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

Academic Article


  • 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.
  • Authors

    Published In

  • Neuropsychologia  Journal
  • Keywords

  • Adult, Age of Onset, Confidence Intervals, Disease Progression, Female, Humans, Huntington Disease, Male, Memory Disorders, Middle Aged, Neuropsychological Tests, Sex Factors, Statistics as Topic, Verbal Learning
  • Digital Object Identifier (doi)

    Author List

  • Solomon AC; Stout JC; Johnson SA; Langbehn DR; Aylward EH; Brandt J; Ross CA; Beglinger L; Hayden MR; Kieburtz K
  • Start Page

  • 1767
  • End Page

  • 1776
  • Volume

  • 45
  • Issue

  • 8