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

Andrea C. Solomon, Julie C. Stout, Shannon A. Johnson, Douglas R. Langbehn, Elizabeth H. Aylward, Jason Brandt, Christopher A Ross, Leigh Beglinger, Michael R. Hayden, Karl Kieburtz, Elise Kayson, Elaine Julian-Baros, Kevin Duff, Mark Guttman, Martha Nance, David Oakes, Ira Shoulson, Elizabeth Penziner, Jane S. Paulsen

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
Pages (from-to)1767-1776
Number of pages10
JournalNeuropsychologia
Volume45
Issue number8
DOIs
StatePublished - 2007

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Episodic Memory
Huntington Disease
Corpus Striatum
Verbal Learning
Disease Progression
Magnetic Resonance Imaging
Control Groups
DNA
Health

Keywords

  • Basal ganglia
  • CAG
  • Cognitive
  • Early detection
  • Genetic risk
  • Striatum

ASJC Scopus subject areas

  • Behavioral Neuroscience
  • Neuropsychology and Physiological Psychology

Cite this

Verbal episodic memory declines prior to diagnosis in Huntington's disease. / Solomon, Andrea C.; Stout, Julie C.; Johnson, Shannon A.; Langbehn, Douglas R.; Aylward, Elizabeth H.; Brandt, Jason; Ross, Christopher A; Beglinger, Leigh; Hayden, Michael R.; Kieburtz, Karl; Kayson, Elise; Julian-Baros, Elaine; Duff, Kevin; Guttman, Mark; Nance, Martha; Oakes, David; Shoulson, Ira; Penziner, Elizabeth; Paulsen, Jane S.

In: Neuropsychologia, Vol. 45, No. 8, 2007, p. 1767-1776.

Research output: Contribution to journalArticle

Solomon, AC, Stout, JC, Johnson, SA, Langbehn, DR, Aylward, EH, Brandt, J, Ross, CA, Beglinger, L, Hayden, MR, Kieburtz, K, Kayson, E, Julian-Baros, E, Duff, K, Guttman, M, Nance, M, Oakes, D, Shoulson, I, Penziner, E & Paulsen, JS 2007, 'Verbal episodic memory declines prior to diagnosis in Huntington's disease', Neuropsychologia, vol. 45, no. 8, pp. 1767-1776. https://doi.org/10.1016/j.neuropsychologia.2006.12.015
Solomon, Andrea C. ; Stout, Julie C. ; Johnson, Shannon A. ; Langbehn, Douglas R. ; Aylward, Elizabeth H. ; Brandt, Jason ; Ross, Christopher A ; Beglinger, Leigh ; Hayden, Michael R. ; Kieburtz, Karl ; Kayson, Elise ; Julian-Baros, Elaine ; Duff, Kevin ; Guttman, Mark ; Nance, Martha ; Oakes, David ; Shoulson, Ira ; Penziner, Elizabeth ; Paulsen, Jane S. / Verbal episodic memory declines prior to diagnosis in Huntington's disease. In: Neuropsychologia. 2007 ; Vol. 45, No. 8. pp. 1767-1776.
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