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Year : 2019  |  Volume : 7  |  Issue : 2  |  Page : 224-232

Moyamoya disease

Department of Neurosurgery, Kasturba Medical College, Manipal University, Manipal, Karnataka, India

Correspondence Address:
Girish Menon
Department of Neurosurgery, Kasturba Medical College, Manipal University, Manipal - 576 104, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/amhs.amhs_156_19

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Moyamoya disease (MMD) is a unique cerebrovascular disease characterized initially by an obliterative vasculopathy followed by a compensatory proliferative vasculopathy. Coined by Suzuki and Takaku in 1969, the term moyamoya (MM) refers to a “puff of smoke” like appearance of small collaterals seen traversing the basal ganglia and thalamus, in response to progressive stenosis and occlusion of supraclinoid internal carotid artery. The natural history is unclear but is generally one of the gradual progressions. The etiopathogenesis of MMD is unknown, but the syndromic form may be associated with certain genetic conditions, such as Down's syndrome and neurofibromatosis, and may occur following cranial radiation. The clinical manifestations are predominantly ischemic and usually confined to the anterior circulation. Hemorrhagic presentation is less common and occurs more often in adults. Similarly, posterior circulation may get involved in later stages. Treatment is essentially surgical, and a combined revascularization strategy involving a direct superficial temporal artery to middle cerebral artery anastomosis along with a pial synangiosis provides a reasonable stroke-free survival. Considerable controversy exists on the etiopathogensis, diagnostic guidelines, management protocols, ideal surgical approach, and the role of surgery in hemorrhagic MMD. This review attempts to summarize the current advances of MMD on the aspects of epidemiology, etiology, clinical features, imaging diagnosis, and treatment.

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