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Scale for the Assessment and Rating of Ataxia
Availability
Freely available at this website: Scale for the Assessment and Rating of Ataxia
Classification

Core: Friedreich's Ataxia (FA)
Supplemental: Multiple Sclerosis (MS)
Supplemental - Highly Recommended: for measuring ataxia for Mitochondrial Disease (Mito)

Short Description of Instrument
This tool is specifically aimed at and restricted to the evaluation of coordination.
Construct measured: Severity of ataxia
Generic vs. disease specific: Generic
Means of administration: In person by a trained examiner
Intended respondent: Patient
# of items: 8
# of subscales and names of sub-scales: N/A
# of items per sub-scale: N/A
Patients with incoordination.
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.
No specific mitochondrial disease, although ataxia/incoordination is a common feature of many.
 
Comments/Special Instructions
Scoring: Scoring is based on a scale of 0-4, 0-6 or 0-8, depending on which item is being tested. 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 0-4/6/8
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.
Rationale/Justification
Strengths/Weaknesses: 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.
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.
Administration: Administration time can range from between 5 - 40 minutes, depending upon the ability of the patient.
References
Key Reference: Schmitz-Hubsch T, Tezenas du Montcel S, Baliko L, Berciano J, Boesch S et al. Scale for the assessment and rating of ataxia: development of a new clinical scale. Neurology 2006; 66: 1717-1720.
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.
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.
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]
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.
Bürk 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.
Marelli C, Figoni J, Charles P, Anheim M, Tchikviladze M, Vincitorio CM, du Montcel ST, Brice A, Golmard JL, Dürr 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, PÉrez LV, Latorre P, Sequeiros J, Matilla-Dueñas 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.
Jacobi H, Bauer P, Giunti P, Labrum R, Sweeney MG, Charles P, Dürr A, Marelli C, Globas C, Linnemann C, SchÖls L, Rakowicz M, Rola R, Zdzienicka E, Schmitz-Hübsch 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.
Bürk K, M?lzig U, Wolf S, Heck S, Dimitriadis K, Schmitz-Hübsch T, Hering S, Lindig TM, Haug V, Timmann D, Degen I, Kruse B, DÖrr JM, Ratzka S, Ivo A, SchÖls 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.
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.
Weyer A, Abele M, Schmitz-Hübsch 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.
Subramony SH. SARA--a new clinical scale for the assessment and rating of ataxia. Nat Clin Pract Neurol. 2007 Mar;3(3):136-7.
Schmitz-Hübsch 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, SchÖls L, Szymanski S, van de Warrenburg BP, Dürr 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.