Evidence of presymptomatic cognitive decline in Huntington's disease

Rhea Diamond Department of Neurology, Boston University School of Medicine, ; Department of Brain and Cognitive Sciences, Massachusetts Institute of Technology,

Roberta F. White Department of Neurology, Boston University School of Medicine, ; Department of Psychology, Boston Veterans Administration Medical Center, ; Department of Psychiatry, Tufts University Medical School,

Richard H. Myers Department of Neurology, Boston University School of Medicine, ; Department of Neurology, Massachusetts General Hospital, Harvard Medical School,

Carol Mastromauro Department of Neurology, Boston University School of Medicine, ; Department of Neurology, Massachusetts General Hospital,

Walter J. Koroshetz Department of Neurology, Massachusetts General Hospital, Harvard Medical School,

Nelson Butters Psychology Service, San Diego Veterans Administration Medical Center, ; Department of Psychiatry, University of California, San Diego

Deborah M. Rothstein Department of Adult Psychology, Charles River Hospital, Wellesley, MA

Mark B. Moss Department of Anatomy, Boston University School of Medicine, ; Department of Psychiatry, Massachusetts General Hospital, Harvard Medical School,

Jennifer Vasterling Psychology Service, New Orleans Veterans Affairs Medical Center,

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Abstract

Asymptomatic persons at risk for Huntington's disease (HD) (N = 28) were as-sessed with neuropsychological, psychiatric, and neurologic tests while undergoing genetic linkage studies to determine their probability of carrying the HD gene. Those participants who were subsequently identified as probable gene carriers did not differ on neurologic or psychiatric examination from those subsequently identified as probable noncarriers. Neuropsychological data are presented for a subset of participants free of other conditions (such as alcoholism) putting them at risk for cognitive deficits. Among these subjects, probable gene carriers were inferior to probable noncarriers on the neuropsychological battery as a whole and on several individual tests involving learning and memory. The results suggest the presence of cognitive decline prior to identifiable motor impairments in HD.

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Evidence of presymptomatic cognitive decline in Huntington's disease

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Evidence of presymptomatic cognitive decline in Huntington's disease

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