Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2018 Apr 15;5(3):290-295.
doi: 10.1002/mdc3.12618. eCollection 2018 May-Jun.

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

Jessica Y Winder  1 , Raymund A C Roos  1 , Jean-Marc Burgunder  2   3 , Johan Marinus  1 , Ralf Reilmann  4   5   6
Affiliations

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

Jessica Y Winder et al. Mov Disord Clin Pract. .

Abstract

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.

Keywords: Huntington's disease; Unified Huntington's Disease Rating Scale‐Total Motor Score; annual online certification; interrater reliability.

PubMed Disclaimer

References

    1. Ross CA, Pantelyat A, Kogan J, Brandt J. Determinants of functional disability in Huntington's disease: role of cognitive and motor dysfunction. Mov Disord 2014;29:1351–1358. - PMC - PubMed
    1. Huntington's Disease Collaborative Research Group . A novel gene containing a trinucleotide that is expanded and unstable on Huntington's disease chromosomes. Cell 1993;72:971–983. - PubMed
    1. Roos RAC. Huntington's disease: a clinical review. Orphanet J Rare Dis 2010;5:40. - PMC - PubMed
    1. Huntington Study Group . Unified Huntington's Disease Rating Scale: reliability and consistency. Mov Disord 1996;11:136–142. - PubMed
    1. Siesling S, van Vugt JPP, Zwinderman KAH, Kieburtz K, Roos RAC. Unified Huntington's Disease Rating Scale: a follow up. Mov Disord 1998;13:915–919. - PubMed

LinkOut - more resources