Hi! It has been a long time since I posted here - been quite busy. I did post earlier (pg 13 of this thread) but wanted to give an update and share the information we have gathered since then.
As I stated previously, my 12 yo daughter was experiencing very debilitating neurological symptoms - severe occipital headache, dizziness, blurred vision, light and sound sensitivity, nausea, weakness in her limbs, tachycardia and so on. She had spent about 8 months in a darkened bedroom - it was awful. Cervical fusion appeared to be the only option to try and there was no guarantee that it would take the symptoms away. Then I found the Driscoll Theory - an American eye doctor (she and her children have EDS and the scary neurological symptoms that go along) has pieced together existing research and came up with her own theory about the cause of neurological and autonomic symptoms in EDS. She is currently running several clinical trials to prove her theory and will be publishing the results soon.
Briefly, her theory is that the membranes in the brain (which are made of connective tissue) are faulty from birth in EDSers, allowing cerebrospinal fluid to build up, causing high intracranial pressure. This pressure builds up over time until it reaches a tipping point, pools on top of the brain and pushes the brain downwards - putting pressure on the brain stem and other brain structures. The result is the constellation of neurological (Chiari/cervical instability/ brain stem compression) and autonomic dysfunction (POTS) symptoms that make EDS patients so very ill. Mast Cell disease and CSSVI also play a big part in this theory but I am trying to simplify it here.
Best of all, there is real treatment if this theory applies to you - Diamox to reduce high pressure, Zyrtec and Zantac to treat mast cell disease and CSSVI angioplasty if that is your problem. Even if you have Chiari or cervical instability, this treatment may help your surgeries be more successful or let you avoid surgery for now.
After a long and challenging journey, we finally got Diamox prescribed for my daughter - and it has been incredible. Within 2 hours of taking the Diamox for the first time, her headache starting going away and her dizziness was better as well. Now, as long as she takes her medicine daily, that horrible pressure headache is gone, her positional blurred vision is gone and many of her other symptoms are improving. She also started basic mast cell treatment - Zyrtec and Zantac - and that is the other big piece of the puzzle. Her food sensitivities are vastly improved, the nausea and abdominal pain are basically gone and even the POTS symptoms are improving. The theory is rather complicated- it has a lot of 'moving parts' so to speak, but it all makes so much sense when you start looking at it.
This theory may not apply to everyone but, if you have EDS AND headaches AND other neurological symptoms AND POTS, you really should check out prettyill.com. There are videos and articles that you can help you understand if the theory fits you. It is revolutionary and some of it flies in the face of previous medical understanding - particularly as it pertains to the treatment of POTS but it is cutting edge research that is going to change the lives of EDSers in a big way. It has already given my daughter her life back and I would be remiss if I didn't share our success with you.
As always, I would be happy to answer any questions - about the theory itself or our experience with Diamox and mast cell treatment. BTW, these high pressure headaches do share some features of migraines but, obviously, migraine treatment would not solve the problem. Also, this level of high pressure is relatively subtle, compared to other causes of high intracranial pressure, and won't necessarily show up on an MRI until years have passed and symptoms have progressed significantly. Lumbar punctures often make the problem worse and will typically have normal results, since the pressure is not in the lower part of the spinal cord. Do not expect your doctor to know anything about this theory or treatment - you will need to figure this one out for yourself and present the information to your doctor if you think it would help you.
Whether your headache is high pressure or not, I hope you find relief! And I hope this information helps a bit.
Daughter Emily diagnosed 3/14/2011 with HEDS;
High intracranial pressure treated successfully with Diamox
suspected Mast Cell disease (likely Mast Cell Activation Syndrome)