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). Source: http://www.huntington-study-group.org/UHDRS.html 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: http://persoweb.francenet.fr/~mondor/uhdrs.htm