COSMIN database

Ataxia Rating Scales-Psychometric Profiles, Natural History and Their Application in Clinical Trials

Saute, J. A., Donis, K. C., Serrano-Munuera, C., Genis, D., Ramirez, L. T., Mazzetti, P., Perez, L. V., Latorre, P., Sequeiros, J., Matilla-Duenas, A., and Jardim, L. B.
We aimed to perform a comprehensive systematic review of the existing ataxia scales. We described the disorders for which the instruments have been validated and used, the time spent in its application, its validated psychometric properties, and their use in studies of natural history and clinical trials. A search from 1997 onwards was performed in the MEDLINE, LILACS, and Cochrane databases. The web sites and were also used to identify the endpoints used in ongoing randomized clinical trials. We identified and described the semiquantitative ataxia scales (ICARS, SARA, MICARS, BARS); semiquantitative ataxia and non-ataxia scales (UMSARS, FARS, NESSCA); a semiquantitative non-ataxia scale (INAS); quantitative ataxia scales (CATSYS 2000, AFCS, CCFS and CCFSw, and SCAFI); and the self-performed ataxia scale (FAIS). SARA and ICARS were the best studied and validated so far, and their reliability sustain their use. Ataxia and non-ataxia scores will probably provide a better view of the overall disability in long-term trials and studies of natural history. Up to now, no clear advantage has been disclosed for any of them; however, we recommend the use of specific measurements of gait since gait ataxia is the first significant manifestation in the majority of ataxia disorders and comment on the best scales to be used in specific ataxia forms. Quantitative ataxia scales will be needed to speed up evidence from phase II clinical trials, from trials focused on the early phase of diseases, and for secondary endpoints in phase III trials. Finally, it is worth remembering that estimation of the actual minimal clinically relevant difference is still lacking; this, together with changes in quality of life, will probably be the main endpoints to measure in future therapeutic studies
Publication year:
Symptom status:
Physical state
Functional status:
Physical functioning
Adults (18-65)
Children (0-18)
Seniors (65+)
Congenital malformations, deformations and chromosomal abnormalities and related symptoms
Diseases of and symptoms related to the nervous system
Symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified
PRO / non-PRO:
Non-patient Reported Outcome
Patient Reported Outcome
Type of measurement instrument:
1 - Questionnaires
4 - Performance-based tests
6 - Clinical rating scales
ACFS - Ataxia Functional Composite Scale
BARS - Brief Ataxia Rating Scale
CATSYS - Coordination Ability and Tremor System (Also; CATSYS 7 and CATSYS 2000)
CCFS (and CCFSw)- Composite Cerebellar Functional Score and its Writing Version
FAIS - Friedreich’s Ataxia Impact Scale (Also: FAIS-OBS - 65-item version for observational studies; FAIS-MORE - 63-item version for studies of more disabled patients; FAIS-LESS - 63-item version for persons with less disability)
FARS - Friedreich Ataxia Rating Scale
FARS Part IV, Z2 and Z3 - Friedreich’s Ataxia Rating Scale Part IV, Z2 and Z3
ICARS - International Cooperative Ataxia Rating Scale
INAS - Inventory of Non-ataxia Symptoms
MICARS - Modified International Cooperative Ataxia Rating Scale
NESSCA - Neurological Examination Score for the Assessment of Spinocerebellar Ataxia
SARA - Scale for Assessment and Rating of Ataxia
SCAFI - Spinocerebellar Ataxia Functional Index
UMSARS - Unified Multiple System Atrophy Rating Scale