IIH and CSF Leaks
My Story
IIH stands for idiopathic intracranial hypertension. It’s quite a mouthful and for some, it becomes a way of life. As a rare disease, not a lot is known about it and a cure is no closer now than it was ten years ago when I was diagnosed with it. Back then though, it was called pseudo tumor cerebra. Another nice mouthful of complicated words.
That is what they tell you after I’d gotten a CAT scan, MRI and then a large needle pushed through my spine to the CSF fluid behind it. I went into the ER (several ERs actually) and I came out with that diagnosis. At the time it was devastating, I was pregnant and I wasn’t told all that much about it. Then I was told to research my new disease online and to take the pills that were prescribed. They were going to help the headache that I thought I was going to die from. I was already feeling better since the lumbar puncture.
IIH basically is too much pressure in the brain. We all make CSF fluid in our brains to suspend the organ from our skulls as we move around. Mine makes too much or it doesn’t drain enough because out of the blue my system went into overdrive and I had well over double the average amount of pressure in my head. The sterile fluid that was supposed to be protecting my brain was now squeezing it on all sides.
It feels like my head is going to explode, literally. I made the off-color comment or maybe I was being serious that I needed them to screw a hole in my head. It was more real than I could have imagined and drain shunts are one of the many options that I had to look forward to.
That was ten years ago. I had three years of Diamox and I went into remission. The headaches the pressure vanished without a trace and I had almost five years of relatively no pain. It was nice, but it didn’t last forever. The headaches came back and while I hadn’t seen a neurologist in years, I knew that I was going to have to.
I put it off though. I didn’t want to believe that it was back. I couldn’t believe it, but it was becoming clear that it was true. The IIH was back. Before I could get an appointment though, I had to move and then on the day of the move, my nose started to leak a clear fluid when I tipped my head down. It was a fluke, strange and I pushed it out of my mind, figuring that since I had moved to another state, my allergies were acting up and it would go away.
It didn’t go away though and after a couple of weeks of this nasal drip of clear, water-like fluid that tasted like the salty ocean, I realized that something was wrong. I ran across an article about leaking noses and leaky brains. It was about Spontaneous Cerebrospinal Fluid Rhinorrhea. Or CSF Rhino for short. It wasn’t a seasonal allergy post-nasal drip that was coming out, but the CSF fluid that was supposed to be cradling my brain. So much pressure over so long had made a hole in my skull and it was now leaking out.
I went from high pressure to low pressure and a host of new problems emerged. Apparently low pressure can cause a new kind of headache, one that goes away when I would lay down flat. More so, it was hard to stomach the idea that brain juice, as my eight year old calls it, was now coming out of my face. The sterile environment was in danger of any kind of germ or bacteria getting into it and that could be a deadly problem.
Getting someone else to realize the same thing took time. I went to the same emergency room that had diagnosed me eight years before with IIH and I told them what I was afraid that this drip was. They didn’t really think that I was right and called it a post-nasal drip. I was referred back to neurology and got an appointment after a couple of months.
By then I was sure that it was a CSF leak and I started to take more care with germs and sick people in general. I read up on it as much as I could and didn’t really like the options that were there. It was most likely going to have to be a brain surgery to fix it. I didn’t like the idea of that at all, still don’t.
After the initial visit, it was concluded that it was in fact a CSF leak. The drip by then was pretty substantial and it was sent for a Beta-2-transferrin test to make sure. I did another MRI that showed pooling in my sinuses. I had a leak, but it didn’t show up. There were assumptions made at where it was and talk of brain surgery.
That was two years ago and I still leak. I leak more than the 18 ounces that I am supposed to make, but I still haven’t gotten it fixed. There was another pregnancy in that time that put everything off and after more research I realized that re-leak and multiple surgeries was going to be in my future.
Right now I can get up and play with my newborn and eight year old. If I get surgery I will not be able to do so, so for now I choose to wait, hope maybe that something better will come along.
Purpose
I write this because I was told by several doctors and even brain specialist that IIH cannot cause a CSF leak. It can. The more time I spent on forums and reading up on new studies and reports done, it was far more common than I ever would have thought. I’ve talked to over fifty people that have a CSF leak from their IIH and I want to make sure that there is one more voice making it so. Most people find out about their leak after contracting meningitis, a potentially fast and deadly infection in the CSF fluid.
My own neurologist told me that it wasn’t possible, but it is. Here are some of the symptoms that can be associated with a spontaneous CSF leak from IIH, as well as needed test to get the proper diagnosis. That sometimes can be the hardest part and if you’re reading this with IIH, you already know this personally as well.
Symptoms
Clear fluid that can come out of ears, nose or mouth. Ears may feel full, instead of actually coming out. Out of the nose it can also go down your throat and it will usually be out of one nostril.
Some people describe it as sweet, metallic or salty. Mine is super salty, like a mouthful of ocean water.
Headaches that get worse as the day progresses and you are on your feet. They will get better when you lay down flat.
If you had high pressure like me at the time of the leak, most likely, some will have pain relief most of the day because the drip is getting rid of excess fluid, much like a shunt would.
Breakout where it is leaking as your skin adjusts to the new fluid touching it. Mine bled for the first week and looked horrible, but over time it seemed to get used to it and only gets red once and a while.
Dizziness when standing upright too fast
Nausea in the later part of the day, as opposed to early morning with high intracranial pressure.
Vomiting later in the day.
Vertigo most of the time.
I also had several spells with my heartrate going really fast or really slow. 40-170.
Now What?
Ways to figure out if you have a leak.
You can drip the fluid onto a hard, non-porous surface and it will dry clear, no snail trail like in snot.
It will also sometimes leave a ring around the dried area.
You can test the fluid with a diabetic tester for a high glucose reading.
The best and definitive way though is through a beat-2 transferrin test. I mentioned it before and now because the ER doctor didn’t know how to test for it to be a leak.
If you do decide to get it tested and don’t drip that much, I suggest collecting in clear jar or container and refrigerating immediately. The test is looking for a protein that is delicate to heat and can be destroyed if refrigeration isn’t done.
If you do have a leak…
Don’t panic, but learn the symptoms of meningitis. This is your biggest threat. If you get high fever, headache, vomiting and a stiff neck where you can’t touch your chin to your chest, go to the hospital immediately with suspicions. Meningitis moves quickly and can be deadly in a matter of hours.
Research this disease because most people, including doctors are stumped. Do not agree to anything until you’ve researched it as much as possible. There are tons of studies out and information that can be found with a quick google search.
Reach out to one of many forums on cranial leaks and IIH. You will get more knowledge from there than anywhere else. The community is helpful and all you have to do is ask and people that are going through the same thing will help you to understand what is going on.