Late Thursday night I sent an email to my former neurologist to see if he could help me get an appointment with the headache specialist he wanted me to see. He had given me his email for just this occasion, but I felt guilty using it anyway. To my surprise, he replied 4 minutes later and said he’d take care of it. I got an email the next morning, followed by a phone call and less than 12 hours after I had hit sent, I had an appointment with the new neurologist. For today, at noon.
Yea, I was surprised too.
So I met my new neurologist today, she’s just begun a fellowship in headaches in the same clinic as my old neurologist. It’s a pretty coveted fellowship, so clearly the girl is smart.
She had me take her through my whole history, starting when the headaches started in 2005. Going through the Chiari diagnosis, the watching and waiting, the progression of symptoms, the surgery. Then the CSF leak from the surgery, the period of no headaches, the return of the headaches, the lumbar punctures, the low pressure, the negative tests and on and on. We talked about all the medications I’ve tried for these headaches (there are a lot, none of which have helped) and the outcomes of all those medications.
Then she did a neuro exam, which was nearly perfectly normal. After the exam she just sat and talked with me. She said she’s confident that it’s not a number of things, most of which I already knew. It’s nice to be on the same page with a doctor. And even better, she believes she knows what the problem is.
Low pressure caused by a CSF leak.
Now, when I told my family this like 3 of them said (and I quote) “And?”
So yea, this is not a new diagnosis. The difference is that she finally made it make sense. My last neurologist ruled out a CSF leak and low pressure as the cause for a few reasons. First, my previous scans (cisternogram and myelogram) were negative for CSF leaks. That’s pretty strong evidence against a leak. Second, my headache was not positional. Typically with a CSF leak, when you are upright, it is severe, when you are laying flat, it’s better. And mine is not. And third, when I get leaks from lumbar punctures, the headaches are different.
But, it turns out most of that is not actually a persuasive argument agains a leak.
The new neurologist thinks I have a CSF leak around my brain, not my spine. And in those situations, the cisternogram and myelogram would not be sensitive tests to detect it. Especially if the leak is not constant and not huge, which is her belief. She thinks it’s a slow, relatively small leak that might not be open when I’m laying still in an MRI tube.
She also said that the reason the headache is not positional could be that the leak is not always open. She thinks the reason that my head sometimes hurts more when I’m laying down is because the leak itself is provoked in different positions. So it might be that I’m sleeping in a position that causes the hole to open. And the reason the headache isn’t as severe is because the leak is small, unlike the ones I’ve had after lumbar punctures.
The thing that sealed the deal was a review of my MRI from November.
My last neurologist had told me that the MRI showed that my cerebellum had herniated further, indicating a progression of my Chiari that might require further decompression surgery. It turns out, that wasn’t actually the case. The MRI showed “slumping” of my brainstem, which is a sign of significant low pressure. It’s also not a sign that we want to toy around with, the brainstem is pretty freaking important.
Her attending, who is a really well respected and very experienced headache specialist, agreed with her assessment. And he threw in an extra tid bit that has me reeling a little. He thinks that I may never have had a Chiari Malformation at all. At least not in the technical sense. I absolutely did have my cerebellum squish out into my spinal canal requiring brain and spinal surgery, but he thinks mine might not have been caused by having a small skull or a low lying cerebellum, but instead of from low pressure. He thinks my entire 7 year history of headaches might all come back to a CSF leak.
It’s like for 7 years everyone has been telling me that the sky is blue because it reflects the ocean. And then today they were like, actually, it’s turquoise. And now I’m all, holy shit. The sky just might be turquoise. THE SKY MIGHT BE TURQUOISE.
I’m hesitant to be too excited because if we find a leak (and this is a big IF since it has alluded several scans already) using a scan I’ll be having later this month, the only way to fix it is surgery. You may or may not know this, but I am keenly disinterested in more surgery. Especially since if we find it on this scan, it’ll be brain surgery, and that’s pretty much my least favorite kind of surgery. And more than that, I just can’t quite get my hopes up that we’ll find it at all because I’ve been down this path before and the results have not been great.
Thankfully the new neurologist is aware of that possibility, so she’s trying a two pronged attack. In addition to the scan, she’s putting me on a SUPER high dose of Vitamin A, which should increase my production of CSF, which should cause the headaches to decrease. It could further validate the hypothesis that low pressure is the cause of all of this and might even make it easier to see on the scan.
I’m teetering on the boundaries of excited and scared. It’s been a while since we thought we knew what the problem was, and instead we’ve spent a lot of time taking stabs in the dark. Today someone turned on a small light, and got us on a path. The end of the path is uncertain and scary, the odds that we’re still lost are fairly high. But we’re trying.
And for now, that’ll do.