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 1960s, 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 high incidence of the disease found
in certain Japanese families. 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 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
This article reprinted with
permission from the National Institute of Neurological Disorders and
Columbia Presbyterian Medical Center
Disease UCLA Neurosurgery Department
Moya Disease Chicago Institute of Neurosurgery and Neuroresearch
ischemic stroke in children with moyamoya disease. May 2004. Germany
effects of indirect bypass surgery on collateral vessel formation in
pediatric moyamoya disease Feb. 2004. Japan
manifestations of infantile moyamoya disease. Oct. 2003. Japan
treatment of moyamoya disease in pediatric age 2001
encephaloduroarteriosynangiosis and bifrontal
encephalogaleo(periosteal)synangiosis in pediatric moyamoya disease
and combined revascularization in pediatric moyamoya disease
Aneurysms & Moyamoya Disease -