Unified Huntington's Disease Rating Scale: reliability and consistency. Huntington Study Group

Mov Disord. 1996 Mar;11(2):136-42. doi: 10.1002/mds.870110204.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Activities of Daily Living / classification
  • Activities of Daily Living / psychology
  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Child
  • Cohort Studies
  • Female
  • Follow-Up Studies
  • Humans
  • Huntington Disease / diagnosis*
  • Huntington Disease / genetics
  • Huntington Disease / psychology
  • Male
  • Middle Aged
  • Neuropsychological Tests / statistics & numerical data*
  • Observer Variation
  • Prospective Studies
  • Psychometrics
  • Reproducibility of Results