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Scale for the Assessment and Rating of Ataxia (SARA)
Availability
Please visit this website for more information about the instrument: Scale for the Assessment and Rating of Ataxia
Classification
Core: Friedreich's Ataxia (FA)
 
Supplemental - Highly Recommended: Mitochondrial Disease (Mito)
Recommendations for use: Indicated for studies measuring ataxia.
 
Supplemental: Multiple Sclerosis (MS)
Short Description of Instrument
The Scale for Assessment and Rating of Ataxia (SARA) is a clinical rating scale for ataxic patients (Schmitz-Huebsch et al., 2006), and is specially aimed at and restricted to the evaluation of coordination.
 
Consisting of eight items (1: gait, 2: stance, 3: sitting, 4: speech, 5: nose-finger test, 6: finger chase, 7: fast alternating hand movements, and 8: heel-shin slide), the test can be performed with reasonable consistency in all age-groups, even in the pediatric population, as long as the patients are able (physically/ mentally) to perform these simple movements.
 
Administration time can range from between 5 - 40 minutes, depending upon the ability of the patient.
 
No specific mitochondrial disease, although ataxia/incoordination is a common feature of many.
Comments/Special Instructions
Background: The SARA is a clinical scale based on a semiquantitative assessment of cerebellar ataxia on an impairment level. It has eight items: gait, stance, sitting, speech, finger-chase test, nose-finger test, fast alternating movements, and heel-shin test. This scale was developed due to the need for a reliable and valid clinical scale measuring the severity of ataxia.
Scoring and Psychometric Properties
Scoring: Scoring is based on a scale of 0-4, 0-6, or 0-8, depending on which item is being assessed. A score of 0 indicates a patient's normal ability to perform the task, while the higher number indicates a patient's inability to perform the task. Individual instructions, as well as definitions of scores, can be found on the actual scale.
 
SARA consists of eight items, together yielding a total score between 0 (no ataxia) and 40 (most severe ataxia); (1) gait (score 0-8); (2) stance (score 0-6); (3) sitting (score 0-4); (4) speech disturbances (score 0-6); (5) finger chase (score 0-4); (6) nose-finger test (score 0-4); (7) fast alternating hand movements (score 0-4); and (8) heel-shin slide (score 0-4). All limb kinetic functions are rated independently for both sides (items 5-8) and the arithmetic mean of both sides is included in the total score. Semi-quantitatively. Scales vary from 0-4/0-6/0-8.
 
Psychometric Properties: This scale has been shown to be a reliable and valid scale for measuring ataxia. Its scores correlate closely with other scales of ataxia, as well as activities of daily living.
Rationale/Justification
Strengths: SARA has good metric properties and inter-rater reliability, is easy to use, and seems to be a promising outcome measure for future clinical trials.
 
Weaknesses: The Minimally Clinically Important Difference (MCID) is not clearly measured, and the SARA does not include oculomotor function. The SARA is subject to inter- and intra-rater variability and its use in clinical trials is limited by the availability of a trained examiner. (Taheri Amin et al., 2023)
References
Key Reference:
Schmitz-Huebsch T, du Montcel ST, Baliko L, Berciano J, Boesch S, Depondt C, Giunti P, Globas C, Infante J, Kang JS, Kremer B, Mariotti C, Melegh B, Pandolfo M, Rakowicz M, Ribai P, Rola R, Schoels L, Szymanski S, van de Warrenburg BP, Duerr A, Klockgether T, Fancellu R. Scale for the assessment and rating of ataxia: development of a new clinical scale. Neurology. 2006 Jun 13;66(11):1717-20.
 
Additional References:
Brandsma R, Spits AH, Kuiper MJ, Lunsing RJ, Burger H, Kremer HP, Sival DA; Childhood Ataxia and Cerebellar Group. Ataxia rating scales are age-dependent in healthy children. Dev Med Child Neurol. 2014 Jun;56(6):556-63.
 
Buerk K, Maelzig U, Wolf S, Heck S, Dimitriadis K, Schmitz-Huebsch T, Hering S, Lindig TM, Haug V, Timmann D, Degen I, Kruse B, Doerr JM, Ratzka S, Ivo A, Schoels L, Boesch S, Klockgether T, Klopstock T, Schulz JB. Comparison of three clinical rating scales in Friedreich ataxia (FRDA). Mov Disord. 2009 Sep 15;24(12):1779-84.
 
