Introduction Postural instability is a major characteristic of Huntington’s disease (HD) causing considerable disability in many patients. Valid and reliable instruments measuring balance in HD patients are crucial in order to perform high quality research and clinical care regarding HD-related postural instability. However, little is known about the measurement properties of instruments that assess this phenomenon in HD patients.

Aims To critically appraise and compare the measurement properties of assessment tools that measure postural stability in patients with HD.

Methods A systematic review of the literature in PubMed, Embase, Web of Science and CINAHL was performed. The COSMIN-checklist was applied to evaluate the methodological quality of studies found after this literature search. Only full text, original articles, published in English, French, German or Dutch until January 2014 were included. These articles had to discuss the development or evaluation of measurement properties of clinimetric, posturographic or neurophysiological instruments that assess postural balance in manifest HD patients. The presentation of the outcome of analyses was mandatory.

Results Criteria were met by 11 studies evaluating 23 assessment tools. The number of evaluated measurement properties varied from one to three per article. The methodological quality was rated as either poor or fair. After combining different studies most evaluated measurement properties were found for the Berg Balance Scale, including reliability, measurement error, content validity, structural validity and responsiveness. Four of them were rated as poor.

Conclusions Instruments that assess postural stability in HD patients are currently not sufficiently validated. Future studies are needed to provide better evidence for reliability, validity and responsiveness of these instruments.