Verbal episodic memory declines prior to diagnosis in Huntington's disease.

Full Abstract

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.

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Author information

Author/s: Solomon, Andrea C (AC); Stout, Julie C (JC); Johnson, Shannon A (SA); Langbehn, Douglas R (DR); Aylward, Elizabeth H (EH); Brandt, Jason (J); Ross, Christopher A (CA); Beglinger, Leigh (L); Hayden, Michael R (MR); Kieburtz, Karl (K); Kayson, Elise (E); Julian-Baros, Elaine (E); Duff, Kevin (K); Guttman, Mark (M); Nance, Martha (M); Oakes, David (D); Shoulson, Ira (I); Penziner, Elizabeth (E); Paulsen, Jane S (JS); Predict-HD investigators of the Huntington Study Group;

Affiliation: Department of Psychological and Brain Sciences, Indiana University, 1101 E. 10th Street, Bloomington, IN 47405, USA.

Grants: 01579 (Agency:PHS HHS) ; 40068 (Agency:PHS HHS) ; K02 MH001579-05 (Agency:NIMH NIH HHS) ; R01 NS040068-06 (Agency:NINDS NIH HHS)

Journal and publication information

Publication Type: Journal Article; Multicenter Study; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.

Journal: Neuropsychologia (Neuropsychologia), published in England. (Language: eng)

Reference: 2007-Apr; vol 45 (issue 8) : pp 1767-76

Dates: Created 2007/03/16; Completed 2007/06/21; Revised 2008/10/23;

PMID: 17303196, status: MEDLINE (last retrieval date: 12/26/2008)

Sourced from the National Library of Medicine. Abstract text and other information may be subject to copyright.

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MeSH headings (categories)

This article was linked to the MESH Headings shown below.

Adult
Age of Onset
Confidence Intervals
Disease Progression
Female
Humans
Huntington Disease - complications; diagnosis; genetics

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