Author:
Yang Seong Kyu,Lee Byeongcheon,Ko Yoona,Kim Jun Yup
Abstract
Vascular Ehlers-Danlos 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 hollow-organ rupture as the leading cause of death, mounting to the median life expectancy of mid-40s. We report a rare case of vascular Ehlers-Danlos syndrome presenting late in the fifth decade of life with multiple arterial dissections isolated to cerebral arteries.
Publisher
Korean Neurological Association