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  • Title: [Ischemic cerebrovascular complications in Rendu-Osler disease: a case].
    Author: Albucher JF, Carles P, Giron J, Waubant E, Guiraud-Chaumeil B, Chollet F, Clanet M.
    Journal: Rev Neurol (Paris); 1996 Apr; 152(4):283-7. PubMed ID: 8763658.
    Abstract:
    Rendu-Osler disease is a familial disorder transmitted as an autosomal dominant trait of high penetrance. It is characterized by telengiectasias of the skin, mucous membranes and viscera, associated with recurrent bleedings. Neurological complications (brain abcesses and hemorrhagic manifestations) occur in 10% of the patients. Neurological symptoms are often associated with arteriovenous fistula of the lung (50%). Ischaemic strokes occuring in such patients with an hemorrhagic disease while unfrequent, have been described. The pathophysiology of stroke in that case remains unclear. Polycythemia causing hyperviscosity, air embolism following hemoptysis, paradoxical embolism through right-to-left shunt have been proposed. We report a new case of ischaemic strokes occuring in a caucasian forty-year-old woman, with Rendu-Osler disease (familial history, epistaxis, telengiectasias) and with an arteriovenous malformation of the right lung. She presented two strokes and one transient ischaemic attack. Her pulmonary malformation was occluded by embolization. The role of arteriovenous malformation in the pathophysiology of strokes is discussed.
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