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May 11, 2021; 96 (19) ArticleOpen Access

Timing and Impact of Psychiatric, Cognitive, and Motor Abnormalities in Huntington Disease

Branduff McAllister, James F. Gusella, G. Bernhard Landwehrmeyer, Jong-Min Lee, Marcy E. MacDonald, Michael Orth, Anne E. Rosser, Nigel M. Williams, Peter Holmans, Lesley Jones, Thomas H. Massey, on behalf of the REGISTRY Investigators of the European Huntington's Disease Network
First published March 25, 2021, DOI: https://doi.org/10.1212/WNL.0000000000011893
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Article Figures & Data

Figures

  • Figure 1
    Figure 1 Initial Manifestation of HD Varies With Age and CAG Length

    All included individuals had a pathogenic CAG length (36–93) and confirmed Huntington disease (HD) onset age determined by a rating clinician. (A) Frequency of different onset types in 4 age groups, chosen to show juvenile HD and then 20-year bins for clarity. Total n= 6,289, <20 years n = 188, 20 to 40 years n = 2,216, 40 to 60 years n = 3,276, >60 years n = 609. (B) Frequency of different onset types in 6 CAG length groups, chosen for clarity across the pathogenic range. Total n = 6,289, 36 to 39 CAG n = 156, 40 to 44 CAG n = 3,813, 45 to 49 CAG n = 1,735, 50 to 54 CAG n = 387, 55 to 59 CAG n = 97, >60 CAG n = 101.

  • Figure 2
    Figure 2 Onsets of Cognitive and Psychiatric Symptoms Relative to Motor Onset in HD

    Age at onset of motor symptoms was subtracted from the age at onset of each cognitive/psychiatric symptom when present. Timings of up to ± 40 years relative to motor onset shown. Only individuals with a rater-confirmed age at onset and CAG length (36–93) were included. Data from Huntington Disease (HD) Clinical Characteristics Questionnaire. (A) Cognitive impairment n = 3,225; (B) apathy n = 2,852; (C) depression n = 3,495; and (D) irritability n = 3,235.

  • Figure 3
    Figure 3 Mean Ages at Onset for Motor and Psychiatric Symptoms at Different CAG Repeat Lengths

    Shown are the mean ages at symptom onset as recorded by the Huntington Disease Clinical Characteristics Questionnaire for apathy (n = 2,739), cognitive impairment (n = 3,069), depression (n = 3,399), irritability (n = 3,117), and motor symptoms (n = 4,889).

Tables

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Letters: Rapid online correspondence

  • Author Response: Timing and Impact of Psychiatric, Cognitive, and Motor Abnormalities in Huntington Disease
    • Thomas H Massey, Clinical Academic Neurologist, Cardiff University
    • Branduff McAllister, Research Associate, Cardiff University
    • Anne E Rosser, Professor, Cardiff University
    • Lesley Jones, Professor, Cardiff University
    Submitted December 10, 2021
  • Reader Response: Timing and Impact of Psychiatric, Cognitive, and Motor Abnormalities in Huntington Disease
    • Thomas D Bird, Professor, University of Washington, Seattle, WA, USA
    • Herwig Lange, Professor, George Huntington Institute, Muenster, Germany
    • Travis Cruickshank, Professor, Centre for Precision Health, Perth, Australia
    • Matthias Dose, Professor, Kliniken des Bezirks Oberbayern, Munchen, Germany
    • Clare Eddy, Professor, Birmingham & Solihull Mental Health, NHS Foundation Trust, National Centre for Mental Health, University of Birmingham, UK
    • Mayke Oosterloo, Professor, Maastricht UMC, Maastricht, The Netherlands
    • Jane S. Paulsen, Professor, University of Wisconsin, Madison, WI, USA
    • Ralf Reilmann, Professor, George Huntington Institute, Muenster, Germany
    • Hugh Rickards, Professor, Birmingham & Solihull Mental Health, NHS Foundation Trust, National Centre for Mental Health, University of Birmingham, UK
    • Raymund A.C. Roos, Professor, Department of Neurology, LUMC, Leiden, The Netherlands
    • Adolf Weindl, Professor, Department of Neurology, Technical University of Munich, Munich, Germany
    Submitted October 18, 2021
  • Reader Response: Timing and Impact of Psychiatric, Cognitive, and Motor Abnormalities in Huntington Disease
    • Ciaran M. Considine, Assistant Professor of Neurology, Vanderbilt University Medical Center
    • Mary Edmondson, Founding Medical Director of HD Reach, Vidant Behavioral Health
    • Erin Stimming, Associate Professor of Neurology and Director of the Huntington Disease Center of Excellence, McGovern Medical School at University of Texas Health Science Center-Houston
    • Claudia Testa, Professor of Neurology and Founding Director of the Huntington Disease Center of Excellence, Virginia Commonwealth University
    • Karen E. Anderson, Professor of Neurology & Psychiatry, Director of the Huntington’s Disease Care, Education & Research Center at MedStar, Georgetown University Hospital
    • Daniel O. Claassen, Associate Professor of Neurology, Division Chief of Behavioral & Cognitive Neurology, Director of the Huntington Disease Cen, Vanderbilt University Medical Center
    Submitted April 29, 2021
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