Objective: To provide an update on the MDS-sponsored Systematic Review of Rating Scales for Huntington’s Disease (HD). 

Background: The use and validity of clinical rating scales in HD has not been critically appraised. The current review aims to provide a recommendation for the use of these scales, and identify gaps in their validity that ultimately will increase the quality of clinical measurement in HD. We have previously published the review on ratings scales in the behaviour domain. ¹

Methods: The Sub-Committee on the Systematic Review of Rating Scales for Huntington’s Disease was composed of four independent teams that reviewed clinical rating scales in the 1) behaviour, 2) motor, 3) cognitive, and 4) functional ability and quality of life domains. We used standard methodology and the classification system for rating scales developed previously by the Rating Scales Committee of the MDS. Recommendations for use of a rating scale are aligned with the purposes of measurement of severity, screening and diagnosis.

Results: 113 rating scales were considered for in-depth review, and 65 rating scales were included in the review: motor = 9, cognitive=14, functional ability=27, and QoL=15. The following scales reached a “recommended” level: Motor domain – the Unified Huntington Disease Ratings Scale – motor section for measurement of severity. Functional ability domain – the Tinetti Mobility Test for screening purposes (gait and balance problems), the Berg Balance Scale (balance impairment), and Six-Minute Walk Test (walking endurance) for measurement of severity. QoL domain – Medical Outcomes Study 36-Item Short-Form Health Survey for measurement of severity. There was no “recommended” scale for caregiver-centered QoL, as well as for patient participation in Activities of Daily Living. Cognitive domain – none.

Conclusions: Many rating scales have been used in HD, and some are HD-specific. The Sub-Committee found that further validation of existing clinical rating scales is required in the various domains. Limitations of patient self-report in HD, validity according to the disease stage and responsiveness are important aspects in need of further development. Prodromal HD stage requires particular attention for scale development, namely due to the presence of ceiling/floor effects; this disease stage likely requires a dedicated rating scale.

References: * authors report on behalf of the Members of the MDS Sub-Committee on Systematic Review of Rating Scales for Huntington’s Disease

1. Mestre TA, van Duijn E, Davis AM, Bachoud-Lévi AC, Busse M, Anderson KE, Ferreira JJ, Mahlknecht P, Tumas V, Sampaio C, Goetz CG, Cubo E, Stebbins GT, Martinez-Martin P; Members of the MDS Committee on Rating Scales Development Rating scales for behavioral symptoms in Huntington’s disease: Critique and recommendations. Mov Disord. 2016 Oct;31(10):1466-1478

« Back to 2017 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/update-on-the-mds-sponsored-systematic-review-on-clinical-rating-scales-for-huntingtons-disease-motor-cognitive-functional-ability-and-quality-of-life-domains/