Interrater agreement in the assessment of motor manifestations of Huntington's disease

Penelope Hogarth, Elise Kayson, Karl Kieburtz, Karen Marder, David Oakes, Diana Rosas, Ira Shoulson, Nancy S. Wexler, Anne B. Young, Hongwei Zhao, Charles Adler, Roger Albin, Tetsuo Ashizawa, Thomas Bird, Karen Blindauer, Scott Bundlie, James Caress, John Caviness, Sylvain Chouinard, Cynthia ComellaPeter Como, Jody Corey-Bloom, Timothy Counihan, Wallace Deckel, Richard Dubinsky, James Duffy, Leon Dure, Stewart Factor, Andrew Feigin, Hubert Fernandez, Joseph Friedman, Timothy Greenamyre, Mark Guttman, Robert Hauser, Steven Hersch, Bonnie Hersh, Susan Hickenbottom, Donald Higgins, Douglas Hobson, George Jackson, Joseph Jankovic, Danna Jennings, William Johnson, William Koller, Elan Louis, Anne Louis Lafontaine, Carl Leventhal, William Mallonee, Karen Marder, Kenneth Marek, Wayne Martin, Marc Mentis, Eric Molho, Martha Nance, Christopher O'Brien, Constance Orme, David Palmer, Jane Paulsen, George Paulson, Alan Percy, Susan Perlman, Joel Perlmutter, Gerald Podskalny, Kimberly Quiad, Brad Racette, Juan Rachez-Ramos, Lynn Raymond, David Richman, Ted Roberts, Robert Rodnitzky, Christopher Ross, Guy Rouleau, Allen Rubin, Daniel Sax, Neil Schimke, Kathleen Shannon, Paul Shelton, Aileen Shinaman, Leslie Shinobu, Cliff Shults, Eric Siemers, Oksana Suchowersky, Francis Walker, William Weiner, Vicki Wheelock, Joanne Wojcieszek, Frederick Wooten, Ernesto Bonilla, Jang Ho Cha, Margot de Young, Kenneth Fischbeck, Amerigo Negrette, John B. Penney, Maria Ramos, Robert Snodgrass, Leslie Thompson

Research output: Contribution to journalArticlepeer-review

54 Scopus citations


With prospects improving for experimental therapeutics aimed at postponing the onset of illness in preclinical carriers of the Huntington's disease (HD) gene, we assessed agreement among experienced clinicians with respect to the motor manifestations of HD, a relevant outcome measure for preventive trials in this population. Seventy-five clinicians experienced in the evaluation of patients with early HD and six non-clinicians were shown a videotape compiled from the film archives of the United States-Venezuela Collaborative HD Research Project. Observers were asked to rate a 2-3-minute segment of the motor examination for each of 17 at-risk subjects. The rating scale ranged from 0 (normal) to 4 (unequivocal extrapyramidal movement disorder characteristic of HD). As measured by a weighted κ statistic, there was substantial agreement among the 75 clinicians in the judgment of unequivocal motor abnormalities comparing scale ratings of 4 with ratings that were not 4 (weighted κ = 0.67; standard error (SE) = 0.09). Agreement among the non-clinicians was only fair (weighted κ = 0.28; SE = 0.10). Even under the artificial conditions of a videotape study, experienced clinicians show substantial agreement about the signs that constitute the motor manifestations of illness in subjects at risk for HD. We expect these findings to translate to a similar level of interobserver agreement in the clinical trial setting involving experienced investigators examining live patients.

Original languageEnglish (US)
Pages (from-to)293-297
Number of pages5
JournalMovement Disorders
Issue number3
StatePublished - Mar 2005


  • Clinical trials
  • Disease progression
  • Huntington's disease

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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