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 - Mar 14 2007

Keywords

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

ASJC Scopus subject areas

  • Experimental and Cognitive Psychology
  • Cognitive Neuroscience
  • Behavioral Neuroscience

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  • Cite this

    Solomon, A. C., Stout, J. C., Johnson, S. A., Langbehn, D. R., Aylward, E. H., Brandt, J., Ross, C. A., Beglinger, L., Hayden, M. R., Kieburtz, K., Kayson, E., Julian-Baros, E., Duff, K., Guttman, M., Nance, M., Oakes, D., Shoulson, I., Penziner, E., & Paulsen, J. S. (2007). Verbal episodic memory declines prior to diagnosis in Huntington's disease. Neuropsychologia, 45(8), 1767-1776. https://doi.org/10.1016/j.neuropsychologia.2006.12.015