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When does Huntington's disease begin?

Published online by Cambridge University Press:  01 September 1998

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

Keywords

Huntington's diseaseGeneticsPresymptomatic testingMRI scansCognitive tests
Type
Research Article
Information
Journal of the International Neuropsychological Society , Volume 4 , Issue 5 , September 1998 , pp. 467 - 473
Copyright
© 1998 The International Neuropsychological Society

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