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Unified Huntington’s Disease Rating Scale (UHDRS)

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Purpose

A clinical rating scale developed to assess four domains of clinical performance and capacity in Huntington’s Disease (HD)--motor function, cognitive function, behavioral abnormalities, and functional capacity.

Acronym UHDRS

Area of Assessment

Functional Mobility
Cognition
Behavior

Cost

Not Free

Actual Cost

$300.00

Cost Description

• Clinicians of academic or not-for-profit organizations –$300
• Clinicians of for-profit organizations- $1000 plus $5 per use
• Full pricing list http://huntingtonstudygroup.org/uhdrs-pricing-schedule/

Key Descriptions

  • The UHDRS has four parts with separate scoring for each part:

    Part I: motor function. 31 items with 5-point ordinal scale ranging from 0-4 with the highest score indicating inability to perform the motor task.

    Part II: cognitive function. 3 items with higher scores indicating better cognitive performance.
    1. Verbal Fluency Test
    2. Symbol Digit Modalities Test (SDMT)
    3. Stroop Interference Test.

    Part III: behavioral assessment. 10 items with a 5-point ordinal scale ranging from 0-4 with the highest score indicating severe behavioral symptoms; 4 items requiring the evaluator to answer yes/no questions about the overall clinical impression with respect to the participant showing clinical evidence of confusion, dementia, depression and requiring pharmacotherapy (1 point for yes)

    Part IV: functional capacity. This domain is divided into three sections:
    1. Huntington’s Disease Functional Capacity Scale (HDFCS) is reported as the Total Functional Capacity Score (TFC) which has a total of 25 Yes/No questions assessing the total functional capacity of the individual. A score of 1 given to all yes replies.
    2. Independence Scale rated from 10 to 100 with higher scores indicating better functioning than lower scores
    3. Functional Capacity- 5 items with 4-point ordinal scale ranging from 0 to 3 with the highest score indicating higher functional capacity.

  • Summary
     Higher scores on Part I indicate inability to perform motor task.
     Higher scores on Part II indicate better cognitive performance.
     Higher scores on Part III indicate severe behavioral symptoms.
     Higher scores on Independence Scale of Part IV indicate better functioning and higher scores on functional capacity indicate higher functional capacity.

Number of Items

74

Equipment Required

  • Paper and Pen

Time to Administer

30 minutes

Required Training

No Training

Instrument Reviewers

Komal Pande, PT, Post Professional Student at the University of Indianapolis

ICF Domain

Body Function
Activity
Participation

Considerations

  • Despite the extensive number of items, the UHDRS has proven to be beneficial in clinical practice within the Movement Disorder Center (Klempir, et al., 2006).
  • The Motor section of the UHDRS has excellent psychometric properties and can be utilized for quick assessment and follow-up of the motor function in HD (Klempir, et al., 2006).
  • The scale may be particularly beneficial in analyzing clinical changes in a controlled environment, such as a clinical trial utilizing experimental interventions (Siesling et al., 1998).
  • The authors recommend an annual assessment of the Total Motor Score in every individual with HD to evaluate the motor progression of the disease and a complete UHDRS assessment every second year for each patient with HD to assess longitudinal changes in disease progression (Siesling, 1998).

Movement and Gait Disorders

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Normative Data

Huntington’s Disease:

(Keiburtz, K. et al, 1996; n = 489 from 20 places in North America; 229 men, 227 women, 33 unknown gender; 426 were Caucasian, 15 were African American, 48 were of unknown race; 198 participants inherited HD from their mother, 211 inherited from their father, inheritance was unknown in 88; mean age of participants (yrs)=49.6, Range=11-91; Age onset HD(yrs) =41.6 Range= 6-74; Duration HD (yrs)= 8.9 Range=1-35)

 

 

UHDRS

Mean Score (Standard Deviation)

Part I

47.2 (22.0)

Part II

Verbal Fluency: 16.3(10.2)

Symbol Digit: 21.0(10.8)

Stroop Test:

Color: 52.7(21.3)

Word: 38.8(16.5)

Color word: 21.3(10.9)

Part III

11.9 (10.3)

Mood: 6.1(6.3)

Behavior: 4.0(4.2)

Psychosis: 0.4 (1.6)

Obsessive: 1.5(3.1)

 

Part IV

Functional Checklist Score: 15.9(7.2)

Independence Scale: 72.3(19.9)

TFC: 6.6(3.8)

 

Huntington’s Disease:

(Keiburtz, K. et al, 1996; n = 489 from 20 places in North America; 229 men, 227 women, 33 unknown gender; 426 were Caucasian, 15 were African American, 48 were of unknown race; 198 participants inherited HD from their mother, 211 inherited from their father, inheritance was unknown in 88; mean age of participants (yrs)=49.6, Range=11-91; Age onset HD(yrs) =41.6 Range= 6-74; Duration HD (yrs)= 8.9 Range=1-35)

 

 

UHDRS

Mean Score (Standard Deviation)

Part I

47.2 (22.0)

Part II

Verbal Fluency: 16.3(10.2)

Symbol Digit: 21.0(10.8)

Stroop Test:

Color: 52.7(21.3)

Word: 38.8(16.5)

Color word: 21.3(10.9)

Part III

11.9 (10.3)

Mood: 6.1(6.3)

Behavior: 4.0(4.2)

Psychosis: 0.4 (1.6)

Obsessive: 1.5(3.1)

 

Part IV

Functional Checklist Score: 15.9(7.2)

Independence Scale: 72.3(19.9)

TFC: 6.6(3.8)

 

 

 

 

 

 

 

 

 

 

 

 

 

