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Progression in Prediagnostic Huntington Disease
  1. Jason Rupp
  1. Department of Medical and Molecular Genetics, Indiana University School of Medicine, United States
    1. Tanya Blekher
    1. Department of Ophthalmology, Indiana University School of Medicine, United States
      1. Jacqueline Jackson
      1. Department of Medical and Molecular Genetics, Indiana University School of Medicine, United States
        1. Xabier Beristain
        1. Department of Neurology, Indiana University School of Medicine, United States
          1. Jeanine Marshall
          1. Department of Medical and Molecular Genetics, Indiana University School of Medicine, United States
            1. Siu Hui
            1. Department of Medicine, Division of Biostatistics, Indiana University School of Medicine, United States
              1. Joanne Wojcieszek
              1. Department of Neurology, Indiana University School of Medicine, United States
                1. Tatiana Foroud (tforoud{at}iupui.edu)
                1. Department of Medical and Molecular Genetics, Indiana University School of Medicine, United States

                  Abstract

                  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; n=68), or 2) with the CAG expansion (CAG+; n=38). The CAG+ were 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+ 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 4 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 CAG+ had a constant rate of decline throughout the prediagnostic period that was more rapid than in CAG- subjects.

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