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. 2011 Apr;26(5):877-84.
doi: 10.1002/mds.23574. Epub 2011 Mar 2.

An Item Response Analysis of the Motor and Behavioral Subscales of the Unified Huntington's Disease Rating Scale in Huntington Disease Gene Expansion Carriers

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Free PMC article

An Item Response Analysis of the Motor and Behavioral Subscales of the Unified Huntington's Disease Rating Scale in Huntington Disease Gene Expansion Carriers

Anthony L Vaccarino et al. Mov Disord. .
Free PMC article

Abstract

Although the Unified Huntington's Disease Rating Scale (UHDRS) is widely used in the assessment of Huntington disease (HD), the ability of individual items to discriminate individual differences in motor or behavioral manifestations has not been extensively studied in HD gene expansion carriers without a motor-defined clinical diagnosis (ie, prodromal-HD or prHD). To elucidate the relationship between scores on individual motor and behavioral UHDRS items and total score for each subscale, a nonparametric item response analysis was performed on retrospective data from 2 multicenter longitudinal studies. Motor and behavioral assessments were supplied for 737 prHD individuals with data from 2114 visits (PREDICT-HD) and 686 HD individuals with data from 1482 visits (REGISTRY). Option characteristic curves were generated for UHDRS subscale items in relation to their subscale score. In prHD, overall severity of motor signs was low, and participants had scores of 2 or above on very few items. In HD, motor items that assessed ocular pursuit, saccade initiation, finger tapping, tandem walking, and to a lesser extent, saccade velocity, dysarthria, tongue protrusion, pronation/supination, Luria, bradykinesia, choreas, gait, and balance on the retropulsion test were found to discriminate individual differences across a broad range of motor severity. In prHD, depressed mood, anxiety, and irritable behavior demonstrated good discriminative properties. In HD, depressed mood demonstrated a good relationship with the overall behavioral score. These data suggest that at least some UHDRS items appear to have utility across a broad range of severity, although many items demonstrate problematic features.

Conflict of interest statement

Potential conflict of interest: Nothing to Report

Figures

FIGURE 1
FIGURE 1
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 1
FIGURE 1
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 1
FIGURE 1
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 1
FIGURE 1
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 1
FIGURE 1
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 1
FIGURE 1
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 1
FIGURE 1
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 1
FIGURE 1
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 1
FIGURE 1
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 1
FIGURE 1
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 1
FIGURE 1
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 1
FIGURE 1
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 1
FIGURE 1
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 1
FIGURE 1
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 1
FIGURE 1
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 1
FIGURE 1
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 2
FIGURE 2
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 2
FIGURE 2
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 2
FIGURE 2
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 2
FIGURE 2
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 2
FIGURE 2
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 2
FIGURE 2
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 2
FIGURE 2
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 2
FIGURE 2
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 2
FIGURE 2
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 2
FIGURE 2
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 2
FIGURE 2
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 2
FIGURE 2
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 2
FIGURE 2
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 2
FIGURE 2
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 2
FIGURE 2
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 2
FIGURE 2
OCCs for representative UHDRS motor items in relation to UHDRS Motor Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 3
FIGURE 3
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 3
FIGURE 3
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 3
FIGURE 3
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 3
FIGURE 3
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 3
FIGURE 3
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 3
FIGURE 3
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 3
FIGURE 3
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 3
FIGURE 3
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 3
FIGURE 3
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 3
FIGURE 3
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 3
FIGURE 3
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in prHD participants. The remaining OCCs are available as supplemental material online.
FIGURE 4
FIGURE 4
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 4
FIGURE 4
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 4
FIGURE 4
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 4
FIGURE 4
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 4
FIGURE 4
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 4
FIGURE 4
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 4
FIGURE 4
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 4
FIGURE 4
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 4
FIGURE 4
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 4
FIGURE 4
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.
FIGURE 4
FIGURE 4
OCCs for representative UHDRS behavioral items in relation to UHDRS Behavioral Subscale Score in HD participants. The remaining OCCs are available as supplemental material online.

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