Unified huntington’s disease rating scale: Reliability and consistency

Karl Kieburtz, John B. Penney, Peter Corno, Neal Ranen, Ira Shoulson, Andrew Feigin, Davi Abwender, J. Timothy Greenarnyre, Donald Higgins, Frederick J. Marshall, Joshua Goldstein, Kimberly Steinberg, Charles Shih, Irene Richard, Charlyne Hickey, Carol Zimmerman, Constance Orme, Kathy Claude, David Oakes, Daniel S. SaxAnthony Kim, Steven Hersch, Randi Jones, Alexander Auchus, David Olsen, Cheryl Bissey-Black, Allen Rubin, Rose Schwartz, Richard Dubinsky, William Mallonee, Carolyn Gray, Nan Godfrey, Greg Suter, Kathleen M. Shannon, Glenn T. Stebbins, Jean A. Jaglin, Karen Marder, Stuart Taylor, Elan Louis, Carol Moskowitz, Deborah Thorne, Naomi Zubin, Nancy Wexler, Michael R. Swenson, Jane Paulsen, Neal Swerdlow, Roger Albin, Christine Wernette, Francis Walker, Wayne Martin, Huntington Study Group

Research output: Contribution to journalArticle

1314 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)136-142
Number of pages7
JournalNeurology
Volume11
Issue number2
DOIs
StatePublished - Feb 27 2001

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Keywords

  • Behavioral abnormalities
  • Clinical research
  • Cognitive function
  • Functional capacity
  • Huntington’s disease

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Kieburtz, K., Penney, J. B., Corno, P., Ranen, N., Shoulson, I., Feigin, A., Abwender, D., Timothy Greenarnyre, J., Higgins, D., Marshall, F. J., Goldstein, J., Steinberg, K., Shih, C., Richard, I., Hickey, C., Zimmerman, C., Orme, C., Claude, K., Oakes, D., ... Huntington Study Group (2001). Unified huntington’s disease rating scale: Reliability and consistency. Neurology, 11(2), 136-142. https://doi.org/10.1002/mds.870110204