moyamoya disease


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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.
[Jap. addlebrained]

moyamoya disease

[moi′əmoi′ə]
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 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 ?
A diagnosis of moyamoya disease was made after clinical and laboratory exclusion of secondary causes and with the use of imaging.
The term moyamoya disease or primary moyamoya, however, is reserved for cases where no underlying or secondary cause can be identified, as in our patient.
Cerebral Circulation and Metabolism in Adults' Moyamoya Disease -PET Study.
Novel epidemiological features of moyamoya disease.
In four families, members younger than age 20 suffered a stroke and five strokes resulted from Moyamoya disease, a rare stroke disease in which the internal carotid arteries become occluded.
Case of moyamoya disease in a patient with advanced acquired immunodeficiency syndrome.
The association of Moyamoya disease and congenital heart defect such as coarctation of the aorta has been infrequently reported.
A correspondent, Hasmik Manukyan, mother of 22-year-old Arthur who suffers from moyamoya disease, said that she was glad that her son could continue his treatment in Turkey.
Children with moyamoya disease have no clear contributing etiology, but children with moyamoya syndrome have contributory diagnoses such as Down syndrome or neurofibromatosis.
Carolyn Dadd, 15, has moyamoya disease (see Editor's Note).
Main opposition Republican People's Party (CHP) deputy Canan Aritman, during a parliamentary gathering on April 24, approached Prime Minister Recep Tayyip Erdogan and asked him to help grant a residence permit to Manukyan, 21, as he has to be treated in Turkey for the deadly moyamoya disease, an inherited disease in which certain arteries in the brain are constricted.
The patient's underlying condition is Moyamoya disease, a progressive cerebrovascular disorder caused by blocked arteries at the base of the brain.