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Brain Lang. 2013 Dec;127(3):317-22. doi: 10.1016/j.bandl.2013.10.002. Epub 2013 Oct 30.

Speech in spinocerebellar ataxia.

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Department of Clinical Science, Intervention and Technology, Division of Speech and Language Pathology, Karolinska Institutet, 141 86 Stockholm, Sweden; Department of Speech and Language Pathology, Karolinska University Hospital, 171 76 Stockholm, Sweden. Electronic address:


Spinocerebellar ataxias (SCAs) are a heterogeneous group of autosomal dominant cerebellar ataxias clinically characterized by progressive ataxia, dysarthria and a range of other concomitant neurological symptoms. Only a few studies include detailed characterization of speech symptoms in SCA. Speech symptoms in SCA resemble ataxic dysarthria but symptoms related to phonation may be more prominent. One study to date has shown an association between differences in speech and voice symptoms related to genotype. More studies of speech and voice phenotypes are motivated, to possibly aid in clinical diagnosis. In addition, instrumental speech analysis has been demonstrated to be a reliable measure that may be used to monitor disease progression or therapy outcomes in possible future pharmacological treatments. Intervention by speech and language pathologists should go beyond assessment. Clinical guidelines for management of speech, communication and swallowing need to be developed for individuals with progressive cerebellar ataxia.


Ataxic dysarthria; Instrumental acoustic analysis; Perceptual analysis; Progression of dysarthria; Speech and language pathology intervention; Spinocerebellar ataxia

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