What is moyamoya disease?

Moyamoya disease is a condition where moyamoya occurs on both sides of the brain in a progressive fashion and there's no underlying cause that can be found. Moyamoya syndrome is when the same thing occurs but there is an underlying cause such as sickle cell disease, trisomy 21, type 1 neurofibromatosis and prior cranial irradiation.

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 appearance of a tangle of tiny vessels formed to compensate the blockage. Moyamoya disease was first described in Japan in the 1960s and has since been found in individuals in the United States, Europe, Australia and Africa. The disease primarily affects children, but can also occur in adults. In children, the first symptom of Moyamoya disease is often stroke, or recurrent transient ischemic attacks (TIA, commonly referred to as "mini-strokes"), frequently accompanied by muscular weakness or paralysis affecting one side of the body, or seizures. Adults most often experience a hemorrhagic stroke due to recurring blood clots in the affected brain vessels. Individuals with this disorder may have disturbed consciousness, speech deficits (usually aphasia), sensory and cognitive impairments, involuntary movements and vision problems. Since it tends to run in families, researchers think that Moyamoya disease is the result of inherited genetic abnormalities. Studies that look for the abnormal gene(s) may help reveal the biomechanisms that cause this disorder.

This answer is based on source information from the National Institute of Neurological Disorders and Stroke.

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