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Clinical Scales of Cerebellar Ataxias

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Handbook of the Cerebellum and Cerebellar Disorders
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Abstract

Clinical scales represent an important tool not only for the initial grading/scoring of disease and assessment of progression but also for the quantification of therapeutic effects in clinical trials. There are several scales available for the clinical evaluation of cerebellar symptoms. While some scales have been developed and evaluated for specific cerebellar disorders such as Friedreich ataxia (FA), others reliably capture cerebellar symptoms with no respect to the underlying etiology. Each scale has its strengths and weaknesses. Extensive scales are certainly useful for thorough documentation of specific features of certain phenotypes, but this gain of information is not always essential for the purpose of a study. Therefore, compact and manageable scales like SARA or BARS are often preferred compared to more complex scales in observational and therapeutic studies.

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Abbreviations

ADL:

Activities of daily living

BARS:

Brief ataxia rating scale

CACG-EPNS:

Childhood Ataxia and Cerebellar Group of the European Pediatric Neurology Society

EA:

Episodic ataxia

MBI:

Barthel Index

CGI:

Clinical Global Impression

FA:

Friedreich ataxia

FARS:

Friedreich Ataxia Rating Scale

FIM:

Functional Independence Measure

ICARS:

International Cooperative Ataxia Rating Scale

ICC:

Intraclass correlation coefficient

IRR:

Interrater reliability

Min:

Minutes

MRI:

Magnetic resonance imaging

MRS:

Magnetic resonance spectroscopy

MSA:

Multiple system atrophy

NPC:

Niemann-Pick type C

PI:

Prediction interval

QoL:

Quality of life

SARA:

Scale for the Assessment and Rating of Ataxia

SCA:

Spinocerebellar ataxia

T25FW:

Timed 25-meter foot walk

UHDRS:

Unified Huntington’s Disease Rating Scale

UPDRS:

Unified Parkinson’s Disease Rating Scale

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Conflicts of Interest

K. Bürk and D. A. Sival do not have any conflict of interest.

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Correspondence to Katrin Bürk .

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Bürk, K., Sival, D.A. (2019). Clinical Scales of Cerebellar Ataxias. In: Manto, M., Gruol, D., Schmahmann, J., Koibuchi, N., Sillitoe, R. (eds) Handbook of the Cerebellum and Cerebellar Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-97911-3_80-2

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  • DOI: https://doi.org/10.1007/978-3-319-97911-3_80-2

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-97911-3

  • Online ISBN: 978-3-319-97911-3

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