Dysarthria and broader motor speech deficits in Dravet syndrome

SJ Turner; A Brown; M Arpone; V Anderson; AT Morgan; IE Scheffer

Journal article

Dysarthria and broader motor speech deficits in Dravet syndrome

SJ Turner, A Brown, M Arpone, V Anderson, AT Morgan, IE Scheffer

Neurology | LIPPINCOTT WILLIAMS & WILKINS | Published : 2017

DOI: 10.1212/WNL.0000000000003635

Abstract

Objective: To analyze the oral motor, speech, and language phenotype in 20 children and adults with Dravet syndrome (DS) associated with mutations in SCN1A. Methods: Fifteen verbal and 5 minimally verbal DS patients with SCN1A mutations (aged 15 months-28 years) underwent a tailored assessment battery. Results: Speech was characterized by imprecise articulation, abnormal nasal resonance, voice, and pitch, and prosody errors. Half of verbal patients had moderate to severely impaired conversational speech intelligibility. Oral motor impairment, motor planning/programming difficulties, and poor postural control were typical. Nonverbal individuals had intentional communication. Cognitive skills ..

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University of Melbourne Researchers

Vicki Anderson Author

Angela Morgan Author

Ingrid Scheffer Author

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Grants

Awarded by Australian Research Council

Funding Acknowledgements

S.J.T. is supported by National Health and Medical Research Council (NHMRC) Postgraduate Scholarship (101777), Australian National University Gowrie Scholarship, and Speech Pathology Australia Nadia Verrall Memorial Research Grant.V.A. is supported by NHMRC Senior Practitioner Fellowship (2010-2019). A.T.M. is supported by NHMRC Career Development Award (607315, 2010-2015) and Practitioner Fellowship (1105008, 2016-2020). I. E. S. is supported by NHMRC Program Grant (628952, 2011-2015;1091593,2016-2020) and Senior Practitioner Fellowship ( 1006110,2011-2015;1104831,2016-2020). The project is also supported by Australian Research Council Discovery Project (DP120100285) to A.T.M. and I.E.S.