Scale for Assessment and Rating of Ataxia

Last Updated

May 07, 2015

Purpose

SARA is a clinical scale that is based on a semi-quantitative assessment of cerebellar ataxia (spinocerebellar, Friedreich’s and sporadic ataxia) on an impairment level. It can also be used as a rehabilitation index of gait status and ADL independence in ataxic stroke patients.

Link to Instrument

Instrument Details

Acronym SARA

Cost

Free

Neuromuscular Conditions

SARA is an 8-item performance based scale, yielding a total score of 0 (no ataxia) to 40 (most severe ataxia).
The scores are based on patient performance of:
1) Gait
2) Stance
3) Sitting
4) Speech disturbance
5) Finger chase
6) Nose-finger test
7) Fast alternating hand movements
8) Heel-shin slide

Number of Items

Stopwatch
10 meter walkway
Examination table

22 minutes

Mean time (patients) : 14.2 ± 7.5 minutes
Mean time (healthy persons): 7.2 ± 2.6 minutes

Required Training

No Training

Instrument Reviewers

Initially reviewed by Sneha Prabhu (Post-professional Student at University of Indianapolis) on 12/05/2014

ICF Domain

Body Structure
Body Function
Activity

Considerations

The Minimally Clinically Important Difference (MCID) is not clearly measured.
It does not include oculomotor function.
The use of SARA with ataxic stroke patients as a rehabilitation index for gait ability and independence in the performance of daily living activities can be clinically valuable for both the assessment of ataxia and for rehabilitation planning.

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Standard Error of Measurement (SEM)

Spinocerebellar ataxia: (Schmitz-hübsch et al., 2010; n=171, SCA 1=43, SCA 2=61, SCA 3=37, SCA 6=30; mean age = 50.9 ± 13.5 years; mean disease duration= 11.8 ± 5.6 years; follow-up mean interval = 385 ± 41 days from baseline)

SEM calculated with ICC from the patient’s global impression stable group (n = 171) : 1.28

Minimal Detectable Change (MDC)

Spinocerebellar Ataxia: (Schmitz-hübsch et al., 2010; from baseline to one year follow-up)

MDC for individual score differences in SARA was < 3.5. (p < 0.0001)
MDC for group limits in point scores (n=171) was < 0.3.

Cut-Off Scores

The score ranges from 0 (no ataxia) to 40 (severe ataxia). The individual item scores are as follows:

Gait (0 to 8)
Stance (0 to 6)
Sitting (0 to 4)
Speech disturbance(0 to 6)
Finger chase (0 to 4)
Nose-finger test (0 to 4)
Fast alternating hand movements (0 to 4)
Heel-shin slide (0 to 4)
Limb kinetic functions (items 5 to 8) are rated independently for both body sides, and its arithmetic mean is included in total score.

Ataxic stroke : (Kim et al, 2011; n=52; mean age= 61.63 ± 15.30 years; Independent gait group= 26, Q-Cane gait group= 8, Independent walker gait group= 12, W/C ambulation group= 8; ataxic patients with grade four or more in the defective area)

The cut-off scores for gait status were:

Independent gait	8 or lower
Q-Cane gait	11.5 or lower
Walker Gait	12.25 or lower

The cut-off scores for Performance of Daily living activities was as follows:

Mild dependence	5.5 or lower
Minimal dependence	10.0 or lower
Moderate dependence	14.25 or lower
Maximal dependence	23 or higher

Normative Data

Spinocerebellar Ataxia : ( Schmitz-hübsch T, Du montcel ST, Baliko L, et al; n=229; patients=119, mean age= 50.3 ± 13.2 years; controls = 110, mean age= 47.1 ± 15.0 years; mean disease duration= 13.3 ± 8.3 years)

Mean SARA Score for spinocerebellar ataxia = 15.9 ± 8.5 (range = 1.5 to 40)
Mean SARA Score for controls = 0.4 ± 11 (range = 0 to 7.5)⁴

Ataxic stroke: (Kim et al, 2011; n=52; mean age= 61.63 ± 15.30 years; Independent gait group= 26, Q-Cane gait= 8, Walker gait= 12, W/C ambulation= 8; ataxic patients with grade four or more in the defective area)

No differences in SARA scores were found in terms of stroke type or lesion location.

