moyamoya disease

(redirected from moya moya syndrome)

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.
[Jap. addlebrained]

moyamoya disease

An 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.
 
Clinical findings
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.
 
Management
Revascularisation using the parietal branch of the superficial temporal artery.
References in periodicals archive ?
Anita Redmond, 20, and Elizabeth Carty, nine, were diagnosed with Moya Moya Syndrome two years ago.
"Two neighbours would have more chance of winning the Lotto than contracting Moya Moya Syndrome.