UNIFIED HUNTINGTONS-DISEASE RATING-SCALE - RELIABILITY AND CONSISTENCY

The Unified Huntington's Disease Rating Scale (UHDRS) was developed as a clinical rating scale to assess four domains of clinical performanc e and capacity in HD: motor function, cognitive function, behavioral a bnormalities, and functional capacity. We assessed the internal consis tency and the intercorrelations for the four domains and examined chan ges in ratings over time. We also performed an interrater reliability study of the motor assessment. We found there was a high degree of int ernal consistency within each of the domains of the UHDRS and that the re were significant intercorrelations between 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 ti me. The UHDRS assesses relevant clinical features of HD and appears to be appropriate for repeated administration during clinical studies.