Abstract-There are anecdotal reports of early cerebrovascular comphcatlons occurrmg m patients with glucocortlcoldremediable aldosteromsm (GRA) The issue has never been systematically evaluated In this study, we retrospectively reviewed the Internatlonal Repstry for GRA to see If there was an assoclatlon between cerebrovascular comphcatlons and GRA We searched the records of 376 patients from 27 genetically proven GRA pedigrees for premature death or cerebrovascular comphcatlons Each case was subsequently verified through the referring phynclan, or autopsy reports The number of comphcatlons occurrmg m patients with proven GRA were compared to GRA negative subjects from the same pedigrees There were 18 cerebrovascular events m 15 patients with proven GRA (n= 167) and none m the GRA negative group (n=194, P< 001) There were an additional 15 events m 15 subjects that were suspected of having GRA based on clmlcal history Seventy percent of events were hemorrhagic strokes, the overall case fatality rate was 61% The mean (2 SD) age at the time of the mltlal event was 31 72 11 3 years In total, 48% of all GRA pedigrees and 18% of all GRA patients had cerebrovascular comphcatlons, which 1s slmllar to the frequency of aneurysm m adult polycystlc kidney disease GRA 1s associated with high morbldlty and mortality from early onset of hemorrhage stroke and ruptured mtracramal aneurysms Screening for intracranial aneurysm with magnetic resonance anglography 1s advised for patients with genetically proven GRA (Hypertension. 1998;31[part 2]:445-450.)

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