Progression in prediagnostic Huntington disease

Jason Rupp, Tanya Blekher, Jacqueline Jackson, Xabier Beristain, Jeanine Marshall, Siu Hui, Joanne Wojcieszek, Tatiana Foroud

Research output: Contribution to journalArticle

34 Scopus citations


Objective To examine rates of decline in individuals at risk for Huntington disease (HD). Methods 106 individuals at risk for HD completed a battery of neurocognitive, psychomotor and oculomotor tasks at two visits, approximately 2.5 years apart. Participants were classified as: (1) without the CAG expansion (normal controls, NC; n68) or (2) with the CAG expansion (CAG+; n38). The CAG+ group was further subdivided into those near to (near; n=19) or far from (far; n-19) their estimated age of onset. Longitudinal performance in the CAG+ group was evaluated with a repeated measures model with two main effects (time to onset, visit) and their interaction. Analysis of covariance was employed to detect differences in longitudinal performance in the three groups (NC, near and far). Results In the CAG+, the interaction term was significant (p≤0.02) for four measures (movement time, alternate button tapping, variability of latency for a memory guided task and percentage of errors for a more complex memory guided task), suggesting the rate of decline was more rapid as subjects approached onset. Longitudinal progression in the three groups differed for several variables (p<0.05). In most, the near group had significantly faster progression than NC; however, comparisons of the NC and far groups were less consistent. Conclusions Different patterns of progression were observed during the prediagnostic period. For some measures, CAG+ subjects closer to estimated onset showed a more rapid decline while for other measures the CAG+ group had a constant rate of decline throughout the prediagnostic period that was more rapid than in NC.

Original languageEnglish (US)
Pages (from-to)379-384
Number of pages6
JournalJournal of Neurology, Neurosurgery and Psychiatry
Issue number4
StatePublished - Apr 2010

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology
  • Psychiatry and Mental health

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