Clinical measures of dysarthria in Friedreich Ataxia

Authors

  • Arunjot Singh MS,

    1. Departments of Neurology and Pediatrics, University of Pennsylvania School of Medicine and The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
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  • Elizabeth Epstein BA,

    1. Departments of Neurology and Pediatrics, University of Pennsylvania School of Medicine and The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
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  • Lauren M. Myers BA,

    1. Departments of Neurology and Pediatrics, University of Pennsylvania School of Medicine and The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
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  • Jennifer M. Farmer MS,

    1. Departments of Neurology and Pediatrics, University of Pennsylvania School of Medicine and The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
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  • David R. Lynch MD, PhD

    Corresponding author
    1. Departments of Neurology and Pediatrics, University of Pennsylvania School of Medicine and The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    • Division of Neurology, Children's Hospital of Philadelphia, 502 Abramson Building, Philadelphia, PA 19104-4318
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  • Potential conflict of interest: None reported.

Abstract

Dysarthria in Friedreich Ataxia (FA) is difficult to quantify. This study evaluated a series of performance measures for speech in 22 patients with genetically confirmed FA and 16 age-matched controls. Tests included the PATA examination, the PATAKA examination, the Oral Motor component of the Boston Aphasia examination, the Boston Cookie Theft description task, and the Assessment of Intelligibility of Dysarthric Speech. All measures, except the Cookie theft description task, demonstrated significantly lower scores for patients with FA when compared with controls and correlated with measures of disease progression. Thus, four of five measures capture speech dysfunction in FA and may provide feasible, inexpensive, quantitative testing for therapeutic monitoring in FA. © 2009 Movement Disorder Society

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