whether the fluid draining from my nose was CSF, I went to an ENT here in Denver that specializes in the repair of sinus CSF leaks. You might want to check locally for an ENT with some CSF leak experience, mine was much more aware of all the symptoms. He couldn’t do anything because my imaging didn’t show the leak yet, but is there waiting if we ever turn anything up, at least.

Leslie

[Dr. Diana]

Leslie, Your son sounds so much like ours! I was able to put it all together (as you read in the book) by studying his head circumference growth as compared to his body’s growth. My son’s symptoms, like your son’s, included behavioral problems and occurred at about 18 months of age (when the sutures of the skull normally close). Like your son, our son’s growth also stopped. It was scary as heck, as I’m sure you can relate! There are articles about how external communicating hydrocephalus can affect behavior. With his history of large head circumferences, is that enough for your doctor to perform an MRI? Likely not, but honestly, it is enough for me! 😉 Because so many of us do not have papilledema which most doctors think is necessary for high ICP, without papilledema, it is a tough sell. With our son, we trialed Diamox before his MRI (because my response was so dramatic and he was SO miserable). Then we sort of backed up and did an MRI. I wonder if your son is old enough to respond to your questions about the symptoms of high ICP? That may help… Keep up the quest, Leslie, because relieving that pressure, if it is high, is life-changing. It is just so hard to figure out if his pressure is still high… I understand that. Big hug,

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