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moy·a·moy·a dis·ease[MIM*252350] Because moyamoya is not a proper name, it is spelled with a lowercase m.
A cerebrovascular disorder occurring predominantly in the Japanese, in which the vessels of the base of the brain become occluded and revascularized with a fine network of vessels; it occurs commonly in young children and is manifested by convulsions, hemiplegia, mental retardation, and subarachnoid hemorrhage; the diagnosis is made by the angiographic picture.
a cerebrovascular disorder in which the main cerebral arteries at the base of the brain are replaced by a fine network of vessels. It is caused by progressive stenosis of the large-caliber vessels and development of collateral network. It tends mainly to affect Japanese children and young adults and is characterized by convulsions, hemiplegia, mental retardation, and subarachnoid hemorrhage. Patients who survive into adulthood are susceptible to massive intracerebral hemorrhage caused by rupture of the fragile network of new vessels. Few patients live beyond 30 years of age.
moyamoya diseaseAn idiopathic disease pattern characterised by bilateral stenosis of the internal carotid arteries, accompanied by a network of abnormal collateral vessels that bypass the stenosis. It is named for the “smoky” (in Japanese, moya-moya) angiographic appearance of prominent collateral vessels of the basal ganglia that accompany narrowed and distorted cerebral arteries with thin collateral vessels, which arise from the circle of Willis, with progressive occlusion.
Moyamoya disease is more common in females who are presenting as TIAs. Initially described in the Japanese and characterised by a familial tendency, it is also well described in Caucasians. It may follow a febrile illness and present with an abrupt onset of hemiparesis, transient aphasia and convulsions,
DiffDx, “puff of smoke” pattern
Occlusive intracranial atherosclerosis, radiation arteritis, intravascular tumour proliferation, tuberculous meningitis.
Revascularisation using the parietal branch of the superficial temporal artery.