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 ?
In this paper, we report a female patient with known moyamoya disease and a stable right ovarian cyst who was regularly followed up by the departments of neurology, gynecology and presented with radiological and cystoscopy findings of incidentally diagnosed asymptomatic continent VVF.
Patients admitted with Moyamoya disease and Down syndrome were more likely to present with ischemic stroke and less commonly with hemorrhagic stroke (15.
Other neuroimaging findings--magnetic resonance imaging (MRI), magnetic resonance angiogram (MRA), magnetic resonance venography (MRV), and computed tomography angiography (CTA)--were consistent with moyamoya disease and confirmed by cerebral angiography (Figures 1, 2).
Moyamoya disease is a cerebrovasculopathy of unknown etiology; during the course of which the main and terminal veins of the internal carotid artery undergo progressive vein occlusion.
moyamoya disease in China: its clinical features and outcomes.
We present progressive imaging of a 3-year-old boy confirmed to have idiopathic moyamoya disease, over 6 years using serial MRI with MR angiography, and compare with conventional angiography at the last visit.
Morning glory syndrome: association with moyamoya disease, midline cranial defects, central nervous system anomalies, and persisrent hyaloid arrery remnant.
He was eventually transferred to Hasbro Children's Hospital (Hasbro) in Providence, Rhode Island, where he was diagnosed with Moyamoya disease.
He was transferred to Hasbro Children's Hospital (Hasbro) in Providence Rhode Island, where he was diagnosed with Moyamoya disease.
Cerebral Circulation and Metabolism in Adults' Moyamoya Disease -PET Study.
The conclusion of this section provides in-depth coverage of moyamoya disease, a relatively common occurrence in Asian populations with diagnosis rates on the rise in this time of advanced imaging.
Boyce has been diagnosed with Moyamoya Disease, a rare, progressive cerebrovascular disorder.