Re: Moyamoya

Posted By ccf neuro M.D.* on September 13, 1997 at 16:31:46:

In Reply to: Moyamoya posted by Micheal Guest on September 06, 1997 at 20:19:43:








Our daughter has been diognosted with Moyamoya ,it is rare and theirfore not alot of information seams to be available
could you give us any help about this or info. on other sources?
Any help would be appreciated.
Thank you, Mike Guest
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Mike,
Moya moya is a Japanese term that means "puff of smoke" , and refers specifically to the appearance of the blood vessels in the brain on angiography, which is a test where dye is injected into arteries for the purposes of looking for abnormalities or blockages in them. Moya moya is not a true disease but rather what we call a SYNDROME, which is defined as a cluster of particular medical symptoms that may be due to many possible underlying causes. Thus, in Moya Moya syndrome, one must always look for an underlying cause; if no particular cause is found, the term "idiopathic" (cause unknown) Moya Moya or Moya Moya DISEASE might be appropriate. The problem in Moya Moya sundrome is that most or all of the main, large blood vessels that go to the brain are blocked closed or very very severely narrowed, forcing blood to be diverted into much smaller than usual arteries, which enlarge and become twisty in an attempt to deliver as much blood to the brain as possible, and on angiography look like a puff of smoke as a result of this, rather than being very small or even angiographically invisible like usual. Possible underlying causes of the syndrome include blood clotting disorders where blood clots too easily, vasculitis, which is inflammation of the arteries, and fibromuscular dysplasia,  which is when the cells lining the blood vessel overgrow and close it off. There are other causes as well, but to list all of them would take too long. Unfortunately, there is no Moyamoya foundation that I am aware of to refer you to for additional information, but I hope this gives you at least some understanding of what the diagnosis means. If you would be interested in having your daughter evaluated by a Cleveland Clinic Stroke neurologist, you may call 1-800-223-2273; I would suggest Dr. Tony Furlan or Dr. Cathy Sila in particular as they both have considerable experience with this disorder. Information provided on the neurology forum is intended for general medical informational purposes only; actual diagnosis and treatment of your daughters medical illness should be strictly in conjunction with her treating physician(s).