Variables	Values
Stroke type
Ischemic	11.70 ± 6.75
Hemorrhage	9.30 ± 5.73
Lesion
ACA territory	7.00 ± 0.00
MCA territory	13.42 ± 7.90
PCA territory	13.75 ± 1.06
Brainstem	12.91 ± 7.12
cerebellum	8.34 ± 4.45

Mean SARA scores for gait status :

Independent gait	5.53
Q-Cane gait	9.89
Walker gait	14.97
W/C ambulation	19.6

Mean SARA scores for ADL independence:

Total dependence	26.5
Severe dependence	16.25
Maximal dependence	12.29
Moderate dependence	10.00
Minimal dependence	3.68

Test/Retest Reliability

Spinocerebellar ataxia :(Schmitz-hübsch et al, 2006)

Excellent test-retest reliability (ICC = 0.90)

Friedreich’s Ataxia: (Burk et al., 2009)

Excellent test-retest reliability (ICC = 0.99)

Interrater/Intrarater Reliability

Spinocerebellar ataxia :(Schmitz-hübsch et al, 2006)

Excellent inter-rater reliability (ICC = 0.98)
All single items had excellent inter-rater reliability (ICC > 0.8), with exception of left heel-shin test (ICC = 0.74).

Sporadic Ataxia Disorders: (Weyer et al., 2007)

Excellent inter-rater reliability (ICC = 0.98)
Excellent intra-rater reliability (ICC = 0.99)

Chinese version of SARA: (Tan S, Niu HX, Zhao L, et al., 2013)

Excellent inter-rater reliability (ICC = 0.86)

Internal Consistency

Spinocerebellar Ataxia : (Schmitz-hübsch et al, 2006)

Excellent Internal consistency (Cronbach’s alpha = 0.94)

Friedreich’s Ataxia: (Burk et al., 2009)

Excellent internal consistency (Cronbach’s alpha = 0.89)

Sporadic Ataxia Disorders : (Weyer et al., 2007)

Excellent internal consistency (Cronbach’s alpha = 0.97)

Chinese version of SARA: (Tan S, Niu HX, Zhao L, et al., 2013)

Adequate internal consistency (Cronbach’s alpha = 0.78)

Criterion Validity (Predictive/Concurrent)

Concurrent Validity:

Spinocerebellar Ataxia (Subramony SH, 2007)

Excellent concurrent validity of the SARA with the Barthel Index (r = -0.8, P < 0.0001)
Excellent concurrent validity with Unified Huntington’s Disease Rating Scale (UHRDS-IV) (r = -0.89, P < 0.0001)

Construct Validity

Construct Validity:

Friedreich’s ataxia (Saute et al., 2012)

Excellent construct validity of SARA items with International Cooperative Ataxia Rating Scale (ICARS) (r = 0.953)
Excellent construct validity of SARA with Friedreich’s Ataxia rating Scale (FARS) (r = 0.938) (Saute et al., 2012)

Floor/Ceiling Effects

Spinocerebellar Ataxia (Schmitz-hübsch et al., 2010)

Adequate floor effects at 1 year follow-up = 0.6%

Responsiveness

Spinocerebellar Ataxia (Schmitz-hübsch et al., 2010)

Moderately responsive to correctly classify cases with subjectively relevant worsening (Effect Size = 0.613)

Bibliography

Saute JA, Donis KC, Serrano-munuera C, et al. Ataxia rating scales--psychometric profiles, natural history and their application in clinical trials. Cerebellum. 2012; 11(2):488-504.

Subramony SH. SARA--a new clinical scale for the assessment and rating of ataxia. Nat Clin Pract Neurol.2007; 3(3):136-7.

Schmitz-hübsch T, Fimmers R, Rakowicz M, et al. Responsiveness of different rating instruments in spinocerebellar ataxia patients. Neurology. 2010; 74(8):678-84.

Schmitz-hübsch T, Du montcel ST, Baliko L, et al. Scale for the assessment and rating of ataxia: development of a new clinical scale. Neurology . 2006; 66(11):1717-20.

Bürk K, Mälzig U, Wolf S, et al. Comparison of three clinical rating scales in Friedreich ataxia (FRDA). Mov Disord. 2009; 24(12):1779-84.

Weyer A, Abele M, Schmitz-hübsch T, et al. Reliability and validity of the scale for the assessment and rating of ataxia: a study in 64 ataxia patients. Mov Disord. 2007; 22(11):1633-7.

Tan S, Niu HX, Zhao L, et al. Reliability and validity of the Chinese version of the Scale for Assessment and Rating of Ataxia. Chin Med J. 2013; 126(11):2045-8.

Kim BR, Lim JH, Lee S, Park S, Koh SE, Lee IS, Jung H, Lee J. Usefulness of the Scale for the Assessment and Rating of Ataxia (SARA) in Ataxic Stroke Patients. Ann Rehabil Med. 2011; 35: 772-780.