The Driscoll Theory, authored by Dr. Diana Driscoll, an EDS patient herself, is a groundbreaking explanation of why some EDS patients are so ill with neurological and autonomic symptoms. The ‘real’ title of the theory is:
The Role of External Communicating Hydrocephalus, Mast Cell Disease and CCSVI as the cause of POTS (Postural Orthostatic Tachycardia Syndrome) in Ehlers-Danlos Syndrome
Those of us who have been helped (blessed) by this theory, are more than content to refer to it as ‘The Driscoll Theory’. Actually, at my house, we don’t think of it as a theory at all – it is simply proven fact to us. We were convinced that my daughter’s neurological and POTS symptoms were caused by cervical instability and the only treatment was a fusion. We were fortunate to find this theory and have been able to avoid surgery – my daughter is benefiting amazingly from finally having the proper treatment and is feeling so much better these days. I am dedicated to sharing this theory with as many EDS families as possible.
It is a hefty, complex theory so I will only attempt to hit the highlights here:
- External Communicating Hydrocephalus causes subtle levels of High Intracranial Pressure, often from birth. This results in a constellation of neurological and autonomic dysfunction symptoms that can manifest at any age. Poor CSF drainage resulting from defective collagen causes CSF to build up on top of the brain and pushes the brain down, putting pressure on the brain stem and other structures. This downward pressure blocks CSF draining out of the brain and creates a constellation of symptoms – base of the brain, pressure headache; dizziness; tachycardia; fainting/near fainting; nausea; blurred vision; numbness in the extremities and so on – in short, symptoms of Chiari, Cervical Instability, Brainstem compression and POTS. Diamox, a mild diuretic, can quickly reduce the build up of CFS and relieve the debilitating symptoms overnight. Even if cervical instability requires a fusion or a Chiari Malformation calls for a decompression, using Diamox to reduce the CSF pressure above the brain could make those surgeries more successful.
- CCSVI (Chronic Cerebrospinal Venus Insufficiency) Stenosed (narrowed) veins in the cervical region result in poor blood and CSF drainage, contributing to the cycle of building pressure above the brain. Angioplasty can open the narrowed veins and restore proper drainage. This is a big area of research in MS; EDSers are at increased risk of developing MS so the connection is intriguing. [Edited to point out that the veins of EDSers are fragile and CCSVI treatment is problematic, although stenosed veins may indeed be a problem. No one should run out and seek CCSVI treatment. It is an intriguing connection but available treatment is not necessarily the answer.]
- Mast Cell Disease is at the root of the Driscoll Theory. In mast cell disease – mastocytosis or mast cell activation syndrome – mast cells degranulate and release a host of toxic chemicals throughout the body. Mast cells can hide all over the body – in the gut, in the brain, in the bladder, etc. When they are in the brain, they can affect the production of CSF and cause autonomic dysfunction symptoms. They can damage blood vessels, causing them to be ” ‘leakier’ and weaker” than they already are in EDS patients. In the gut, they cause serious GI issues, including food sensitivities that mimic gluten intolerance and more. Flushing, tachycardia, fainting/near fainting, abdominal pain,headaches, fatigue are symptoms of mast cell disease and, not coincidentally, are also symptoms of POTS. Treatment includes H1 and H2 histamine blockers, like Zyrtec and Zantac. A mast cell stabilizer like Gastrocrom is the next line of treatment. Some researchers believe that POTS = mast cell disease (there is certainly a connection with hyperadrenergic POTS); other doctors are successfully treating IBS with mast cell stabilizers. Mast cell disease is a big area of research for the EDS community and offers a very interesting avenue of investigation.
If you would like to download the whole theory, go to Dr. Diana’s web site. You can watch videos in which Dr. Diana explains portions of the theory and answers questions, or go to the articles and downloads section of her web site to download the document and other articles. I encourage everyone with EDS, POTS and/ or neurological symptoms to check out this theory and discuss the treatment options with your doctor. It is truly groundbreaking and the EDS community stands to benefit in a big way from Dr. Diana’s research.
[Also edited to say, that the printed version of the theory is unavailable and some of these links are broken because of the actions of some unscrupulous people. If you are interested in learning about the Driscoll Theory, please go to prettyill.com and watch Dr. Diana’s videos. You can get so much info from her videos…I suggest you start here or here for information about the theory or here to understand who Dr. D is as a patient.]