a.e.g.

(1999)

Unified Huntington’s Disease Rating Scale (UHDRS)

(AAN, 1995)
Nombre: Varón [ ] Mujer [ ]
Fecha: F. nacimiento: Edad:
Estudios/Profesión: N. Hª: Lateralidad:
Observaciones:

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) 4-mute

0-complete
1-jerky * Tongue Protrusion
2-interrupted/full range 0-normal
3-incomplete range 1-<10 seconds
4-cannot pursue 2-<5 seconds
3-cannot fully protrude
* Ocular Pursuit (vertical) 4-cannot beyond lips
0-complete
1-jerky * Finger Taps (right)
2-interrupted/full range 0-normal (15/5sec)
3-incomplete range 1-mild slowing or reduction in amp.
4-cannot pursue 2-moderately impaired. may have occasional
arrests (7- 10/15sec)
* Saccade Initiation (horizontal) 3-severely impaired. Frequent hesitations and
0-normal arrests
1-increased latency 4-can barely perform
2-suppressible blinks/head movements to
initiate * Finger Taps (left)
3-unsuppressible head movements 0-normal (15/5sec)
4-cannot initiate 1-mild slowing or reduction in amp.
2-moderately impaired. may have occasional
* Saccade Initiation (vertical) arrests (7- 10/15sec)
0-normal 3-severely impaired. Frequent hesitations and
1-increased latency arrests
2-suppressible blinks/head movements to 4-can barely perform
initiate
3-unsuppressible head movements * Pronate/Supinate (right)
4-cannot initiate 0-normal
1-mild slowing/irregular
* Saccade Velocity (horizontal) 2-moderate slowing and irregular
0-normal 3-severe slowing and irregular
1-mild slowing 4-cannot perform
2-moderate slowing
3-severely slow, full range * Pronate/Supinate (left)
4-incomplete range 0-normal
1-mild slowing/irregular
* Saccade Velocity (vertical) 2-moderate slowing and irregular
0-normal 3-severe slowing and irregular
1-mild slowing 4-cannot perform
2-moderate slowing
3-severely slow, full range * Fist-Hand-Palm Sequence
4-incomplete range 0->4 in 10 seconds without cues
1-<4 in 10 sec. without cues
* Dysarthria 2->4 in 10 sec. with cues
0-normal 3-<4 in 10 sec. with cues
1-unclear, no need to repeat 4-cannot perform
2-must repeat * Rigidity-arms (right)
3-mostly incomprehensible 0-absent
 1-slight or only with activation * Maximal Chorea (Trunk)
2-mild/moderate 0-absent
3-severe, full range of motion 1-slight/intermittent
4-severe with limited range 2-mild/common or moderate/intermittent
3-moderate/common
* Rigidity-arms (left) 4-marked/prolonged
0-absent
1-slight or only with activation * Maximal Chorea (RUE)
2-mild/moderate 0-absent
3-severe, full range of motion 1-slight/intermittent
4-severe with limited range 2-mild/common or moderate/intermittent
3-moderate/common
* Bradykinesia 4-marked/prolonged
0-normal
1-minimally slow * Maximal Chorea (LUE)
2-mildly but clearly slow 0-absent
3-moderately slow 1-slight/intermittent
4-marked slowing, long delays in initiation 2-mild/common or moderate/intermittent
3-moderate/common
* Maximal Dystonia(trunk) 4-marked/prolonged
0-absent
1-slight/intermittent * Maximal Chorea (LLE)
2-mild/common or moderate/intermittent 0-absent
3-moderate/common 1-slight/intermittent
4-marked/prolonged 2-mild/common or moderate/intermittent
3-moderate/common
* Maximal Dystonia(RUE) 4-marked/prolonged
0-absent
1-slight/intermittent * Maximal Chorea (RLE)
2-mild/common or moderate/intermittent 0-absent
3-moderate/common 1-slight/intermittent
4-marked/prolonged 2-mild/common or moderate/intermittent
3-moderate/common
* Maximal Dystonia(LUE) 4-marked/prolonged
0-absent
1-slight/intermittent * Gait
2-mild/common or moderate/intermittent 0-normal narrow base
3-moderate/common 1-wide base, and/or slow
4-marked/prolonged 2-wide base, walks with difficulty
3-walks with assistance
* Maximal Dystonia(RLE) 4-cannot attempt
0-absent
1-slight/intermittent * Tandem Walking
2-mild/common or moderate/intermittent 0-normal for 10 steps
3-moderate/common 1-1-3 deviations
4-marked/prolonged 2->3 deviations
3-cannot complete
* Maximal Dystonia(LLE) 4-cannot attempt
0-absent
1-slight/intermittent * Retropulsion
2-mild/common or moderate/intermittent 0-normal
3-moderate/common 1-recovers spontaneously
4-marked/prolonged 2-would fall if not caught
3-falls spontaneously
* Maximal Chorea (Face) 4-cannot stand
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