Cerebrovascular accidents at a young age in Rendu-Osler-Weber disease; a survey in the Netherlands Antilles
Thirty-two patients (17 men and 15 women) are presented in whom the diagnosis Rendu-Osler-Weber disease was established between 1980 and 1990 during hospitalisation in the Leeward Islands of the Netherlands Antilles. Among this group there were eight families, each of which contained two patients. Estimated point-prevalence rate in 1991 was 19.4/100,000, which is ten times higher than has been reported so far. Epistaxis, gastrointestinal blood loss and cerebrovascular accidents (CVA) were the main presenting symptoms. Mean age at diagnosis was 53.2 years. Arteriovenous fistulae in the lung developed in 11 patients (34%). Eleven patients experienced a total of 12 CVAs at a mean age of 48 years; four of these patients had an ischaemic CVA in the presence of pulmonary fistulae, while three patients had a haemorrhagic stroke in the presence of a cerebral arteriovenous malformation. Thus, cerebrovascular accidents at an early age are common complications in Rendu-Osler-Weber disease and warrant further investigation of this disease among inhabitants of the Leeward Islands of the Netherlands Antilles.