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Intra-individual variability in prodromal Huntington disease and its relationship to genetic burden

  • Author(s): Musso, M
  • Westervelt, HJ
  • Long, JD
  • Morgan, E
  • Woods, SP
  • Smith, MM
  • Lu, W
  • Paulsen, JS
  • Cross, S
  • Ryan, P
  • Epping, EA
  • Chiu, E
  • Preston, J
  • Goh, A
  • Antonopoulos, S
  • Loi, S
  • Chua, P
  • Komiti, A
  • Raymond, L
  • Decolongon, J
  • Fan, M
  • Coleman, A
  • Ross, CA
  • Varvaris, M
  • Yoritomo, N
  • Mallonee, WM
  • Suter, G
  • Samii, A
  • Macaraeg, A
  • Jones, R
  • Wood-Siverio, C
  • Factor, SA
  • Barker, RA
  • Mason, S
  • Guzman, NV
  • McCusker, E
  • Griffith, J
  • Loy, C
  • Gunn, D
  • Orth, M
  • Süßmuth, S
  • Barth, K
  • Trautmann, S
  • Schwenk, D
  • Eschenbach, C
  • Quaid, K
  • Wesson, M
  • Wojcieszek, J
  • Guttman, M
  • Sheinberg, A
  • Karmalkar, I
  • Perlman, S
  • Clemente, B
  • Geschwind, MD
  • Sha, S
  • Satris, G
  • Warner, T
  • Burrows, M
  • Rosser, A
  • Price, K
  • Hunt, S
  • Marshall, F
  • Chesire, A
  • Wodarski, M
  • Hickey, C
  • Panegyres, P
  • Lee, J
  • Tedesco, M
  • Maxwell, B
  • Perlmutter, J
  • Barton, S
  • Smith, S
  • Miedzybrodzka, Z
  • Rae, D
  • D'Alessandro, M
  • Craufurd, D
  • Bek, J
  • Howard, E
  • Mazzoni, P
  • Marder, K
  • Wasserman, P
  • Kumar, R
  • Erickson, D
  • Nickels, B
  • Wheelock, V
  • Kjer, L
  • Martin, A
  • Farias, S
  • Suchowersky, O
  • Martin, W
  • King, P
  • Wieler, M
  • Sran, S
  • Ahmed, A
  • Rao, S
  • Reece, C
  • et al.
Abstract

© INS. The International Neuropsychological Society 2015. The current study sought to examine the utility of intra-individual variability (IIV) in distinguishing participants with prodromal Huntington disease (HD) from nongene-expanded controls. IIV across 15 neuropsychological tasks and within-task IIV using a self-paced timing task were compared as a single measure of processing speed (Symbol Digit Modalities Test [SDMT]) in 693 gene-expanded and 191 nongene-expanded participants from the PREDICT-HD study. After adjusting for depressive symptoms and motor functioning, individuals estimated to be closest to HD diagnosis displayed higher levels of across- and within-task variability when compared to controls and those prodromal HD participants far from disease onset (FICV(3,877) = 11.25; p<.0001; FPacedTiming(3,877) = 22.89; p<.0001). When prodromal HD participants closest to HD diagnosis were compared to controls, Cohen's d effect sizes were larger in magnitude for the within-task variability measure, paced timing (-1.01), and the SDMT (-0.79) and paced tapping coefficient of variation (CV) (-0.79) compared to the measures of across-task variability [CV (0.55); intra-individual standard deviation (0.26)]. Across-task variability may be a sensitive marker of cognitive decline in individuals with prodromal HD approaching disease onset. However, individual neuropsychological tasks, including a measure of within-task variability, produced larger effect sizes than an index of across-task IIV in this sample. (JINS, 2015, 21, 8-21)

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