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Psychometric properties and responsiveness of Neuro-QoL Cognitive Function in persons with Huntington disease (HD)

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Abstract

Purpose

Individuals with Huntington disease (HD) experience progressive cognitive decline that may appear years before motor manifestations of the disease. These declines have a profound effect on health-related quality of life (HRQOL) over the disease course, and thus it is important that self-report measures of cognitive function are validated for use in longitudinal studies.

Methods

359 individuals with premanifest or manifest HD completed baseline and at least one follow-up (12- and 24-month) assessment. Neuro-QoL™ Cognitive Function was administered at each time-point. Participants completed a self-reported global rating of cognitive change, as well as performance-based cognitive changes (using the Symbol Digit Modalities Test). Standardized response means (SRMs) and general linear models evaluated whether Neuro-QoL™ Cognitive Function was responsive to change over time with respect to self-reported and performance-based anchors. Test–retest reliability and known-group validity were also examined.

Results

Responsiveness was supported by effect sizes that were small in magnitude, but in the expected direction relative to self-reported and performance-based change. General linear models generally supported 12- and 24-month responsiveness relative to self-reported cognitive change and 12-month responsiveness relative to performance-based change. Test–retest reliability was excellent, and the measure exhibited known-group validity.

Conclusion

Longitudinal analyses generally indicate that the Neuro-QoL™ Cognitive Function measure is sensitive to change over time in individuals with HD. Neuro-QoL Cognitive Function changes reflect self-reported cognitive change at 12 and 24 months and performance-based change at 12 months. This measure may be useful in clinical trials or longitudinal observation studies.

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Acknowledgements

Work on this manuscript was supported by the National Institutes of Health (NIH), National Institute of Neurological Disorders and Stroke (R01NS077946) and the National Center for Advancing Translational Sciences (UL1TR000433). In addition, a portion of this study sample was collected in conjunction with the Predict-HD study. The Predict-HD study was supported by the NIH, National Institute of Neurological Disorders and Stroke (R01NS040068), the NIH, Center for Inherited Disease Research (provided supported for sample phenotyping), and the CHDI Foundation (award to the University of Iowa). We thank the University of Iowa, the Investigators and Coordinators of this study, the study participants, the National Research Roster for Huntington Disease Patients and Families, the Huntington Study Group, and the Huntington’s Disease Society of America. We acknowledge the assistance of Jeffrey D. Long, Hans J. Johnson, Jeremy H. Bockholt, Roland Zschiegner, and Jane S. Paulsen. We also acknowledge Roger Albin, Kelvin Chou, and Henry Paulsen for the assistance with participant recruitment. The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH. HDQLIFE Site Investigators and Coordinators: Noelle Carlozzi, Praveen Dayalu, Stephen Schilling, Amy Austin, Matthew Canter, Siera Goodnight, Jennifer Miner, Nicholas Migliore (University of Michigan, Ann Arbor, MI); Jane Paulsen, Nancy Downing, Isabella DeSoriano, Courtney Shadrick, Amanda Miller (University of Iowa, Iowa City, IA); Kimberly Quaid, Melissa Wesson (Indiana University, Indianapolis, IN); Christopher Ross, Gregory Churchill, Mary Jane Ong (Johns Hopkins University, Baltimore, MD); Susan Perlman, Brian Clemente, Aaron Fisher, Gloria Obialisi, Michael Rosco (University of California Los Angeles, Los Angeles, CA); Michael McCormack, Humberto Marin, Allison Dicke (Rutgers University, Piscataway, NJ); Joel Perlmutter, Stacey Barton, Shineeka Smith (Washington University, St. Louis, MO); Martha Nance, Pat Ede (Struthers Parkinson’s Center); Stephen Rao, Anwar Ahmed, Michael Lengen, Lyla Mourany, Christine Reece, (Cleveland Clinic Foundation, Cleveland, OH); Michael Geschwind, Joseph Winer (University of California – San Francisco, San Francisco, CA), David Cella, Richard Gershon, Elizabeth Hahn, Jin-Shei Lai (Northwestern University, Chicago, IL).

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Authors and Affiliations

  1. Department of Physical Medicine and Rehabilitation, University of Michigan, Ann Arbor, MI, USA

    Noelle E. Carlozzi & Nicholas R. Boileau

  2. Departments of Psychiatry, Neurology, and Psychological and Brain Sciences, The University of Iowa, Iowa City, IA, USA

    Jane S. Paulsen

  3. College of Nursing, Texas A&M, Bryan, TX, USA

    Nancy R. Downing

  4. Department of Psychological and Brain Sciences, University of Massachusetts, Amherst, MA, USA

    Rebecca Ready

  5. Department of Neurology, Washington University in St. Louis, St. Louis, MO, USA

    Joel S. Perlmutter & Stacey Barton

  6. Department of Radiology, Washington University in St. Louis, St. Louis, MO, USA

    Joel S. Perlmutter

  7. Department of Medical Social Sciences, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    David Cella & Jin-Shei Lai

  8. Department of Neurology, University of Michigan, Ann Arbor, MI, USA

    Kelvin L. Chou

  9. Department of Pathology, Rowan-School of Medicine, Stratford, NJ, USA

    Michael K. McCormack

  10. Department of Psychiatry, Rutgers-Robert Wood Johnson Medical School, Piscataway, NJ, USA

    Michael K. McCormack

  11. Department of Physical Medicine and Rehabilitation, University of Michigan, North Campus Research Complex, 2800 Plymouth Road, Building NCRC B14, Room G216, Ann Arbor, MI, 48109-2800, USA

    Noelle E. Carlozzi

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  1. Noelle E. Carlozzi
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  2. Nicholas R. Boileau
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  11. Jin-Shei Lai
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Correspondence to Noelle E. Carlozzi.

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The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. University of Michigan Medical School Institutional Review Board, HUM00055669, initial approval 2/1/2012. Cleveland Clinic Institutional Review Board, IRB# 13-460, initial approval 4/26/2013. Indiana University Institutional Review Board (IRB-01), Protocol # 1208009383, initial approval 9/7/2012. Johns Hopkins Medicine Institutional Review Board, Study # NA_00079341, initial approval 12/13/2012. University of Medicine & Dentistry of New Jersey (which was subsumed by Rutgers University) Institutional Review Board, Study ID Pro2012002196, initial approval 4/4/2013. Park Nicollet Institute Institutional Review Board, Study 04334-13-A, initial approval 11/15/2013. University of California San Francisco Institutional Review Board, IRB # 13-10880 Reference # 065701, initial approval 9/4/2013. University of California Los Angeles Institutional Review Board, IRB # 12-000743, initial approval 6/12/2012. University of Iowa Institutional Review Board, IRB ID # 201301724, initial approval 1/17/2013. Washington University in St. Louis Institutional Review Board, IRB ID # 201206052, initial approval 8/14/2012. Northwestern University Institutional Review Board deemed this study (STU00200337) was determined exempt (“Not Human Research Determination”) on 2/9/2015.

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Carlozzi, N.E., Boileau, N.R., Paulsen, J.S. et al. Psychometric properties and responsiveness of Neuro-QoL Cognitive Function in persons with Huntington disease (HD). Qual Life Res 29, 1393–1403 (2020). https://doi.org/10.1007/s11136-019-02391-7

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