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Rendu-Osler-Weber syndrome: a current perspective on cerebral manifestations
M J McDonald1, B P Brophy, C Kneebone
1Department of Neurosurgery, Flinders Medical Centre, Adelaide, South Australia, Australia.
Abstract:
Rendu-Osler-Weber syndrome (hereditary haemorrhagic telangiectasia) is a vascular dysplasia characterized by recurrent epistaxis, mucocutaneous telangiectasia and a family history of the disorder. Although rare, it may cause significant morbidity to healthy and young individuals. We report three cases highlighting the cerebral manifestations of this disorder. These cases include cerebral abscess, cerebral haemorrhage and embolic stroke. These cerebral manifestations are due to complications associated with pulmonary arteriovenous fistulae or cerebral vascular malformations. The current management and screening for this disorder are reviewed.
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