Journal
NEUROPSYCHOLOGIA
Volume 45, Issue 8, Pages 1767-1776Publisher
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.neuropsychologia.2006.12.015
Keywords
cognitive; early detection; genetic risk; CAG; striatum; basal ganglia
Funding
- NIMH NIH HHS [K02 MH001579-05] Funding Source: Medline
- NINDS NIH HHS [R01 NS040068-06, R01 NS040068] Funding Source: Medline
- PHS HHS [40068, 01579] Funding Source: Medline
- NATIONAL INSTITUTE OF MENTAL HEALTH [K02MH001579] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE [R01NS040068] Funding Source: NIH RePORTER
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Previous studies of verbal episodic memory in pre-diagnostic Huntington's disease (HD) have yielded mixed results; some evidence suggests that memory decline is evident prior to the onset of pronounced neurological signs of HD, whereas other data indicate that memory function remains normal throughout the pre-diagnostic period. This study examines verbal episodic memory in a sample of CAG expanded individuals who have not yet been clinically diagnosed, and who represent a wide range of points along the continuum from health to disease. The Hopkins Verbal Learning Test-Revised (HVLT-R) was administered to 479 participants (428 with the HD CAG expansion and 51 without), and performance was compared to neurobiological indices of disease progression, including a DNA-based estimate of proximity to clinical diagnosis, magnetic resonance imaging (MRI) measures of striatal volume, and neurologist ratings of motor signs. Lower HVLT-R scores were associated with closer proximity to clinical diagnosis and smaller striatal volumes; these relationships were found even in groups with no neurological signs of HD. The CAG expanded groups, including those with only minimal neurological signs, had significantly lower HVLT-R scores than the control group, and performance was worse in sub-groups that had more neurological signs consistent with HD. These findings indicate that verbal episodic memory is affected in early pre-diagnostic HD and may decline as striatal volumes decrease and individuals approach the motor diagnostic threshold. (c) 2007 Elsevier Ltd. All rights reserved.
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