Ten-year Rate of Longitudinal Change in Neurocognitive and Motor Function in Prediagnosis Huntington Disease

Overview

Journal Mov Disord

Date 2008 Sep 12

PMID 18785217

Citations 28

Authors

Andrea C Solomon

Julie C Stout

Marjorie Weaver

Sarah Queller

Allison Tomusk

Kathryn Burr Whitlock

Siu L Hui

Jeanine Marshall

Jacqueline Gray Jackson

Eric R Siemers

Xabier Beristain

Joanne Wojcieszek

Tatiana Foroud

Affiliations

Soon will be listed here.

Abstract

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.

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