STROKE IN FABRYS-DISEASE

This study was performed to characterize the frequency, clinical prese ntation and etiology of cerebrovascular complications in patients with Fabry's disease. Thirty-three patients (age range 6-64 years) with Fa bry's disease were reviewed, eight (24%) of whom suffered cerebrovascu lar complications. All patients developed ischemic strokes involving s mall arterial vessels which occurred in equal frequency in carotid and vertebrobasilar distributions. In six of these eight patients, a stro ke occurred prior to age 40 years. Stroke frequently complicates Fabry 's disease and represents a significant source of morbidity in patient s affected with this condition; it usually involves thrombosis of smal l arteries affected by the disease process.