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J Clin Neurosci. 2013 Oct;20(10):1454-5. doi: 10.1016/j.jocn.2012.10.039. Epub 2013 Jul 3.

Coincidence of ischemic stroke and recurrent brain haemorrhage in a patient with Klippel-Trenaunay Syndrome.

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Department of Neurology, University Hospital Freiburg, Breisacherstrasse 64, Freiburg 79106, Germany. Electronic address:


Cerebrovascular manifestations in Klippel-Trenaunay Syndrome (KTS) have been reported but are extremely rare. Case reports describe brain embolism in KTS-associated coagulopathy as well as bleeding due to arteriovenous malformations. We describe a 45-year-old patient with KTS and both acute ischemic stroke and repeated cerebral haemorrhage. The underlying aetiology of both events remained undetermined despite extensive diagnostic work-up, including coagulation tests and dynamic MR angiography. It is most likely that both a pathological coagulation and increased vessel fragility comparable to amyloid angiopathy were responsible for the combined brain lesions in this patient. We conclude that KTS is a very rare but relevant aetiology of cerebral ischemia and that anticoagulation treatment in these cases should be carefully considered as the risk of cerebral haemorrhage is probably elevated.


Amyloid angiopathy; Intracerebral bleeding; Ischemic stroke; Klippel-Trenaunay Syndrome

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