Stroke in Ehlers-Danlos Syndrome kyphoscoliotic type (EDSVIA): dissection or vasculitis?

Publication date: Available online 29 May 2017
Source:Pediatric Neurology
Author(s): Annegret Quade, Martin Wiesmann, Joachim Weis, Ingo Kurth, Houman Jalaie, Marianne Rohrbach, Martin Häusler
A 13-year-old girl affected with the kyphoscoliotic type of Ehlers-Danlos syndrome (EDS VIA) suffered a stroke of the right middle cerebral artery. MR angiography and arteriography were not indicative of arterial dissection but of cerebral vasculitis. Immunosuppressive treatment resulted in clinical improvement. Six weeks after the stroke she suddenly died from hemorrhagic shock due to spontaneous aortic artery ruptures. Altered collagen function in EDS type VIa may differently affect cerebral and non-cerebral arteries.



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