Buerk K, Schulz SR, Schulz JB. Monitoring progression in Friedreich ataxia (FRDA): the use of clinical scales. J Neurochem. 2013 Aug;126 Suppl 1:118-24.
 
Jacobi H, Bauer P, Giunti P, Labrum R, Sweeney MG, Charles P, Duerr A, Marelli C, Globas C, Linnemann C, Schols L, Rakowicz M, Rola R, Zdzienicka E, Schmitz-Huebsch T, Fancellu R, Mariotti C, Tomasello C, Baliko L, Melegh B, Filla A, Rinaldi C, van de Warrenburg BP, Verstappen CC, Szymanski S, Berciano J, Infante J, Timmann D, Boesch S, Hering S, Depondt C, Pandolfo M, Kang JS, Ratzka S, Schulz J, Tezenas du Montcel S, Klockgether T. The natural history of spinocerebellar ataxia type 1, 2, 3, and 6: a 2-year follow-up study. Neurology. 2011 Sep 13;77(11):1035-41.
 
Marelli C, Figoni J, Charles P, Anheim M, Tchikviladze M, Vincitorio CM, du Montcel ST, Brice A, Golmard JL, Durr A. Annual change in Friedreich's ataxia evaluated by the Scale for the Assessment and Rating of Ataxia (SARA) is independent of disease severity. Mov Disord. 2012 Jan;27(1):135-8.
 
Saute JA, Donis KC, Serrano-Munuera C, Genis D, Ramirez LT, Mazzetti P, Perez LV, Latorre P, Sequeiros J, Matilla-Duenas A, Jardim LB; Iberoamerican Multidisciplinary Network for the Study of Movement Disorders (RIBERMOV) Study
Group. Ataxia rating scales--psychometric profiles, natural history and their application in clinical trials. Cerebellum. 2012 Jun;11(2):488-504.
 
Schmitz-Huebsch T, du Montcel ST, Baliko L, Berciano J, Boesch S, Depondt C, Giunti P, Globas C, Infante J, Kang JS, Kremer B, Mariotti C, Melegh B, Pandolfo M, Rakowicz M, Ribai P, Rola R, Schoels L, Szymanski S, van de Warrenburg BP, Duerr A, Klockgether T, Fancellu R. Scale for the assessment and rating of ataxia: development of a new clinical scale. Neurology. 2006 Jun 13;66(11):1717-20.
 
Subramony SH. SARA--a new clinical scale for the assessment and rating of ataxia. Nat Clin Pract Neurol. 2007 Mar;3(3):136-7.
 
Taheri Amin A, Faber J, Oender D, Kimmich O, Synofzik M, Ashizawa T, Klockgether T, Grobe-Einsler M. Comparison of Live and Remote Video Ratings of the Scale for Assessment and Rating of Ataxia. Mov Disord Clin Pract. 2023 Aug 7;10(9):1404-1407.
 
Ueda N, Hakii Y, Koyano S, Higashiyama Y, Joki H, Baba Y, Suzuki Y, Kuroiwa Y, Tanaka F. Quantitative analysis of upper-limb ataxia in patients with spinocerebellar degeneration. J Neurol. 2014 Jul;261(7):1381-6.
 
Weyer A, Abele M, Schmitz-Huebsch T, Schoch B, Frings M, Timmann D, Klockgether T. Reliability and validity of the scale for the assessment and rating of ataxia: a study in 64 ataxia patients. Mov Disord. 2007 Aug 15;22(11):1633-7.
 
Winser SJ, Smith C, Hale LA, Claydon LS, Whitney SL. Balance outcome measures in cerebellar ataxia: a Delphi survey. Disabil Rehabil. 2014 Apr 29. [Epub ahead of print]
 
Yabe I, Matsushima M, Soma H, Basri R, Sasaki H. Usefulness of the Scale for Assessment and Rating of Ataxia (SARA). J Neurol Sci. 2008 Mar 15;266(1-2):164-6.
 
Yasui K, Yabe I, Yoshida K, Kanai K, Arai K, Ito M, Onodera O, Koyano S, Isozaki E, Sawai S, Adachi Y, Sasaki H, Kuwabara S, Hattori T, Sobue G, Mizusawa H, Tsuji S, Nishizawa M, Nakashima K. A 3-year cohort study of the natural history of spinocerebellar ataxia type 6 in Japan. Orphanet J Rare Dis. 2014 Jul 23;9(1):118.
 
Document last updated March 2024