Journal of Neurology

, Volume 259, Issue 3, pp 420–426 | Cite as

The scale for the assessment and rating of ataxia correlates with dysarthria assessment in Friedreich's ataxia

  • Andreas Eigentler
  • Johanna Rhomberg
  • Wolfgang Nachbauer
  • Irmgard Ritzer
  • Werner Poewe
  • Sylvia BoeschEmail author
Original Communication


Dysarthria is an acquired neurogenic sensorimotor speech symptom and an integral part within the clinical spectrum of ataxia syndromes. Ataxia measurements and disability scores generally focus on the assessment of motor functions. Since comprehensive investigations of dysarthria in ataxias are sparse, we assessed dysarthria in ataxia patients using the Frenchay Dysarthria Assessment. The Frenchay Dysarthria Assessment is a ten-item validated test in which eight items focus on the observation of oral structures and speech functions. Fifteen Friedreich's ataxia patients and 15 healthy control individuals were analyzed using clinical and logopedic methodology. All patients underwent neurological assessment applying the Scale for the Assessment and Rating of Ataxia. In Friedreich's ataxia patients, the Frenchay sub-item voice showed to be most affected compared to healthy individuals followed by items such as reflexes, palate, tongue, and intelligibility. Scoring of lips, jaw, and respiration appeared to be mildly affected. Ataxia severity in Friedreich's ataxia patients revealed a significant correlation with the Frenchay dysarthria sum score. The introduction of a binary Adapted Dysarthria Score additionally allowed allocation to distinct dysarthria pattern in ataxias. The Frenchay Dysarthria Assessment proved to be a valid dysarthria measure in Friedreich's ataxia. Its availability in several languages provides a major advantage regarding the applicability in international clinical studies. Shortcomings of the Frenchay test are the multiplicity of items tested and its alphabetic coding. Numerical scoring and condensation of assessments in a modified version may, however, provide an excellent clinical tool for the measurement and scoring of dysarthria in ataxic speech disorders.


Dysarthria Ataxia SARA Frenchay Dysarthria Assessment Friedreich's ataxia 


Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    Ziegler W, Vogel M, Gröne B (2002) Dysarthrie. Grundlagen–Diagnostik–Therapie. Thieme Verlag, 2. unveränderte Aufl, StuttgartGoogle Scholar
  2. 2.
    Spencer KA, Slocomb DL (2007) The neural basis of ataxic dysarthria. Cerebellum 6:58–65PubMedCrossRefGoogle Scholar
  3. 3.
    Böhme G (2003) Sprach-, Sprech-, Stimm- und Schluckstörungen. Band 1 Klinik. Urban & Fischer Verlag, 4. aktualisierte und erweiterte Aufl, MünchenGoogle Scholar
  4. 4.
    Campuzano V, Montermini L, Molto MD et al (1996) Friedreich’s ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion. Science 271:1423–1427PubMedCrossRefGoogle Scholar
  5. 5.
    Durr A, Cossee M, Agid Y, Campuzano V, Mignard C, Penet C, Mandel JL, Brice A, Koenig M (1996) Clinical and genetic abnormalities in patients with Friedreich’s ataxia. N Engl J Med 335:1169–1175PubMedCrossRefGoogle Scholar
  6. 6.
    Harding AE (1981) Friedreich’s ataxia: a clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clustering of clinical features. Brain 104:589–620PubMedCrossRefGoogle Scholar
  7. 7.
    Schmitz-Hübsch T, du Montcel ST, Baliko L et al (2006) Scale for the assessment and rating of ataxia: development of a new clinical scale. Neurology 66:1717–1720PubMedCrossRefGoogle Scholar
  8. 8.
    Weyer A, Abele M, Schmitz-Hübsch T, Schoch B, Frings M, Timmann D, Klockgether T (2007) Reliability and validity of the scale for the assessment and rating of ataxia: a study in 64 ataxia patients. Mov Disord 22:1633–1637PubMedCrossRefGoogle Scholar
  9. 9.
    Cano SJ, Hobart JC, Hart PE, Korlipara LV, Schapira AH, Cooper JM (2005) International Cooperative Ataxia Rating Scale (ICARS): appropriate for studies of Friedreich’s ataxia? Mov Disord 20:1585–1591PubMedCrossRefGoogle Scholar
  10. 10.
    Bürk K, Mälzig U, Wolf S et al (2009) Comparison of three clinical rating scales in Friedreich's ataxia (FRDA). Mov Disord 24:1779–1784PubMedCrossRefGoogle Scholar
  11. 11.
    Klockgether T, Lüdtke R, Kramer B et al (1998) The natural history of degenerative ataxia: a retrospective study in 466 patients. Brain 121:589–600PubMedCrossRefGoogle Scholar
  12. 12.
    Enderby PM (1991) Frenchay Dysarthrie-Untersuchung. Gustav Fischer Verlag, StuttgartGoogle Scholar
  13. 13.
    Kent RD, Kent JF, Duffy JR, Thomas JE, Weismer G, Stuntebeck S (2000) Ataxic dysarthria. J Speech Lang Hearing Res 43:1275–1289Google Scholar
  14. 14.
    Tomik B, Krupinski J, Glodzik-Sobanska L, Bala-Slodowska M, Wszolek W, Kusiak M, Lechwacka A (1999) Acoustic analysis of dysarthria profile in ALS patients. J Neurol Sci 169:35–42PubMedCrossRefGoogle Scholar
  15. 15.
    Schalling E, Hammarberg B, Hartelius L (2007) Perceptual and acoustic analysis of speech in individuals with spinocerebellar ataxia (SCA). Logoped Phoniatr Vocol 32:31–46PubMedCrossRefGoogle Scholar
  16. 16.
    Singh A, Epstein E, Myers LM, Farmer JM, Lynch DR (2010) Clinical measures of dysarthria in Friedreich's ataxia. Mov Disord 25:108–111PubMedGoogle Scholar
  17. 17.
    Schöls L, Bauer P, Schmidt T, Schulte T, Riess O (2004) Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis. Lancet Neurol 3:291–304PubMedCrossRefGoogle Scholar
  18. 18.
    Durr A (2010) Autosomal dominant cerebellar ataxias: polyglutamine expansions and beyond. Lancet Neurol 9:885–894PubMedCrossRefGoogle Scholar
  19. 19.
    Gentil M (1990) Dysarthria in Friedreich disease. Brain Lang 38:438–448PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • Andreas Eigentler
    • 1
  • Johanna Rhomberg
    • 1
  • Wolfgang Nachbauer
    • 1
  • Irmgard Ritzer
    • 1
  • Werner Poewe
    • 1
  • Sylvia Boesch
    • 1
    Email author
  1. 1.Department of NeurologyInnsbruck Medical UniversityInnsbruckAustria

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