(Siesling, S et al, 1997; n=69, 34 men, 29 patients inherited the disease from their father and 33 from their mother, inheritance unknown in 7; Mean age= 46)

UHDRS

Mean Score (Standard Deviation)

Part I

Total Motor Score: 58 (24)

Part II

Verbal Fluency:15 (13)

Symbol digit: 17(15)

Stroop Color: 32(19)

Part III

Behavioral Assessment: 16 (11)

Part IV

Functional Checklist Score:14(7)

Independence Scale: 63(21)

Total Functional Capacity: 5.7(4.2)

 

 

 

Longitudinal change scores in UHDRS per 6 months:

(Keiburtz, K. et al, 1996)

 

UHDRS

Mean Change Score (Standard Deviation)

Part I

Total Motor Score: 3.2(8.4)

Part II

Verbal Fluency: 0.1(5.3)

Symbol digit: 0.1(4.2)

Stroop Color Word: -1.3(13.1)

Part IV

Functional Checklist: -0.9(3.0)

Independence Scale: -3.8(8.6)

TFC: -0.3(2.1)

TFC when initial score ≥ 3: -0.5(1.6)

 

Longitudinal Assessment at 1-year Follow Up:

(Siesling, S et al, 1998; n=78, 36 men and 42 women; 49 from Netherlands, 29 from Rochester; mean follow-up time= 1.07 years, mean age at onset (yrs)= 37.7; mean age at first evaluation (yrs)= 44.4; mean duration of illness (yrs)= 6.7 yrs)

 

 

 

 

UHDRS

Mean Score (Standard Deviation)n=46

Part I

Total Motor Score (69): -5.97

Total Chorea Score (78): -0.51

Total Dystonia (78): -0.58

Part II

Verbal Fluency (45): 1.24

Symbol Digit (39): 2.35

Stroop Color (43): 2.21

Stroop Word (41): 2.24

Stroop Interference (40): 0.18

Part III

Behavioral Severity (73): 0.73

Behavioral Frequency (73): -1.50

Part IV

Weight (45): -1.20

Independence (75): 3.60

 

 

Spincocerebellar Ataxia (Part IV):

(Schimtz-Hubsch, et al, 2006; Patients in first trial, n= 167; F/M ratio= 72/89; mean age= 49.2 ± 14.2; disease duration=10.8±4.4; Patients in second trial, n=119; F/M ration=61/58; age=50.3±13.2; disease duration= 13.3±8.3)

  • Mean scores (Standard Deviation)
  • Part IV: 16.7 (8.0)

 

 

Test/Retest Reliability

Huntington’s Disease:

(Siesling, et al., 1997)

  • Excellent test-retest reliability in the U.S. population (ICC=0.90)
  • Excellent test-retest reliability in the Dutch population (ICC=0.88)

Interrater/Intrarater Reliability

Huntington’s Disease:

(Keiburtz, et al., 1996)

  • Excellent for total motor score (ICC= .94)
  • Excellent for chorea score (ICC= .82)
  • Good for dystonia score (ICC= .62)

(Youssav, K, et al, 2013; n = 69; 49 from outpatient clinic, 14 from nursing home, 20 institutionalized; Males=25, females=44; Age at inclusion (yrs)=69; Age of onset (yrs)=44; Disease duration (yrs)= 44; Education level= 43)

  • Excellent for total motor score (ICC= .97)
  • Excellent for behavioral score (ICC=. 99)

Internal Consistency

Huntington’s Disease:

(Keiburtz, et al., 1996)

  • Motor Scale: Excellent (Cronbach’s alpha=.95)
  • Cognitive Tests: Excellent (Cronbach’s alpha=.90)
  • Behavioral Scale: Excellent (Cronbach’s alpha=.83)
  • Functional Checklist: Excellent (Cronbach’s alpha=.83)

 (Youssav, et al., 2013)

  • Motor Scale= Excellent (Cronbach’s alpha=.82)
  • Cognitive Scale=Excellent (Cronbach’s alpha=.96)
  • Behavioral Scale=Poor (Cronbach’s alpha= .49)
  • Functional Scale= Poor (Cronbach’s alpha=.53)

(Siesling, et al., 1997)

  • Total Motor Score: Excellent in the U.S population (Cronbach’s alpha= .97)
  • Total Motor Score: Excellent in the Dutch population (Cronbach’s alpha=.96)

Criterion Validity (Predictive/Concurrent)

Concurrent Validity:

Huntington’s Disease

(Schmitz-Hubsch et al, 2006)

  • Excellent correlation with the Scale for Assessment and Rating of Ataxia (SARA) (r= -0.89, p < 0.0001)

Bibliography

Klempír, Jirí, et al. "Unified Huntington's disease rating scale: clinical practice and a critical approach." Functional neurology 21.4 (2006): 217.

Kremer, H. P. H., and Hungtington Study Group. "Unified Huntington's disease rating scale: reliability and consistency." (1996).

Schmitz-Hübsch, T., et al. "Scale for the assessment and rating of ataxia Development of a new clinical scale." Neurology 66.11 (2006): 1717-1720. Find on PubMed

Siesling, S., et al. "A shortened version of the motor section of the Unified Huntington's Disease Rating Scale." Movement Disorders 12.2 (1997): 229-234. Find on PubMed

Siesling, Sabine, et al. "Unified Huntington's disease rating scale: a follow up." Movement disorders 13.6 (1998): 915-919. Find on PubMed

Youssov, Katia, et al. "Unified Huntington's disease rating scale for advanced patients: Validation and follow‐up study." Movement Disorders 28.12 (2013): 1717-1723. Find on PubMed

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