Evidence of presymptomatic cognitive decline in Huntington's disease.
Asymptomatic persons at risk for Huntington's disease (HD) (N = 28) were assessed 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.
Diamond
R
R
Department of Neurology, Boston University School of Medicine, MA 02118-2394.
White
R F
RF
Myers
R H
RH
Mastromauro
C
C
Koroshetz
W J
WJ
Butters
N
N
Rothstein
D M
DM
Moss
M B
MB
Vasterling
J
J
D000328:Adult; D003072:Cognition Disorders / Q000235:genetics* / Q000523:psychology; D005260:Female; D006580:Genetic Carrier Screening; D008040:Genetic Linkage / Q000235:genetics*; D005819:Genetic Markers / Q000235:genetics; D005820:Genetic Testing; D006801:Humans; D006816:Huntington Disease / Q000235:genetics* / Q000523:psychology; D007360:Intelligence / Q000235:genetics; D008297:Male; D009483:Neuropsychological Tests; D012307:Risk Factors; D012803:Sick Role
D005819:Genetic Markers