Ten-year rate of longitudinal change in neurocognitive and motor function in prediagnosis Huntington disease.
Longitudinal studies of neurocognitive function in prediagnosis Huntington disease (pre-HD) have been few, and duration of follow-up has been brief. In this study, 155 individuals at-risk for HD completed a battery of cognitive and motor tasks at two study visits approximately 10 years apart. Participants were classified as: (1) at-risk, without the CAG expansion (healthy controls, NC; n = 112), or (2) CAG expanded (CAG+; n = 43). To examine the rate of decline at different stages of the pre-HD period, participants in the CAG+ group were further characterized as converters (i.e., individuals who developed manifest HD over the course of the study; n = 21) or nonconverters (n = 22), and their performances were compared. The CAG+ group exhibited faster rates of neurocognitive decline over the course of the study, relative to the NC group. In addition, more rapid decline was associated with closer proximity to estimated age of disease onset in the CAG+ group. Faster rates of motor and psychomotor decline were observed in the CAG+ converter group, relative to the nonconverters. These findings suggest that neurocognitive decline in pre-HD, particularly in motor and psychomotor domains, begins insidiously and accelerates as individuals approach disease onset.
Solomon
Andrea C
AC
Department of Psychological and Brain Sciences, Indiana University, Bloomington, Indiana 46202-5251, USA.
Stout
Julie C
JC
Weaver
Marjorie
M
Queller
Sarah
S
Tomusk
Allison
A
Whitlock
Kathryn Burr
KB
Hui
Siu L
SL
Marshall
Jeanine
J
Jackson
Jacqueline Gray
JG
Siemers
Eric R
ER
Beristain
Xabier
X
Wojcieszek
Joanne
J
Foroud
Tatiana
T
D000328:Adult; D017668:Age of Onset; D003071:Cognition / Q000502:physiology*; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology*; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D009043:Motor Activity / Q000502:physiology*; D009483:Neuropsychological Tests; D011597:Psychomotor Performance / Q000502:physiology; D011930:Reaction Time / Q000235:genetics; D012720:Severity of Illness Index; D019680:Trinucleotide Repeat Expansion / Q000235:genetics
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