ISCHEMIC STROKES OCCURING IN RENDU-OSLER- DISEASE (HEREDITARY HEMORRHAGIC TELENGIECTASIA) - 1 CASE

Citation
Jf. Albucher et al., ISCHEMIC STROKES OCCURING IN RENDU-OSLER- DISEASE (HEREDITARY HEMORRHAGIC TELENGIECTASIA) - 1 CASE, Revue neurologique, 152(4), 1996, pp. 283-287
Citations number
11
Categorie Soggetti
Clinical Neurology
Journal title
ISSN journal
00353787
Volume
152
Issue
4
Year of publication
1996
Pages
283 - 287
Database
ISI
SICI code
0035-3787(1996)152:4<283:ISOIRD>2.0.ZU;2-S
Abstract
Rendu-Osler disease is a familial disorder transmitted as an autosomal dominant trait of high penetrance. It is characterized by telengiecta sias of the skin, mucous membranes and viscera, associated with recurr ent bleedings. Neurological complications (brain abcesses and hemorrha gic manifestations) occur in 10% of the patients. Neurological symtoms are often associated with arteriovenous fistula of the lung (50%). Is chaemic strokes occuring in such patients with and 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 strok es occuring in a caucasian forty-year-old woman, with Rendu-Osler dise ase (familial history, epistaxis, telengiectasias) and with an arterio venous malformation of the right lung. She presented two strokes and o ne transient ischaemic attack. Her pulmonary malformation was occluded by embolization. The role of arteriovenous malformation in the pathop hysiology of strokes is discussed.