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

Mov Disord. 2016 Oct;31(10):1466-1478. doi: 10.1002/mds.26675.


Behavioral symptoms are an important feature of Huntington's disease and contribute to impairment in quality of life. The Movement Disorder Society commissioned the assessment of the clinimetric properties of rating scales in Huntington's disease to make recommendations regarding their use, following previously used standardized criteria. A systematic literature search was conducted to identify the scales used to assess behavioral symptoms in Huntington's disease. For the purpose of this review, 7 behavioral domains were deemed significant in Huntington's disease: irritability, anxiety, depression, apathy, obsessive-compulsive behaviors, psychosis, and suicidal ideation. We included a total of 27 behavioral rating scales, 19 of which were of a single behavioral domain and the remaining 8 scales included multiple behavioral domains. Three rating scales were classified as "recommended" exclusively for screening purposes: the Irritability Scale for irritability, the Beck Depression Inventory-II, and the Hospital Anxiety and Depression Scale for depression. There were no "recommended" scales for other purposes such as diagnosis, severity, or change in time or to treatment. The main challenges identified for assessment of behavioral symptoms in Huntington's disease are the co-occurrence of multiple behavioral symptoms, the particular features of a behavioral symptom in Huntington's disease, and the need to address stage- and disease-specific features, including cognitive impairment and lack of insight. The committee concluded that there is a need to further validate currently available behavioral rating scales in Huntington's disease to address gaps in scale validation for specific behavioral domains and purpose of use. © 2016 International Parkinson and Movement Disorder Society.

Keywords: Huntington's disease; anxiety; behavior; depression; irritability; rating scales.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Behavioral Symptoms / diagnosis
  • Behavioral Symptoms / etiology
  • Behavioral Symptoms / physiopathology*
  • Humans
  • Huntington Disease / complications
  • Huntington Disease / diagnosis
  • Huntington Disease / physiopathology*
  • Psychiatric Status Rating Scales / standards*
  • Severity of Illness Index*