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KMID : 0359720220400040311
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Journal of the Korean Neurological Association
2022 Volume.40 No. 4 p.311 ~ p.314
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A Case of Vascular Ehlers-Danlos Syndrome Presenting with Multiple Cerebral Arterial Dissections
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Yang Seong-Kyu
Lee Byeong-Cheon
Ko Yoon-A
Kim Jun-Yup
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Abstract
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Vascular EhlersDanlos syndrome (vEDS) is caused by dysfunctional COL3A1 gene for type III procollagen. It presents with translucent skin, easy bruising, and arterial dissection. It has the worst prognosis among various types of EDS with holloworgan rupture as the leading cause of death, mounting to the median life expectancy of mid40s. We report a rare case of vascular EhlersDanlos syndrome presenting late in the fifth decade of life with multiple arterial dissections isolated to cerebral arteries.
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KEYWORD
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Ehlersdanlos syndrome, Dissection, Connective tissue diseases
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