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Unified Huntington's Disease Rating Scale (UHDRS)
Availability
Please visit this website for more information about the instrument: Huntington Study Group website.
Classification
Core: Huntington's Disease (HD): Motor Assessment, Total Functional Capacity, Functional Assessment Checklist, and Independence Scale
Short Description of Instrument
The Unified Huntington's Disease Rating Scale (UHDRS) is a research tool which has been developed by the Huntington Study Group to provide a uniform assessment of
the clinical features and course of HD.
This Scale Consists of 6 Subtests:
Motor Assessment
.
Cognitive Assessment.
Behavioral Assessment
.
The Independence Scale.
Functional Assessment
.
Total Functional Capacity.
UHDRS 99:
The UHDRS was updated in 1999 and represented improved effectiveness and applicability.
*The Symbol Digit Modalities Test (SDMT) and The Stroop Test are also included in the UHDRS but must be acquired separately*.
Scoring
Each question is scored individually. For the Cognitive component, the following tests are scored: Verbal Fluency, Symbol Digit Modality, Stroop Interference Test.
The modified motor score consists of the sum score of items 4-10 and 13-15 of the UHDRS motor assessments.
References
Marder, K., et al., Rate of functional decline in Huntington's disease. Huntington Study Group. Neurology, 2000. 54(2): p. 452-8.
 
Paulsen, J. S., Wang, C., Duff, K., Barker, R., Nance, M., Beglinger, L. et al. (2010). Challenges assessing clinical endpoints in early Huntington disease. Mov Disord, 25(15), 2595-2603.
 
Beglinger, L. J., O'Rourke, J. J., Wang, C., Langbehn, D. R., Duff, K., Paulsen, J. S., et al. (2010). Earliest functional declines in Huntington disease. Psychiatry Res, 178(2), 414- 418.
 
Tabrizi SJ, Scahill RI, Durr A, et al. Biological and clinical changes in pre-manifest and early stage Huntington's disease in the TRACK-HD study: the 12-month longitudinal analysis. Lancet Neurol. Jan 2011;10 (1):31-42.

 

Document last updated March 2021