Speech treatment improves dysarthria in multisystemic ataxia: a rater-blinded, controlled pilot-study in ARSACS

Abstract

We aimed to provide proof-of-principle evidence that intensive home-based speech treatment can improve dysarthria in complex multisystemic degenerative ataxias, exemplified by autosomal recessive spastic ataxia Charlevoix-Saguenay (ARSACS). Feasibility and piloting efficacy of speech training specifically tailored to cerebellar dysarthria was examined through a 4-week program in seven patients with rater-blinded assessment of intelligibility (primary outcome) and naturalness and acoustic measures of speech (secondary outcomes) performed 4 weeks before, immediately prior to, and directly after training (intraindividual control design). Speech intelligibility and naturalness improved post treatment. This provides piloting evidence that ataxia-tailored speech treatment might be effective in degenerative cerebellar disease.

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Acknowledgements

This study was supported by the Ataxia Charlevoix-Saguenay Foundation and from the European Union’s Horizon 2020 research and innovation program under the ERA-NET Cofund action N° 643578. It was supported by the BMBF (01GM1607 to M.S.), under the frame of the E-Rare-3 network PREPARE (to M.S.). A.P.V. received salaried support from the National Health and Medical Research Council, Australia (Career Development Fellowship ID 1082910), and received funding from the Alexander von Humboldt Foundation. This study was supported by the IZKF Promotionskolleg Tübingen (IZKF 2016-1-07) to M.S. and E.K.

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Contributions

AV designed the treatment and contributed to the design of the study, collection, analysis and interpretation of the data, and drafting the manuscript. He also supervised students, led the research team, and obtained funding for the research. LS contributed to data analysis and interpretation and revising the manuscript for intellectual content. AO contributed to data analysis and interpretation and revising the manuscript for intellectual content. E-MK contributed to data analysis and interpretation and revising the manuscript for intellectual content. DS contributed to therapy material preparation and revising the manuscript for intellectual content. CA contributed to therapy material preparation and revising the manuscript for intellectual content. NR contributed to the design of the study, collected data, analysis and interpretation of the data, revising the manuscript for intellectual content, and supervision of students. LS, DT and ES contributed to data interpretation and revising the manuscript for intellectual content. MS contributed to the design of the study, collection, analysis and interpretation of the data, and revising the manuscript for intellectual content. He also supervised students, led the research team, and obtained funding for the research.

Corresponding author

Correspondence to Adam P. Vogel.

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All authors declare that they have no conflict of interest.

Ethical standards

The study received institutional ethical approval (The University of Melbourne 1339394.2, University Hospital Tübingen, Germany Az. 003/2015BO2). The trial was registered with ID ACTRN12616001583437 (anzctr.org.au).

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Vogel, A.P., Stoll, L.H., Oettinger, A. et al. Speech treatment improves dysarthria in multisystemic ataxia: a rater-blinded, controlled pilot-study in ARSACS. J Neurol 266, 1260–1266 (2019). https://doi.org/10.1007/s00415-019-09258-4

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Keywords

  • Speech
  • Dysarthria
  • Rehabilitation
  • Acoustics
  • Ataxic neuropathy
  • Voice