title: Clinical measures of dysarthria in Friedreich Ataxia.
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.
authors.affiliation: Department of Neurology , University of Pennsylvania School of Medicine and The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104-4318, USA.
authors.forename: Arunjot
authors.initials: A
authors.lastname: Singh
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Epstein
authors.forename: Lauren M
authors.initials: LM
authors.lastname: Myers
authors.forename: Jennifer M
authors.initials: JM
authors.lastname: Farmer
authors.forename: David R
authors.initials: DR
authors.lastname: Lynch
mesh_terms: D000328:Adult; D016022:Case-Control Studies; D004401:Dysarthria / Q000175:diagnosis* / Q000209:etiology*; D005260:Female; D005621:Friedreich Ataxia / Q000150:complications*; D006801:Humans; D008297:Male; D013060:Speech / Q000502:physiology; D013223:Statistics as Topic; D055815:Young Adult