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Pathophysiology of Moyamoya Disease

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Abstract

Moyamoya disease is a chronic progressive steno-occlusive disease of the distal internal carotid artery or proximal anterior cerebral artery and the middle cerebral artery with abnormal moyamoya collateral vessels without associated diseases. The disease has been increasingly reported due to the technological advances of diagnostic radiology and an increase of health check-up. The studies regarding the incidence, prevalence, natural clinical course, disease progression, and surgical treatment outcomes have been increasingly reported. Nevertheless, the precise mechanism of the disease still remains to be investigated further. In addition, heterogeneity of the ethnicity, different age at presentation, different degrees of hemodynamic compromise, surgical techniques such as direct bypass or indirect bypass surgery, and relative small sample size could lead to controversial results. This chapter provides insights into the pathophysiology of the disease including histopathological features, genetics, Ring finger protein 213, microRNAs, molecular biomarkers, vascular progenitor cells, proteomics, metabolomics, and associated autoimmune diseases.

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Correspondence to Jeong Eun Kim MD, PhD .

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Jeon, J.P., Kim, J.E. (2017). Pathophysiology of Moyamoya Disease. In: Park, J. (eds) Acute Ischemic Stroke. Springer, Singapore. https://doi.org/10.1007/978-981-10-0965-5_2

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  • DOI: https://doi.org/10.1007/978-981-10-0965-5_2

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