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. 2021 Feb;36(2):283-297.
doi: 10.1002/mds.28313. Epub 2020 Oct 6.

Assessment of Ataxia Rating Scales and Cerebellar Functional Tests: Critique and Recommendations

Santiago Perez-Lloret  1   2   3 , Bart van de Warrenburg  4 , Malco Rossi  5 , Carmen Rodríguez-Blázquez  6 , Theresa Zesiewicz  7 , Jonas A M Saute  8   9   10   11 , Alexandra Durr  12 , Masatoyo Nishizawa  13 , Pablo Martinez-Martin  14 , Glenn T Stebbins  15 , Anette Schrag  16 , Matej Skorvanek  17   18 ; members of the MDS Rating Scales Review Committee
Affiliations

Assessment of Ataxia Rating Scales and Cerebellar Functional Tests: Critique and Recommendations

Santiago Perez-Lloret et al. Mov Disord. 2021 Feb.

Abstract

Background: We assessed the clinimetric properties of ataxia rating scales and functional tests, and made recommendations regarding their use.

Methods: A systematic literature search was conducted to identify the instruments used to rate ataxia symptoms. The identified rating scales and functional ability tests were reviewed and ranked by the panel as "recommended," "suggested," or "listed" for the assessment of patients with discrete cerebellar disorders, using previously established criteria.

Results: We reviewed 14 instruments (9 rating scales and 5 functional tests). "Recommended" rating scales for the assessment of symptoms severity were: for Friedreich's ataxia, the Friedreich's Ataxia Rating Scale, the International Cooperative Ataxia Rating Scale (ICARS), and the Scale for the Assessment and Rating of Ataxia (SARA); for spinocerebellar ataxias, ICARS and SARA; for ataxia telangiectasia: ICARS and SARA; for brain tumors, SARA; for congenital disorder of glycosylation-phosphomannomutase-2 deficiency, ICARS; for cerebellar symptoms in multiple sclerosis, ICARS; for cerebellar symptoms in multiple system atrophy: Unified Multiple System Atrophy Rating Scale and ICARS; and for fragile X-associated tremor ataxia syndrome, ICARS. "Recommended" functional tests were: for Friedreich's ataxia, Ataxia Functional Composite Score and Composite Cerebellar Functional Severity Score; and for spinocerebellar ataxias, Ataxia Functional Composite Score, Composite Cerebellar Functional Severity Score, and SCA Functional Index.

Conclusions: We identified some "recommended" scales and functional tests for the assessment of patients with major hereditary ataxias and other cerebellar disorders. The main limitations of these instruments include the limited assessment of patients in the more severe end of the spectrum and children. Further research in these populations is warranted. © 2020 International Parkinson and Movement Disorder Society.

Keywords: Friedreich's ataxia; ataxia; cerebellar disorders; clinical trials; rating scales; spinocerebellar ataxia.

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