| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
(Hypertension. 1998;31:445.)
© 1998 American Heart Association, Inc.
Scientific Contributions |
Intracranial Aneurysm and Hemorrhagic Stroke in Glucocorticoid-remediable Aldosteronism
From the Endocrine-Hypertension Division, Brigham and Women’s Hospital and Harvard Medical School (W.R.L., R.J.W., B.F.A., R.G.D.), Boston MA 02115 and Howard Hughes Medical Institute, Departments of Medicine and Genetics, Boyer Center for Molecular Medicine (R.P.L.), Yale University School of Medicine, New Haven, CT 06510.
Correspondence to Robert G. Dluhy, MD, Endocrine-Hypertension Division, 221 Longwood Avenue, RFB-2, Boston, MA 02115. E-mail: rgdluhy{at}bics.bwh.harvard.edu
There are anecdotal reports of early cerebrovascular complications occurring in patients with glucocorticoid-remediable aldosteronism (GRA). The issue has never been systematically evaluated. In this study, we retrospectively reviewed the International Registry for GRA to see if there was an association between cerebrovascular complications and GRA. We searched the records of 376 patients from 27 genetically proven GRA pedigrees for premature death or cerebrovascular complications. Each case was subsequently verified through the referring physician, or autopsy reports. The number of complications occurring in patients with proven GRA were compared to GRA negative subjects from the same pedigrees. There were 18 cerebrovascular events in 15 patients with proven GRA (n = 167) and none in the GRA negative group (n = 194; P<.001). There were an additional 15 events in 15 subjects that were suspected of having GRA based on clinical history. Seventy percent of events were hemorrhagic strokes; the overall case fatality rate was 61%. The mean (± SD) age at the time of the initial event was 31.7±11.3 years. In total, 48% of all GRA pedigrees and 18% of all GRA patients had cerebrovascular complications, which is similar to the frequency of aneurysm in adult polycystic kidney disease. GRA is associated with high morbidity and mortality from early onset of hemorrhagic stroke and ruptured intracranial aneurysms. Screening for intracranial aneurysm with magnetic resonance angiography is advised for patients with genetically proven GRA.
Key Words: glucocorticoid-remediable aldosteronism • hyperaldosteronism • cerebrovascular disease • intracranial aneurysm • hemorrhagic stroke
Abbreviations: GRA = Glucocorticoid-remediable Aldosteronism • MR = Magenetic Resonance • BMI = Body-Mass Index • SBP = Systolic Blood Pressure • DBP = Diastolic Blood Pressure • GS = Genetically Screened • DST = Dexamethasone Suppression Test • NA = Not Available • NAF = No Aneurysm Found • ES = Early Stroke • SD = Sudden Death • DC = Death Certificate
This article has been cited by other articles:
 |
|
 |
|
  J. M. Osmond and A. M. Dorrance 11{beta}-Hydroxysteroid Dehydrogenase Type II Inhibition Causes Cerebrovascular Remodeling and Increases Infarct Size after Cerebral Ischemia Endocrinology, February 1, 2009; 150(2): 713 - 719. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. W. Funder, R. M. Carey, C. Fardella, C. E. Gomez-Sanchez, F. Mantero, M. Stowasser, W. F. Young Jr., and V. M. Montori Case Detection, Diagnosis, and Treatment of Patients with Primary Aldosteronism: An Endocrine Society Clinical Practice Guideline J. Clin. Endocrinol. Metab., September 1, 2008; 93(9): 3266 - 3281. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. M. C. Connell, S. M. MacKenzie, E. M. Freel, R. Fraser, and E. Davies A Lifetime of Aldosterone Excess: Long-Term Consequences of Altered Regulation of Aldosterone Production for Cardiovascular Function Endocr. Rev., April 1, 2008; 29(2): 133 - 154. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. M. Dorrance, N. C. Rupp, and E. F. Nogueira Mineralocorticoid Receptor Activation Causes Cerebral Vessel Remodeling and Exacerbates the Damage Caused by Cerebral Ischemia Hypertension, March 1, 2006; 47(3): 590 - 595. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. M C Connell and E. Davies The new biology of aldosterone J. Endocrinol., July 1, 2005; 186(1): 1 - 20. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. Mulatero, S. M. di Cella, T. A. Williams, A. Milan, G. Mengozzi, L. Chiandussi, C. E. Gomez-Sanchez, and F. Veglio Glucocorticoid Remediable Aldosteronism: Low Morbidity and Mortality in a Four-Generation Italian Pedigree J. Clin. Endocrinol. Metab., July 1, 2002; 87(7): 3187 - 3191. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. Mosso, C. E. Gomez-Sanchez, M. F. Foecking, and C. Fardella Serum 18-Hydroxycortisol in Primary Aldosteronism, Hypertension, and Normotensives Hypertension, September 1, 2001; 38(3): 688 - 691. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. W. Funder Editorial: Sex and the Single Gene--FH-1 J. Clin. Endocrinol. Metab., June 1, 2000; 85(6): 2158 - 2159. [Full Text]
|
|
|
|
|
|
M. Stowasser, A. W. Bachmann, P. R. Huggard, T. R. Rossetti, and R. D. Gordon Severity of Hypertension in Familial Hyperaldosteronism Type I: Relationship to Gender and Degree of Biochemical Disturbance J. Clin. Endocrinol. Metab., June 1, 2000; 85(6): 2160 - 2166. [Abstract] [Full Text]
|
|
|
|
|
|
J. A. Wyckoff, E. W. Seely, S. Hurwitz, B. F. Anderson, R. P. Lifton, and R. G. Dluhy Glucocorticoid-Remediable Aldosteronism and Pregnancy Hypertension, February 1, 2000; 35(2): 668 - 672. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Glucocorticoid-Remediable Aldosteronism J. Clin. Endocrinol. Metab., December 1, 1999; 84(12): 4341 - 4344. [Full Text]
|
|
|
|
|
|
M. Stowasser, P. R. Huggard, T. R. Rossetti, A. W. Bachmann, and R. D. Gordon Biochemical Evidence of Aldosterone Overproduction and Abnormal Regulation in Normotensive Individuals with Familial Hyperaldosteronism Type I J. Clin. Endocrinol. Metab., November 1, 1999; 84(11): 4031 - 4036. [Abstract] [Full Text]
|
|