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NINDS Moyamoya Disease Information Page


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What is Moyamoya Disease?

Moyamoya disease is a rare, progressive cerebrovascular disorder caused by blocked arteries at the base of the brain in an area called the basal ganglia. The name “moyamoya” means “puff of smoke” in Japanese and describes the look of the tangle of tiny vessels formed to compensate for the blockage.  Moyamoya disease was first described in Japan in the 1960’s and it has since been found in individuals in the United States , Europe, Australia , and Africa.  The disease primarily affects children, but it can also occur in adults.   In children, the first symptom of Moyamoya disease is often stroke, or recurrent transient ischemic attacks (TIA, commonly referred to as “mini-strokes”), frequently accompanied by muscular weakness or paralysis affecting one side of the body, or seizures.  Adults most often experience a hemorrhagic stroke due to recurring blood clots in the affected brain vessels.   Individuals with this disorder may have disturbed consciousness, speech deficits (usually aphasia), sensory and cognitive impairments, involuntary movements, and vision problems.  Because it tends to run in families, researchers think that Moyamoya disease is the result of inherited genetic abnormalities.  Studies that look for the abnormal gene(s) may help reveal the biomechanisms that cause the disorder.

Is there any treatment?

There are several types of revascularization surgery that can restore blood flow to the brain by opening narrowed blood vessels or by bypassing blocked arteries.  Children usually respond better to revascularization surgery than adults, but the majority of individuals have no further strokes or related problems after surgery. 

What is the prognosis?

Without surgery, the majority of individuals with Moyamoya disease will experience mental decline and multiple strokes because of the progressive narrowing of arteries. 

Without treatment, Moyamoya disease can be fatal as the result of intracerebral hemorrhage.

What research is being done?

The NINDS conducts neurological research in laboratories at the NIH and also supports additional research through grants to major medical institutions across the country.  These studies, which range from clinical trials to investigations of basic biological mechanisms, are aimed at discovering how and why diseases develop in the brain, and focus on finding ways to prevent, treat, or cure them. 

NIH Patient Recruitment for Moyamoya Disease Clinical Trials

Organizations

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National Rehabilitation Information Center (NARIC)
8201 Corporate Drive
Suite 600
Landover, MD   20785
naricinfo@heitechservices.com
http://www.naric.com
Tel: 301-459-5900/301-459-5984 (TTY) 800-346-2742
Fax: 301-562-2401

National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
Danbury, CT   06810
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 203-744-0100 Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291

National Stroke Association
9707 East Easter Lane
Suite B
Centennial, CO   80112-3747
info@stroke.org
http://www.stroke.org
Tel: 303-649-9299 800-STROKES (787-6537)
Fax: 303-649-1328

Children's Hemiplegia and Stroke Assocn. (CHASA)
4101 West Green Oaks Blvd., Ste. 305
PMB 149
Arlington, TX   76016
info437@chasa.org
http://www.chasa.org
Tel: 817-492-4325

Related NINDS Publications and Information


Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892



NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.

All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.

Last updated December 8, 2014