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Unified Huntington's Disease Rating Scale (UHDRS)
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UNIFIED HUNTINGTON'S DISEASE RATING SCALE (UHDRS) and DATABASE 
 
The UHDRS is a research tool which has been developed by the HSG to
provide a uniform assessment of the clinical features and course of HD. The UHDRS has undergone extensive reliability and validity testing and has been used as a major outcome measure by the HSG in controlled clinical trials.
 

UHDRS '99

UHDRS '99 represents an update and expansion of the UHDRS to enhance the utility and applicability of this research tool. The revised UHDRS '99 and accompanying manual are the copywritten intellectual property of the HSG.

 

UHDRS Natural History Database

The current UHDRS database numbers more than 4,000 individuals affected by or immediately at risk for HD. A cross-sectional and prospective analysis of the database of the UHDRS instrument was published in Movement Disorders (vol. 11:136-142,1996). In a large cohort using the UHDRS natural history database, the rate of functional decline was evaluated over a maximum of 4 years of observation. A full report was published in Neurology (54:452-458, 2000).

Unified Huntington's Disease
Rating Scale (UHDRS)
Motor Section

This is a rating system to quantify the severity of Huntington's Disease.
It is divided into multiple subsections:motor, cognitive, behavioral,
functional. These scores can be calculated by summing the various
questions of each section. Some sections (such as chorea and dystonia)
require grading each extremity, face, bucco-oral-ligual, and trunk
separately. Eye movements require both horizontal and vertical grades.
  • Ocular Pursuit (horizontal)
    0-complete
    1-jerky
    2-interrupted/full range
    3-incomplete range
    4-cannot pursue

  • Ocular Pursuit (vertical)
    0-complete
    1-jerky
    2-interrupted/full range
    3-incomplete range
    4-cannot pursue

  • Saccade Initiation (horizontal)
    0-normal
    1-increased latency
    2-suppressible blinks/head movements to initiate
    3-unsuppressible head movements
    4-cannot initiate

  • Saccade Initiation (vertical)
    0-normal
    1-increased latency
    2-suppressible blinks/head movements to initiate
    3-unsuppressible head movements
    4-cannot initiate

  • Saccade Velocity (horizontal)
    0-normal
    1-mild slowing
    2-moderate slowing
    3-severely slow, full range
    4-incomplete range

  • Saccade Velocity (vertical)
    0-normal
    1-mild slowing
    2-moderate slowing
    3-severely slow, full range
    4-incomplete range

  • Dysarthria
    0-normal
    1-unclear, no need to repeat
    2-must repeat
    3-mostly incomprehensible
    4-mute

  • Tongue Protrusion
    0-normal
    1-<10 seconds
    2-<5 seconds
    3-cannot fully protrude
    4-cannot beyond lips

  • Finger Taps (right)
    0-normal (15/5sec)
    1-mild slowing or reduction in amp.
    2-moderately impaired. may have occasional arrests (7- 10/15sec)
    3-severely impaired. Frequent hesitations and arrests
    4-can barely perform

  • Finger Taps (left)
    0-normal (15/5sec)
    1-mild slowing or reduction in amp.
    2-moderately impaired. may have occasional arrests (7- 10/15sec)
    3-severely impaired. Frequent hesitations and arrests
    4-can barely perform

  • Pronate/Supinate (right)
    0-normal
    1-mild slowing/irregular
    2-moderate slowing and irregular
    3-severe slowing and irregular
    4-cannot perform

  • Pronate/Supinate (left)
    0-normal
    1-mild slowing/irregular
    2-moderate slowing and irregular
    3-severe slowing and irregular
    4-cannot perform

  • Fist-Hand-Palm Sequence
    0->4 in 10 seconds without cues
    1-<4 in 10 sec. without cues
    2->4 in 10 sec. with cues
    3-<4 in 10 sec. with cues
    4-cannot perform

  • Rigidity-arms (right)
    0-absent
    1-slight or only with activation
    2-mild/moderate
    3-severe, full range of motion
    4-severe with limited range

  • Rigidity-arms (left)
    0-absent
    1-slight or only with activation
    2-mild/moderate
    3-severe, full range of motion
    4-severe with limited range

  • Bradykinesia
    0-normal
    1-minimally slow
    2-mildly but clearly slow
    3-moderately slow
    4-marked slowing, long delays in initiation

  • Maximal Dystonia(trunk)
    0-absent
    1-slight/intermittent
    2-mild/common or moderate/intermittent
    3-moderate/common
    4-marked/prolonged

  • Maximal Dystonia(RUE)
    0-absent
    1-slight/intermittent
    2-mild/common or moderate/intermittent
    3-moderate/common
    4-marked/prolonged

  • Maximal Dystonia(LUE)
    0-absent
    1-slight/intermittent
    2-mild/common or moderate/intermittent
    3-moderate/common
    4-marked/prolonged

  • Maximal Dystonia(RLE)
    0-absent
    1-slight/intermittent
    2-mild/common or moderate/intermittent
    3-moderate/common
    4-marked/prolonged

  • Maximal Dystonia(LLE)
    0-absent
    1-slight/intermittent
    2-mild/common or moderate/intermittent
    3-moderate/common
    4-marked/prolonged

  • Maximal Chorea (Face)
    0-absent
    1-slight/intermittent
    2-mild/common or moderate/intermittent
    3-moderate/common
    4-marked/prolonged

  • Maximal Chorea (BOL)
    0-absent
    1-slight/intermittent
    2-mild/common or moderate/intermittent
    3-moderate/common
    4-marked/prolonged

  • Maximal Chorea (Trunk)
    0-absent
    1-slight/intermittent
    2-mild/common or moderate/intermittent
    3-moderate/common
    4-marked/prolonged

  • Maximal Chorea (RUE)
    0-absent
    1-slight/intermittent
    2-mild/common or moderate/intermittent
    3-moderate/common
    4-marked/prolonged

  • Maximal Chorea (LUE)
    0-absent
    1-slight/intermittent
    2-mild/common or moderate/intermittent
    3-moderate/common
    4-marked/prolonged

  • Maximal Chorea (LLE)
    0-absent
    1-slight/intermittent
    2-mild/common or moderate/intermittent
    3-moderate/common
    4-marked/prolonged

  • Maximal Chorea (RLE)
    0-absent
    1-slight/intermittent
    2-mild/common or moderate/intermittent
    3-moderate/common
    4-marked/prolonged

  • Gait
    0-normal narrow base
    1-wide base, and/or slow
    2-wide base, walks with difficulty
    3-walks with assistance
    4-cannot attempt

  • Tandem Walking
    0-normal for 10 steps
    1-1-3 deviations
    2->3 deviations
    3-cannot complete
    4-cannot attempt

    Source: This was the source when I first obtained this scale.  It no longer works:  http://persoweb.francenet.fr/~mondor/uhdrs.htm