Rating scales for cognition in Huntington's disease: Critique and recommendations

Tiago A. Mestre, Anne Catherine Bachoud-Lévi, Johan Marinus, Julie C. Stout, Jane S. Paulsen, Peter Como, Kevin Duff, Cristina Sampaio, Christopher G. Goetz, Esther Cubo, Glenn T. Stebbins, Pablo Martinez-Martin, the Members of the MDS Committee on Rating Scales Development

Research output: Contribution to journalReview ArticleResearchpeer-review

34 Citations (Scopus)


Cognitive impairment is one of the main features of Huntington's disease and is present across the disease spectrum. As part of the International Parkinson's Disease and Movement Disorder Society-sponsored project to review all clinical rating scales used in Huntington's disease, a systematic review of the literature was performed to identify cognitive scales used in Huntington's disease and make recommendations for their use. A total of 17 cognitive scales were identified and evaluated. None of the scales met criteria for a “recommended” status. For assessing severity of cognitive dysfunction, the Montreal Cognitive Assessment was “recommended with caveats.” The UHDRS Cognitive Assessment, the UHDRS-For Advanced Patients cognitive section, the Alzheimer's Disease Assessment Scale-Cognitive Subscale, the Frontal Assessment Battery, the Mattis Dementia Rating Scale, the Mini-Mental State Examination, and the Repeatable Battery for the Assessment of Neuropsychological Status were “suggested” for evaluating severity of cognitive impairment. The MoCA was “suggested” as a screening tool for cognitive impairment. The major challenge in the assessment of cognition in Huntington's disease is the lack of a formal definition of dementia and/or mild cognitive impairment in this disease. The committee concluded that there is a need to further validate currently available cognitive scales in Huntington's disease, but that it is premature to recommend the development of new scales. Recently developed Huntington's disease-specific scales, such as the Huntington's Disease-Cognitive Assessment Battery, hold promise but require the completion of more comprehensive clinimetric development.

Original languageEnglish
Pages (from-to)187-195
Number of pages9
JournalMovement Disorders
Issue number2
Publication statusPublished - 1 Feb 2018


  • clinimetrics
  • cognition
  • Huntington's disease
  • rating scales
  • validation

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