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 - Northwestern Memorial Hospital - Chicago

Moyamoya Disease

Moyamoya disease is a rare cerebrovascular disorder in the area at the base of the brain known as the basal ganglia, where arteries become blocked.

This progressive disease (meaning it worsens over time) literally means “puff of smoke” in Japanese, describing the look of the tangled blood vessels that form to compensate for the blockage.

First described in Japan in the 1960s, it has been found in the United States, Europe, Africa and Australia, and it primarily affects children, although it can occur in adults.

Signs & Symptoms

In children, the first symptom of Moyamoya disease is most often a stroke, or recurrent transient ischemic attacks (TIAs, also known as mini-strokes).

In adults with moyamoya, hemorrhagic stroke is the most likely to occur, due to recurring blood clots in the affected blood vessels.

Symptoms of moyamoya disease may include:

  • Disturbed consciousness
  • Aphasia or other speech disorders
  • Sensory impairment
  • Cognitive impairment
  • Vision problems
  • Involuntary movement

Moyamoya tends to run in families, which has led researchers to think that it may be a result of an inherited genetic condition.

Research is being conducted to find any abnormal gene(s) that may cause the disorder.

Treatment Options

There are a number of revascularization surgeries that may be performed to restore the flow of blood to the brain.

These procedures open narrowed blood vessels or bypass blocked arteries. This treatment works particularly well with children, but for the majority of individuals, it can effectively end strokes or related complications from Moyamoya disease.

Because Moyamoya disease is a progressive condition, without surgical treatment, individuals with it will experience multiple strokes and continuing mental decline because of the narrowing of the arteries in their brains.

Without treatment, Moyamoya disease can be fatal.

Research on Moyamoya Disease

There are studies being undertaken at the National Institute of Neurological Disorders and Stroke (NINDS), from clinical trials to clinical investigations of biological functions to discover ways to prevent, treat and cure Moyamoya disease and other neurological disorders.

Last UpdateApril 30, 2012