NINDS Moyamoya Disease Information Page: NINDS

Article title: NINDS Moyamoya Disease Information Page: NINDS
Main condition: Moyamoya disease
Conditions: Moyamoya disease

What is Moyamoya Disease?
Moyamoya disease is a rare, progressive cerebrovascular disorder characterized by the narrowing or occlusion of major blood vessels leading into the brain, and the formation of abnormal blood vessels called moyamoya vessels. First described in Japan in the 1960�s, the disease has since been found in the United States, Europe, Australia, and Africa. It primarily affects children, adolescents, and young adults, although it has also been seen in people beyond these ages. Females are more frequently affected than males. The cause of the disease is unknown. Researchers suspect a genetic link because of the 9 percent incidence of the disease found in certain Japanese families. A gene for familial moyamoya disease has been located on chromosome 17q25 and further study of the gene may reveal the cause of the disorder. The name �moyamoya� is Japanese for �cloud of smoke� and was chosen to describe the classic appearance of the abnormal vessels seen in diagnostic tests. Children often present with stroke or recurrent transient ischemic attacks (TIAs), frequently accompanied by hemiparesis (muscular weakness or paralysis affecting one side of the body) or seizures. Adults most often experience stroke (often a hemorrhage). Both children and adults may have disturbed consciousness, speech deficits (aphasia), sensory and cognitive impairments, involuntary movements, and vision problems. Generally, cerebral angiography�a procedure that involves injecting dye into the cerebral arteries before taking an x-ray to make the arteries easier to see�is used to diagnose moyamoya disease.

Is there any treatment?
There is no cure for moyamoya disease. Treatment is symptomatic and supportive. Individuals experiencing TIAs and stroke may be given aspirin, vasodilators, or anticoagulants to reduce the risk of future attacks. There are several different types of revascularization (restoration of blood supply) surgery that may be performed in some cases. Children usually respond better to revascularization surgery than adults.

What is the prognosis?
Progressive deterioration of cognitive function is seen in the majority of individuals with moyamoya disease. Death usually results from intracerebral hemorrhage.

What research is being done?
Within the NINDS research programs, moyamoya disease is addressed primarily through studies associated with TIA and stroke. NINDS is the leading supporter of research on stroke and TIA in the United States and sponsors studies ranging from clinical trials to investigations of basic biological mechanisms.

Organizations

National Rehabilitation Information Center (NARIC)
1010 Wayne Avenue
Suite 800
Silver Spring, MD 20910-5633
naricinfo@kra.com
http://www.naric.com/
Tel: 301-562-2400 800-346-2742
Fax: 301-562-2401

National Organization for Rare Disorders (NORD)
P.O. Box 8923
(100 Route 37)
New Fairfield, CT 06812-8923
orphan@rarediseases.org
http://www.rarediseases.org/
Tel: 203-746-6518 800-999-NORD (6673)
Fax: 203-746-6481

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

Multi-Infarct Dementia
Multi-infarct dementia information sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

Cerebral Arteriosclerosis
Cerebral arteriosclerosis information sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

Stroke: Hope Through Research
An informational booklet about stroke compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

Stroke Information Page
Stroke information sheet compiled by NINDS.

This fact sheet is in the public domain. You may copy it.Provided by:
The National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892

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