Unified Huntington's disease rating scale: Reliability and consistency

  title={Unified Huntington's disease rating scale: Reliability and consistency},
  author={Karl D. Kieburtz and John B. Penney and Peter Corno and Neal G. Ranen and Ira Shoulson and Andrew S. Feigin and David A. Abwender and J. Timothy Greenarnyre and Donald S. Higgins and Frederick J. Marshall and Joshua L. Goldstein and Kimberly Steinberg and Charles Shih and Irene Hegeman Richard and Charlyne Hickey and Carol Zimmerman and Constance Orme and Kathy Claude and David Oakes and DanielS. Sax and A Kim and Steven M. Hersch and Randi Jones and Alexander P. Auchus and David Ben{\'e}e Olsen and Cheryl Bissey-Black and Allen Rubin and Rose Rossi Schwartz and Richard Dubinsky and William M. Mallonee and Carolyn Gray and N E Godfrey and Gr Suter and Kathleen M. Shannon and Glenn T. Stebbins and Jean A. Jaglin and Karen Marder and Stuart Taylor and Elan D. Louis and Carol Brown Moskowitz and Deborah Thorne and Naomi Zubin and Nancy S Wexler and Michael R. Swenson and Jane S. Paulsen and Neal R. Swerdlow and Roger L. Albin and Christine Wernette and Francis O. Walker and Victoria Hunt},
  journal={Movement Disorders},
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… 
Unified Huntington's disease rating scale for advanced patients: Validation and follow‐up study
The U HDRS‐For Advanced Patients (UHDRS‐FAP), designed in order to improve longitudinal assessment of patients at advanced disease stage, is reliable and more sensitive to change than the original UHDRS for cognitive and motor domains.
The unified huntington's disease rating scale for advanced patients: Validation and follow‐up study
The results indicate that the U HDRS‐For Advanced Patients (UHDRS‐FAP) is reliable and more sensitive to change than the original UHDRS for cognitive and motor domains.
Rating Scales for Motor Symptoms and Signs in Huntington's Disease: Critique and Recommendations
Further assessment of existing rating scales, including the UHDRS‐TMS, is necessary to determine sensitivity to change and to screening for the presence of motor signs specific to HD and there is also a need to develop a motor rating scale to be used in positive gene carriers with subtle but not definite motor signs.
Predictors of neuropathological severity in 100 patients with Huntington's disease
Motor impairment appears to be a good clinical measure of neuronal cell loss, at least late in the course of HD and therefore may prove useful in observational and treatment studies.
Rate of change in early Huntington's disease: A clinicometric analysis
It may be possible to select sensitive items to create a simplified version of the UHDRS, which would be more efficient and more sensitive for the assessment of disease progression in clinical trials and natural history studies.
Rating scales for cognition in Huntington's disease: Critique and recommendations
There is a need to further validate currently available cognitive scales in Huntington's disease, but that it is premature to recommend the development of new scales.
Utility of the Parkinson’s disease-Cognitive Rating Scale for the screening of global cognitive status in Huntington’s disease
The PD-CRS is a valid and reliable instrument to assess global cognition in HD in routine clinical care and clinical trials and failed to show robust psychometric properties in this context.
Cognitive deficits in Huntington's disease on the Repeatable Battery for the Assessment of Neuropsychological Status
The current study supports the clinical applicability of the RBANS in patients with HD, with results significantly related to other markers of HD, including motor abnormalities, functional abilities, and other cognitive scores.
Motor outcome measures in Huntington disease clinical trials.


Factors associated with slow progression in Huntington's disease.
Men tended to have a slower disease progression than did women, and this was particularly evident among men inheriting Huntington's disease from affected mothers, and neither the butyrophenone haloperidol nor the tricyclic antidepressant imipramine were related to rate of progression.
Huntington's disease in Venezuela
The rate of decline of these untreated patients from Venezuela was similar to that seen in US patients who had received neuroleptic drugs and Juvenile patients declined nearly twice as fast as adult-onset patients.
Assessment of extrapyramidal disorders.
In the last twenty years the accurate evaluation of extrapyramidal disorders has become essential to assess the efficacy of an increasing number of potentially useful drugs. Not surprisingly, since