Moyamoya disease (patient information)

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 * Associate Editor(s)-In-Chief: Varun Kumar, M.B.B.S.

Overview
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

What are the symptoms of Moyamoya disease?

 * Stroke
 * TIA commonly referred to as mini-stroke
 * Muscular weakness or paralysis affecting one side of the body
 * Seizures
 * Disturbed consciousness
 * Speech deficits (usually aphasia)
 * Sensory and cognitive impairments
 * Involuntary movements
 * Vision problems

Who is at highest risk?
It tends to run in families, researchers think that Moyamoya disease is the result of inherited genetic abnormalities

When to seek urgent medical care?
Doctor should be immediately contacted when you experience the above listed symptoms

Diagnosis
Your physician may do a physical exam and suggest few tests which aid in diagnosis of the condition.
 * Blood tests: To analyse the clotting function of blood.
 * Thyroid function test
 * CT brain: To look for any bleed
 * Cerebral angiography: To localize the blockage in blood vessels supplying the brain.

Treatment options
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.

Where to find medical care for Moyamoya disease?
Directions to Hospitals Treating Moyamoya disease

What to expect (Outlook/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.

Possible complications

 * Paralysis