Language impairment in cerebellar ataxia

Authors

  • Judith van Gaalen MD,

    Corresponding author
    1. Departments of Neurology, Donders Institute for Brain, Cognition and Behavior, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
    • Correspondence to: Dr. J. van Gaalen, Department of Neurology, Radboud University Nijmegen Medical Center, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands; Judith.vangaalen@radboudumc.nl

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  • Bert J.M. de Swart PhD,

    1. Rehabilitation, Donders Institute for Brain, Cognition and Behavior, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
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  • Judith Oostveen,

    1. Rehabilitation, Donders Institute for Brain, Cognition and Behavior, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
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  • Simone Knuijt MA,

    1. Rehabilitation, Donders Institute for Brain, Cognition and Behavior, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
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  • Bart P.C. van de Warrenburg MD, PhD,

    1. Departments of Neurology, Donders Institute for Brain, Cognition and Behavior, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
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  • Berry (H.) P.H Kremer MD, PhD

    1. Department of Neurology, University Medical Center Groningen, Groningen, The Netherlands
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  • Relevant conflicts of interest/financial disclosures: B.v.d.W. is supported by grants from the Netherlands Brain Foundation, the Prinses Beatrix Fonds, the Royal Dutch Society for Physical Therapy, the Radboud University Nijmegen Medical Center, the Gossweiler Foundation, and Biobanking and Biomolecular Research Infrastructure (BBMRI-NL). J.v.G. is supported by the Gossweiler Foundation.

  • Full financial disclosures and author roles may be found in the online version of this article.

ABSTRACT

Background

Several studies have suggested that language impairment can be observed in patients with cerebellar pathology. The aim of this study was to investigate language performance in patients with spinocerebellar ataxia type 6 (SCA6).

Methods

We assessed speech and language in 29 SCA6 patients with standardized linquistic tests and correlated this with the severity of ataxia, as quantified by the Scale of Assessment and Rating of Ataxia.

Results

Individual patients show mild-to-moderate linguistic impairment. Linguistic abnormalities were most distinct on the writing and comprehension subtests. A strong correlation between severity of ataxia and linguistic performance was consistently found.

Conclusions

This study confirms the occurrence of linguistic impairments in patients with cerebellar degenerative diseases, such as SCA6. The relation between linguistic abnormalities and severity of ataxia provides further evidence for a role of the cerebellum in linguistic processing. © 2014 International Parkinson and Movement Disorder Society

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