moyamoya disease

(redirected from Moya moya)

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]
Farlex Partner Medical Dictionary © Farlex 2012

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.
Revascularisation using the parietal branch of the superficial temporal artery.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.
References in periodicals archive ?
Moya moya disease in children usually presents with transient ischemic attack or recurrent stroke and prognosis is variable.
Child was investigated for possibility of sickle cell anemia, neurofibromatosis and Moya moya disease.
DISCUSSION: Moya moya disease is a chronic progressive non-atherosclerotic non-inflammatory non-amyloid occlusive intracranial vasculopathy of unknown cause.
Marie Lochrie, 17, has moya moya, which affects one in two million and causes repeated strokes.
(5.) Takahashi, Magnification angiography in Moya Moya disease.
IMAGE 1: MRI: revealed presence of infarction in the bilateral frontal lobes as classic for a paediatric moya moya. (while haemorrhage from collaterals is common in adults).
IMAGE 4 & 5: MRA: was able to detect abnormal vessels in the basal ganglia, basal cisterns & periventricular white matter which are known as "MOYA MOYA VESSELS".
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.