Unified Huntington's disease rating scale for advanced patients: Validation and follow‐up study

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Background The clinical assessment of motor symptoms in Huntington's disease is usually performed with the Unified Huntington's Disease Rating Scale‐Total Motor Score (UHDRS‐TMS). A high interrater reliability is desirable to monitor symptom progression. Therefore, a teaching video and a system for annual online certification has been developed and implemented. Objectives The aim of this study is to investigate the interrater reliability of the UHDRS‐TMS and of its subitems, and to examine the performance of raters in consecutive years. Methods Data from the online UHDRS‐TMS certification were used. The interrater reliability was assessed for all first‐time participants (n = 944) between 2009 and 2016. Intraclass correlation coefficients (ICC) were calculated for each year separately and the mean was taken as the total ICC. Results The UHDRS‐TMS (ICC = 0.847), tandem walking (0.824), pronate/supinate hands left (0.713), and retropulsion pull test (0.706) showed good interrater reliability. Poor interrater reliability was found for maximal dystonia of the left and right upper extremity (0.187 and 0.322, respectively), maximal dystonia of the left and right lower extremity (0.200 and 0.256, respectively), and maximal dystonia of the trunk (0.389), tongue protrusion (0.266), and rigidity arms left (0.390). Raters performed significantly worse on follow‐up certification compared to their first certification. Conclusions Our results suggest that the rating of dystonia (absent, slight, mild, moderate, or marked) is subjective and difficult to interpret, especially on video. Therefore, changing the dystonia items of the UHDRS‐TMS should be explored. We also recommend that raters should watch the UHDRS‐TMS teaching video before each certification.

Section: Discussioncontrasting

confidence: 62%

Interrater Reliability of the Unified Huntington's Disease Rating Scale‐Total Motor Score Certification

Winder

Roos

Burgunder

et al. 2018

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“…In the same study, the interclass correlation coefficient was lower for the chorea (0.82) and dystonia (0.62) subscores . As expected, the UHDRS‐TMS is negatively correlated with the UHDRS‐Total Functional Capacity scale, as well as with other UHDRS functional scales, and with cognitive scales . Extensive data from multiple observational studies and clinical trials suggest that the UHDRS‐TMS is sensitive to change over time, but there has been no formal clinimetric assessment.…”

Section: Resultssupporting

confidence: 81%

Rating Scales for Motor Symptoms and Signs in Huntington's Disease: Critique and Recommendations

Mestre

Forjaz

Mahlknecht

et al. 2018

Motor symptoms are a major feature of Huntington's disease (HD). The International Parkinson and Movement Disorder Society (MDS) commissioned the assessment of the clinimetric properties of motor rating scales in HD to make recommendations regarding their use, following previously established standardized criteria. After a systematic literature search, a total of 6 rating scales assessing motor symptoms and signs in HD were included for review. Performance testing (reviewed elsewhere) and quantitative motor rating methods were excluded. Only the Unified Huntington's Disease Rating Scale‐Total Motor Score (UHDRS‐TMS) was classified as “recommended” for assessing the severity of motor signs in HD. The following scales were classified as “suggested”: Abnormal Involuntary Movement Scale, the UHDRS‐TMS4, the Quantified Neurological Examination, and the Marsden and Quinn Chorea Severity Scale. The committee also concluded that further assessment of existing rating scales, including the UHDRS‐TMS, is necessary to determine sensitivity to change and to screening for the presence of motor signs specific to HD. There is also a need to develop a motor rating scale to be used in positive gene carriers with subtle but not definite motor signs.

“…There are no available data on test–retest reliability or inter‐rater reliability. Good convergent validity with other components of the UHDRS has been shown, as well as with motor disability, cognitive, and behavioral deficits . The UHDRS‐FAS has been shown to be sensitive to change over time in several studies …”

Section: Rating Scalesmentioning

confidence: 90%

“…The clinimetric data available show that the UHDRS‐IS has moderate inter‐rater reliability but in a modified version that compares caregiver report with patient self‐report (ICC = 0.71, 95% CI: 0.48, 0.85) . Good correlation with other components of the UHDRS, as well as motor disability, cognitive and behavioral deficits has been shown in various studies . Data from clinical trials suggest sensitivity of the UHDRS‐IS to change over time and across disease stages …”

Section: Rating Scalesmentioning

confidence: 99%

Rating Scales and Performance‐based Measures for Assessment of Functional Ability in Huntington's Disease: Critique and Recommendations

Mestre

Busse

Davis

et al. 2018

Limitation of functional ability is a major feature of Huntington's disease (HD). The International Parkinson and Movement Disorder Society (MDS) commissioned the appraisal of the use and clinimetric properties of clinical measures of functional ability that have been applied in HD studies and trials to date, to make recommendations regarding their use based on standardized criteria. After a systematic literature search, we included a total of 29 clinical measures grouped into two categories: (1) performance‐based measures (e.g., balance, walking, and reaching/grasping), and (2) rating scales. Three performance‐based measures are rated as “recommended”: the Tinetti Mobility Test for screening of fall risk and for severity assessment of mobility in patients with manifest HD (up to stage III); the Berg Balance Scale for severity of balance impairment; and the Six‐Minute Walk Test for assessment of walking endurance (severity) in HD subjects with preserved ambulation. No rating scale targeting functional ability reached a “recommended” status either for screening or severity measurement. The main challenges identified in this review include applying widely accepted conceptual frameworks to the identified measures, the lack of validation of clinical measures to detect change over time, and absence of validated measures for upper limb function. Furthermore, measures of capacity or ability to perform activities of daily living had ceiling effects in people with early and pre‐manifest HD. We recommend that the MDS prioritize the development of new scales that capture small, but meaningful changes in function over time for outcome assessment in clinical trials, particularly in earlier stages of HD.