Skip to main content

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

Journal of Neurology volume 266, pages 1260–1266 (2019)Cite this article

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.

This is a preview of subscription content, access via your institution.

Access options

Buy single article

Instant access to the full article PDF.

USD 39.95

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Fig. 1
Fig. 2

References

  1. Ballard KJ, Robin DD, McCabe P, McDonald J (2010) A Treatment for dysprosody in childhood apraxia of speech. J Speech

  2. Constantinescu G, Theodoros D, Russell T, Ward EC, Wilson SM, Wootton R (2011) Treating disordered speech and voice in Parkinson’s disease online: a randomized controlled non-inferiority trial. Int J Lang Commun Disorders 46:1–16

    Google Scholar 

  3. Hargrove PM (2013) Pursuing prosody interventions. Clin Linguist Phonet 27:647–660

    Article  Google Scholar 

  4. Katz S (1983) Assessing self-maintenance: activities of daily living, mobility, and instrumental activities of daily living. J Am Geriatr Soc 31:721–727

    Article  CAS  PubMed  Google Scholar 

  5. Nasreddine ZS, Phillips NA, Bédirian V, Charbonneau S, Whitehead V, Collin I, Cummings JL, Chertkow H (2005) The montreal cognitive assessment, MoCA: a brief screening tool for mild cognitive impairment. J Am Geriatr Soc 53:695–699

    Article  PubMed  Google Scholar 

  6. Park S, Theodoros DG, Finch E, Cardell E (2016) Be clear: a new intensive speech treatment for adults with nonprogressive dysarthria. Am J Speech-Lang Pathol 25:97–110

    Article  PubMed  Google Scholar 

  7. Schiavetti N, Metz DE, Sitler RW (1981) Construct validity of direct magnitude estimation and interval scaling of speech intelligibilityevidence from a study of the hearing impaired. J Speech LangHear Res 24:441–445

    Article  CAS  Google Scholar 

  8. Schmitz-Hubsch T, Du Montcel ST, Baliko L, Berciano J, Boesch S, Depondt C, Giunti P, Globas C, Infante J, Kang J-S, Kremer B, Mariotti C, Melegh B, Pandolfo M, Rakowicz M, Ribai P, Rola R, Schols L, Szymanski S, Van De Warrenburg BP, Durr A, Klockgether T (2006) Scale for the assessment and rating of ataxia: Development of a new clinical scale. Neurology 66:1717–1720

    Article  CAS  Google Scholar 

  9. Synofzik M, Soehn AS, Gburek-Augustat J, Schicks J, Karle KN, Schüle R, Haack TB, Schöning M, Biskup S, Rudnik-Schöneborn S, Senderek J, Hoffmann KT, MacLeod P, Schwarz J, Bender B, Krüger S, Kreuz F, Bauer P, Schöls L (2013) Autosomal recessive spastic ataxia of Charlevoix Saguenay (ARSACS): expanding the genetic, clinical and imaging spectrum. Orphanet J Rare Diseases 8:41

    Article  Google Scholar 

  10. Tamplin J (2008) A pilot study into the effect of vocal exercises and singing on dysarthric speech. Neuro Rehabil 23:207–216

    Google Scholar 

  11. Tjaden K, Sussman JE, Wilding GE (2014) Impact of clear, loud, and slow speech on scaled intelligibility and speech severity in parkinson’s disease and multiple sclerosis. J Speech Lang Hear Res 57:779–792

    Article  PubMed  PubMed Central  Google Scholar 

  12. Vogel AP, Fletcher J, Snyder PJ, Fredrickson A, Maruff P (2011) Reliability, stability, and sensitivity to change and impairment in acoustic measures of timing and frequency. J Voice 25:137–149

    Article  PubMed  Google Scholar 

  13. Vogel AP, Folker JE, Poole ML (2014) Treatment for speech disorder in Friedreich ataxia and other hereditary ataxia syndromes. Cochrane Database Syst Rev 10

  14. Vogel AP, Rommel N, Oettinger A, Horger M, Krumm P, Kraus E-M, Schöls L, Synofzik M (2017) Speech and swallowing abnormalities in adults with POLG associated ataxia (POLG-A). Mitochondrion 37:1–7

    Article  CAS  PubMed  Google Scholar 

  15. Vogel AP, Rommel N, Oettinger A, Stoll LH, Kraus E-M, Gagnon C, Horger M, Krumm P, Timmann D, Storey E, Schöls L, Synofzik M (2018) Coordination and timing deficits in speech and swallowing in autosomal recessive spastic ataxia of Charlevoix–Saguenay (ARSACS). J Neurol 265:2060–2070

    Article  PubMed  Google Scholar 

  16. Weismer G, Laures JS (2002) Direct magnitude estimates of speech intelligibility in dysarthria: effects of a chosen standard. J Speech Lang Hear Res 45:421–433

    Article  PubMed  Google Scholar 

Download references

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.

Author information

Authors and Affiliations

  1. Department of Neurodegeneration, Hertie Institute for Clinical Brain Research, Germany and Center for Neurology, University of Tübingen, University Hospital Tübingen, Tübingen, Germany

    Adam P. Vogel, Lisa H. Stoll, Natalie Rommel, Eva-Maria Kraus, Ludger Schöls & Matthis Synofzik

  2. Centre for Neuroscience of Speech, The University of Melbourne, 550 Swanston Street, Parkville, Melbourne, VIC, 3010, Australia

    Adam P. Vogel

  3. RedenLab, Melbourne, Australia

    Adam P. Vogel

  4. Therapiezentrum, University Hospital Tübingen, Tübingen, Germany

    Lisa H. Stoll & Natalie Rommel

  5. Neurology and Rehabilitation, Kliniken Schmieder, Gailingen am Hochrhein, Germany

    Andreas Oettinger

  6. Department of Neurology, Essen University Hospital, University of Duisburg-Essen, Essen, Germany

    Dagmar Timmann

  7. The University of Queensland, St Lucia, Australia

    Dion Scott & Christina Atay

  8. Department of Medicine, Monash University, Melbourne, Australia

    Elsdon Storey

  9. Center for Neurodegenerative Diseases (DZNE), Tübingen, Germany

    Ludger Schöls & Matthis Synofzik

Authors
  1. Adam P. Vogel
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Lisa H. Stoll
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Andreas Oettinger
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Natalie Rommel
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Eva-Maria Kraus
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Dagmar Timmann
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Dion Scott
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Christina Atay
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Elsdon Storey
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Ludger Schöls
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Matthis Synofzik
    View author publications

    You can also search for this author in PubMed Google Scholar

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.

Ethics declarations

Conflicts of interest

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).

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary material 1 (DOCX 24 KB)

Supplementary material 2 (MOV 150740 KB)

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

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

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00415-019-09258-4

Keywords

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