Unified Huntington's disease rating scale: Reliability and consistency

TLDR

The Unified Huntington’s disease Rating Scale (UHDRS) was developed to assess motor, cognitive, behavioral, and functional domains in Huntington’s disease. The study aimed to evaluate the internal consistency, interdomain correlations, and longitudinal changes in UHDRS ratings. The authors examined internal consistency and interdomain correlations of the four UHDRS domains, tracked rating changes over time, and performed an interrater reliability study of the motor assessment. The UHDRS demonstrated high internal consistency within domains, significant intercorrelations except for the behavioral score, excellent motor interrater reliability, and preliminary evidence that it can track clinical changes over time, supporting its use in repeated clinical studies.

Abstract

Abstract The Unified Huntington's disease Rating Scale (UHDRS) was developed as a clinical rating scale to assess four domains of clinical performance and capacity in HD: motor function, cognitive function, behavioral abnormalities, and functional capacity. We assessed the internal consistency and the intercorrelations for the four domains and examined changes in ratings over time. We also performed an interrater reliability study of the motor assessment. We found there was a high degree of internal consistency within each of the domains of the UHDRS and that there were significant intercorrelations between the domains of the UHDRS, with the exception of the total behavioral score. There was an excellent degree of interrater reliability for the motor scores. Our limited longitudinal database indicates that the UHDRS may be useful for tracking changes in the clinical features of HD over time. The UHDRS assesses relevant clinical features of HD and appears to be appropriate for repeated administration during clinical studies.