Abstract
BackgroundMoyamoya disease (MMD) is a cerebrovascular disease characterized by progressive stenosis or occlusion of the terminal portion of internal carotid arteries and the formation of a vascular network at the base of the brain. The pathogenesis of MMD is still unclear.Methodology/Principal FindingsWe 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. 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). Intracranial hemorrhage was the predominant disease type (75%). Positive family history was observed in 4.6% of patients. 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. All 3 autopsy cases showed vacuolar degeneration in the cerebrovascular smooth muscle cells. 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. The Complement C3 immunoreactivity was negative.Conclusion/SignificanceThis study indicated that aberrant expression of IgG and S100A4 protein in intracranial vascular wall of MMD patients, which suggested that immune-related factors may be involved in the functional and morphological changes of smooth muscle cells, and finally caused the thickened intima. A possible mechanism is that deposits of IgG in the damaged internal elastic lamina may underlie the disruption of internal elastic lamina, which facilitated S100A4 positive SMCs migrated into intima through broken portions of the internal elastic lamina, resulting in lumen stenosis or occlusion, leading to compensatory small vessels proliferation.
Highlights
Conclusion/Significance: This study indicated that aberrant expression of IgG and S100A4 protein in intracranial vascular wall of MMD patients, which suggested that immune-related factors may be involved in the functional and morphological changes of smooth muscle cells, and caused the thickened intima
Moyamoya disease (MMD), 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 [1]
The abnormal vascular networks (Moyamoya vessels) at the base of the brain act as collateral channels because of ischemic changes occurring in the brain tissue
Summary
Moyamoya disease (MMD), 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 [1]. The abnormal vascular networks (Moyamoya vessels) at the base of the brain act as collateral channels because of ischemic changes occurring in the brain tissue These fragile vascular networks resemble a ‘‘puff of smoke’’ (Japanese: Moyamoya) on angiography imaging and the disease name [2]. This clinical disease was first reported in Japan as hypogenesis of the bilateral internal carotid arteries [3]. Moyamoya disease (MMD) is a cerebrovascular disease characterized by progressive stenosis or occlusion of the terminal portion of internal carotid arteries and the formation of a vascular network at the base of the brain.
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