J Korean Neurol Assoc.
2022 Oct;40(4):311-314. 10.17340/jkna.2022.4.4.
A Case of Vascular Ehlers-Danlos Syndrome Presenting with Multiple Cerebral Arterial Dissections
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- 1Department of Neurology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea
- KMID: 2535870
- DOI: http://doi.org/10.17340/jkna.2022.4.4
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.
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