What is Moyamoya Disease?

Moyamoya disease - also called  Spontaneous Occlusion of the Circle of Willis - causes narrowing and occlusion of the main arteries that deliver blood to the brain.   It is a progressive disease, and as the arteries become blocked, tiny innumerable blood vessels form as the body's attempt to re-route blood to the brain.  It is the appearance of these tiny vessels on an MRI that gives Moyamoya its name (Moyamoya means "puff of smoke" in Japanese).  The disease causes successive strokes and mental decline if untreated.

The etiology of Moyamoya disease is unknown, and it can occur at any age. No drug therapy has proven effective against the disease; however, revascularization surgeries have proven effective at rerouting blood to the brain, thus preventing strokes and mental decline.

Only a handful of surgeons in the United States have extensive experience with Moyamoya disease.   If diagnosed with the disease it is very important to see a Moyamoya specialist and get treatment as soon as possible.   A successful surgery dramatically improves the prognosis of this disease, and indeed many Moyamoya sufferers have been able to live relatively normal lives after treatment.

There are some great resources on the Internet for learning about Moyamoya disease.  Here are some of our favorite websites:

Moyamoya Links  ☼  ☼  ☼

Moyamoya.com A website with information and support, founded by a Moyamoya sufferer.  This is a great resource, and features a message board where Moyamoya patients and their families can communicate with others who are affected by this disease.

Pediatric Neurosurgery - Moya moya A web page provided by Columbia Presbyterian that gives a good overall synopsis of Moyamoya disease.

Brain Basics: Preventing a Stroke Information about strokes and what causes strokes, with links to various stroke studies and stroke support groups.

Children's Hospital: FAQ's, Moyamoya  FAQ's about Moyamoya by Dr. Scott

Surgical Management of Moyamoya Disease Great information about Moyamoya surgery by Dr. Steinberg and team.

Dr. Steinberg's Profile Link to Dr. Steinberg's profile at Stanford Hospital.  Dr. Steinberg is one of the leading neurosurgeons with Moyamoya experience, and he performed Daphne's surgeries.

Dr. Scott's Profile Link to Dr. Scott's Profile at Boston Children's.  Dr. Scott is another leading neurosurgeon with Moyamoya experience.

Elastin and Moyamoya A study linking an abnormal elastin gene to Moyamoya disease (this is Daphne's geneticists' current theory on the cause of Daphne's many  issues.  We are still awaiting results from testing)