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KMID : 1001920150570060408
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Journal of Korean Neurosurgical Society
2015 Volume.57 No. 6 p.408 ~ p.414
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Moyamoya Syndrome: A Window of Moyamoya Disease
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Phi Ji-Hoon
Wang Kyu-Chang
Lee Ji-Yeoun
Kim Seung-Ki
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Abstract
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Moyamoya-like vasculopathy develops in association with various systemic diseases and conditions, which is termed moyamoya syndrome. Relatively common diseases and conditions are related to moyamoya syndrome, including neurofibromatosis type 1, Down syndrome, thyroid disease, and cranial irradiation. Moyamoya syndrome shares phenotypical characteristics with idiopathic moyamoya disease. However, they differ in other details, including clinical presentations, natural history, and treatment considerations. The study of moyamoya syndrome can provide clinicians and researchers with valuable knowledge and insight. Although it is infrequently encountered in clinical practice, moyamoya-like vasculopathy can severely complicate outcomes for patients with various underlying diseases when the clinician fails to expect or diagnose moyamoya syndrome development. Furthermore, moyamoya syndrome could be used as a doorway to more enigmatic moyamoya disease in research. More comprehensive survey and investigation are required to uncover the secrets of all the moyamoya-like phenomena.
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KEYWORD
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Moyamoya syndrome , Neurofibromatosis , Down syndrome , Thyroid disease , Radiation
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