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

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 ?
Moyamoya disease is a rare clinical syndrome that requires a high index of suspicion to detect.
Jeon, "An update on the diagnosis and treatment ofadult Moyamoya disease taking into consideration controversial issues," Neurological Research, vol.
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.
Pathologically, Moyamoya disease is characterized by intimal thickening in the walls of the terminal portions of the internal carotid vessels bilaterally.
Moyamoya disease is a rare, progressive cerebrovascular disorder secondary to arterial obstruction at the proximal Circle of Willis arteries.
Understanding and treating moyamoya disease in children.
And there were other two patients who had a prior presumptive diagnosis of CNS vasculitis before a definitive diagnosis was reached, especially a woman also with moyamoya disease, which has never been reported (Figure 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.1 The brain forms compensatory collateral arterial structures in an attempt to achieve a build-up of blood.
Clinical features, surgical treatment and long-term outcome in adult patients with moyamoya disease in China.
Safe treatment of pulmonary hypertension with bosentan in a patient with moyamoya disease and cerebral ischemia.
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.