title: The scale for the assessment and rating of ataxia correlates with dysarthria assessment in Friedreich's ataxia.
abstract: 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.
authors.affiliation: Department of Neurology, Innsbruck Medical University, Anichstrasse 35, 6020, Innsbruck, Austria.
authors.forename: Andreas
authors.initials: A
authors.lastname: Eigentler
authors.forename: Johanna
authors.initials: J
authors.lastname: Rhomberg
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Nachbauer
authors.forename: Irmgard
authors.initials: I
authors.lastname: Ritzer
authors.forename: Werner
authors.initials: W
authors.lastname: Poewe
authors.forename: Sylvia
authors.initials: S
authors.lastname: Boesch
mesh_terms: D000328:Adult; D001259:Ataxia / Q000175:diagnosis* / Q000209:etiology; D004185:Disability Evaluation; D004401:Dysarthria / Q000175:diagnosis* / Q000209:etiology; D005260:Female; D005621:Friedreich Ataxia / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D007802:Language; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination; D012044:Regression Analysis; D012720:Severity of Illness Index; D013067:Speech Perception / Q000502:physiology; D055815:Young Adult