The unified huntington's disease rating scale for advanced patients: Validation and follow‐up study

Youssov, Katia; Dolbeau, Guillaume; Maison, Patrick; Boissé, Marie-Françoise; Langavant, Laurent Cléret de; Roos, Raymund A.C.; Bachoud‐Lévi, Anne‐Catherine

doi:10.1002/mds.25678

Cited by 13 publications

(2 citation statements)

References 18 publications

(16 reference statements)

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“…Clinical examination of the oculomotor items of the UHDRS has its limitations due to difficulty in distinguishing between subtle possible pathology and normal eye movements, and due to interrater reliability. Previous studies have shown that the interrater reliability of the total UHDRS motor score is high, however they did not report the interrater reliability of the UHDRS oculomotor domain [ 3 , 19 ]. Additionally, eye movement abnormalities can have a nonspecific nature as well.…”

Section: Discussionmentioning

confidence: 99%

Premanifest Huntington’s disease: Examination of oculomotor abnormalities in clinical practice

Winder¹,

Roos²

2018

PLoS ONE

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IntroductionDifferent oculomotor abnormalities have been reported to occur in premanifest Huntington’s disease. The aim of this study is to investigate which oculomotor items of the Unified Huntington’s Disease Rating Scale (UHDRS) are affected in premanifest individuals compared to healthy controls, and if CAG repeat length and age are correlated with oculomotor abnormalities in premanifest Huntington’s disease gene carriers.MethodsWe compared baseline data of 70 premanifest individuals and 27 controls who participated in the Enroll-HD study at the Leiden University Medical Center, the Netherlands. Premanifest gene carriers were divided in individuals near to disease onset and individuals far from disease onset.ResultsUsing a logistic regression model, only horizontal ocular pursuit of the six oculomotor items of the UHDRS was significantly more frequently affected in premanifest individuals close to disease onset compared to controls (p = 0.044, OR 13.100). Age was significantly higher in premanifest individuals with affected horizontal ocular pursuit (p = 0.016, OR 1.115) and with affected vertical ocular pursuit (p = 0.030, OR 1.065) compared to premanifest individuals without ocular pursuit deficits.ConclusionsOur results suggest that horizontal ocular pursuit is the only affected oculomotor item of the UHDRS in premanifest individuals and could be used to assess early clinical signs of Huntington’s disease. Saccade initiation and saccade velocity do not seem useful for detecting differences between premanifest individuals and controls.

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Section: Discussionmentioning

confidence: 99%

Premanifest Huntington’s disease: Examination of oculomotor abnormalities in clinical practice

Winder¹,

Roos²

2018

PLoS ONE

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“…However, widely used HD rating scales e.g. UHDRS and UHDRS-FAP, and current treatment guidelines, do not address SD at all [8][9][10][11].…”

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confidence: 99%

Sexual dysfunction in Huntington’s Disease: what do we really know?

Tipton

2020

Neurol Neurochir Pol.

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Introduction. In this edition, Szymuś et al. conducted a systematic review revealing sexual dysfunction to be more prevalent in patients with Huntington's Disease compared to controls. Clinical reflections. Sexual dysfunction in HD (SDHD) is common and significantly affects patient quality of life. Commonly used HD rating scales and treatment guidelines do not explicitly address SDHD, and research studies are limited by size and methodology. Clinical implications. It is important that validated sexual dysfunction screening tools be utilised in clinical and research settings.

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

Winder

Roos

Burgunder

et al. 2018

Movement Disord Clin Pract

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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.

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The unified huntington's disease rating scale for advanced patients: Validation and follow‐up study

Cited by 13 publications

References 18 publications

Premanifest Huntington’s disease: Examination of oculomotor abnormalities in clinical practice

Premanifest Huntington’s disease: Examination of oculomotor abnormalities in clinical practice

Sexual dysfunction in Huntington’s Disease: what do we really know?

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

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