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.