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Journal of the International Neuropsychological Society (1998), 4 : 467-473 Cambridge University Press
doi:10.1017/S1355617798455061
 
Journal of the International Neuropsychological Society (1998), 4:467-473 Cambridge University Press
Copyright © 1998 The International Neuropsychological Society
Research Article

When does Huntington's disease begin?

JEFFREY R. CAMPODONICO a1 , ELIZABETH AYLWARD a1 , ANN-MARIE CODORI a1 , CANDACE YOUNG a1 , LAURA KRAFFT a1 , MICHAEL MAGDALINSKI a1 , NEAL RANEN a1 , PHILLIP R. SLAVNEY a1 and JASON BRANDT a1 c1
a1 Department of Psychiatry and Behavioral Sciences, The Johns Hopkins University School of Medicine

Abstract

Recent studies have detected basal ganglia atrophy in clinically asymptomatic persons with the genetic mutation that causes Huntington's disease (HD). Whether reductions in caudate and putamen volume on MRI scans are associated with changes in cognitive and neurologic functioning was examined in 13 healthy adults with the IT-15 mutation. Reduced striatal volume was found to correlate with greater neurologic (largely motor) impairment, slower mental processing speed, and poorer verbal learning, although none of the participants met even liberal criteria for clinical diagnosis of HD. These correlations are strikingly similar to those observed in symptomatic HD patients, possibly reflecting the earliest manifestations of disease. (JINS, 1998, 4, 467–473.)

(Received August 4 1997)
(Revised January 15 1998)
(Accepted January 30 1998)

Key Words: Huntington's disease; Genetics; Presymptomatic testing; MRI scans; Cognitive tests.

Correspondence:

c1 Reprint requests to: Jason Brandt, Department of Psychiatry and Behavioral Sciences, Meyer 218, The Johns Hopkins Hospital, 600 N. Wolfe Street, Baltimore, MD 21287-7218. E-mail: jbrandt@welchlink.welch.jhu.edu


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