Moyamoya
What
is it?
Moyamoya disease is a rare,
progressive cerebrovascular disorder in which the
major blood vessels leading into the brain become
closed or blocked (occlusion), and abnormal blood
vessels called moyamoya vessels form. The
name “moyamoya” is Japanese for “cloud of
smoke,” which is what the abnormal vessels look
like.
Who
gets it?
Moyamoya disease was first
seen in Japan.
However, the disease has also been seen in
the United States, Europe, Australia, and Africa.
Children, adolescents, and young adults are most often
stricken. Moyamoya
disease affects females more often than males.
What
causes it?
The cause of moyamoya disease
is not known.
However, researchers suspect there may be a
genetic link because of its high rate of incidence
in certain Japanese families.
What
are the symptoms?
The most common symptoms of
moyamoya disease in children is stroke or repeated
transient ischemic attacks (TIAs).
These symptoms are often accompanied by muscular weakness or
paralysis affecting one side of the body (hemiparesis)
and seizures. The most common symptom in adults is
stroke. Other
symptoms in both children and adults include
altered consciousness, speech difficulties
(aphasia), vision problems, involuntary movements,
and difficulty with sensory and cognitive
functions. Most
patients with moyamoya disease will suffer
progressive cognitive deterioration and eventually
die due to intracerebral hemorrhage.
How
is it diagnosed?
Moyamoya disease is usually
diagnosed through cerebral angiography.
In this procedure, a dye is injected into
the cerebral arteries.
When x-rayed, the arteries are easier to
see because of the dye. The
abnormal vessels look like clouds of smoke.
What
is the treatment?
There is no cure for moyamoya
disease. Treatment is aimed at relieving symptoms
and includes aspirin, vasodilators, or
anticoagulants.
Surgery called revascularization may be
performed to restore the blood supply, but is
usually more effective in children than adults.
This information has been designed as a comprehensive and quick reference
guide written by our health care reviewers. The health information written
by our authors is intended to be a supplement to the care provided by your
physician. It is not intended nor implied to be a substitute for
professional medical advice.
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