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Clin Neurol Neurosurg. 2008 Mar;110(3):262-4. Epub 2007 Oct 29.

Intracerebral haemorrhage as first manifestation of pseudoxanthoma elasticum.

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  • 1Department of Neurology, University Hospital Zuerich, Frauenklinikstr. 26, 8091 Zuerich, Switzerland.


Pseudoxanthoma elasticum (PXE), a rare inherited, progressive connective tissue disorder, is an uncommon cause of ischemic stroke, mostly due to arterial occlusive small vessel disease. Intracranial haemorrhage rarely occurs in PXE. We report a patient with cerebral bleeding as the first manifestation of PXE, which has not been described before. This atypical presentation led to delayed diagnosis, which was not made until 3 years after the haemorrhage. PXE is a rare but noteworthy cause of ischemic and haemorrhagic stroke and should be considered in young patients lacking vascular risk factors.

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