Measurement Characteristics and Clinical Utility of the International Cooperative Ataxia Rating Scale in Individuals With Hereditary Ataxias

      The International Cooperative Ataxia Rating Scale (ICARS), developed by the Ataxia Neuropharmacology Committee of the World Federation of Neurology,1 can be used to quantify the level of impairment related to hereditary ataxias, monitor response to pharmacological intervention, and plan clinical trials. The test can be completed in 15 to 30 minutes and is typically administered by a physician or physical therapist. Total scores range from 0 to 100, with higher scores representing greater impairment. A score of 0 represents normal, whereas a score of 100 represents maximum impairment. The scale consists of 19 items with 4 subscales including posture and gait disturbances (34 points), kinetic function (52 points), speech disorder (8 points), and oculomotor disorders (6 points). The ICARS has demonstrated reliability and validity in individuals with spinocerebellar disease,2 Friedreich ataxia,3 multisystem atrophy,4 and chronic alcholism.5
      This Rehabilitation Measures Database summary provides a review of the psychometric properties of the ICARS with spinocerebellar disease, Friedreich ataxia, multisystem atrophy, and chronic alcoholism. A full review of the ICARS as well as reviews of more than 100 other instruments can be found at
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      • 1.
        Trouillas P, Takayanagi T, Hallett M, et al. International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. J Neurol Sci 1997;145:205-211.
      • 2.
        Schmitz-Hubsch T, Tezenas duMontcel S, Baliko L, et al. Reliability and validity of the International Cooperative Ataxia Rating Scale: a study in 156 spinocerebellar ataxia patients. Mov Disord 2006;21:699-704.
      • 3.
        Metz G, Coppard N, Cooper JM, et al. Rating disease progression of Friedreich's ataxia by the International Cooperative Ataxia Rating Scale: analysis of a 603-patient database. Brain 2013;136:259-268.
      • 4.
        Tison F, Yekhlef F, Balestre E, et al. Application of the International Cooperative Ataxia Scale rating in multiple system atrophy. Mov Disord 2002;17:1248-1254.
      • 5.
        Fitzpatrick LE, Jackson M, Crowe SF. Characterization of cerebellar ataxia in chronic alcoholics using the International Cooperative Ataxia Rating Scale (ICARS). Alcohol Clin Exp Res 2012;36: 1942-1951.
      • 6.
        Fahey MC, Corben L, Collins V, et al. How is disease progression in Friedreich's ataxia best measured? A study of four rating scales. J Neurol Neurosurg Psychiatry 2007;78:411-413.
      • 7.
        Burk K, Malzig U, Wolf S, et al. Comparison of three clinical rating scales in Friedreich ataxia (FRDA). Mov Disord 2009;24:1779-1784.
      • 8.
        Morales JA, Donis KC, Serrano-Munuera C, et al; Iberoamerican Multidisciplinary Network for the Study of Movement Disorders (RIBERMOV) Study Group. Ataxia rating scales—psychometric profiles, natural history and their application to clinical trials. Cerebellum 2012;11:488-504.
      This instrument summary is designed to facilitate the selection of outcome measures by trained clinicians. The information contained in this summary represents a sample of the peer-reviewed research available at the time of this summary's publication. The information contained in this summary does not constitute an endorsement of this instrument for clinical practice. The views expressed are those of the summary authors and do not represent those of authors' employers, instrument owner(s), the Archives of Physical Medicine and Rehabilitation, the Rehabilitation Measures Database, the United States Department of Education or the Retirement Research Foundation. The information contained in this summary has not been reviewed externally.
      The Rehabilitation Measures Database and Instrument Summary Tear-sheets are funded by the National Institute on Disability and Rehabilitation Research, United States Department of Education through the Rehabilitation Research and Training Center on Improving Measurement of Medical Rehabilitation Outcomes (H133B090024) and the Retirement Research Foundation (2011-027).