Moyamoya disease (MMD), also known as spontaneous occlusion of the circle of Willis, is a cerebrovascular disease characterized by progressive stenosis or occlusion of the terminal portion of internal carotid arteries. The Complement C3 immunoreactivity was negative. Immunohistochemical study showed the migration of smooth muscle cells in the thickened intima, and aberrant expression of IgG and S100A4 protein in vascular smooth muscle cells. All 3 autopsy cases showed vacuolar degeneration in the cerebrovascular smooth muscle cells. Histopathological findings were a narrowed lumen due to intimal fibrous thickening without significant inflammatory cell infiltration, and the internal elastic lamina was markedly tortuous and stratified. ![]() Positive family history was observed in 4.6% of patients. Intracranial hemorrhage was the predominant disease type (75%). The onset age distribution was found to have 1 peak at 40–49 year-old age group, no significant difference was observed in the female-to-male ratio (F/M = 1.2). We retrospectively analyzed clinical data for 65 consecutive patients with MMD in our institutions and evaluated the histopathological and immunohistochemical findings of intracranial vessels from 3 patients.
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