TY - JOUR
T1 - Assessment of Ataxia Rating Scales and Cerebellar Functional Tests
T2 - Critique and Recommendations
AU - and members of the MDS Rating Scales Review Committee
AU - Perez-Lloret, Santiago
AU - van de Warrenburg, Bart
AU - Rossi, Malco
AU - Rodríguez-Blázquez, Carmen
AU - Zesiewicz, Theresa
AU - Saute, Jonas A.M.
AU - Durr, Alexandra
AU - Nishizawa, Masatoyo
AU - Martinez-Martin, Pablo
AU - Stebbins, Glenn T.
AU - Schrag, Anette
AU - Skorvanek, Matej
AU - Brown, Richard G.
AU - Goldman, Jennifer G.
AU - de Siqueira Tosin, Michelle Hyczy
AU - Shukla, Aparna Wagle
AU - Violante, Mayela Rodriguez
AU - Weintraub, Daniel
N1 - Publisher Copyright:
© 2020 International Parkinson and Movement Disorder Society
Copyright:
Copyright 2020 Elsevier B.V., All rights reserved.
PY - 2020
Y1 - 2020
N2 - 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.
AB - 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.
KW - ataxia
KW - cerebellar disorders
KW - clinical trials
KW - Friedreich's ataxia
KW - rating scales
KW - spinocerebellar ataxia
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U2 - 10.1002/mds.28313
DO - 10.1002/mds.28313
M3 - Review article
C2 - 33022077
AN - SCOPUS:85097863851
JO - Movement Disorders
JF - Movement Disorders
SN - 0885-3185
ER -