Abstract:
INTRODUCTION:Herein we present an exploratory study of orofacial function in children with congenital myotonic dystrophy (CDM) vs. healthy controls. METHODS:We evaluated 41 children with CDM and 29 healthy controls for speech and swallow function and for lingual and labial strength. RESULTS:The Iowa Oral Performance Instrument (IOPI), measuring tongue strength, and a lip force meter (LFM), measuring lip strength, had excellent interrater reliability with intraclass correlation coefficients (ICCs) of 0.75 (n = 19, P < 0.001) and 0.96 (n = 20, P < 0.001), respectively. Mean overall lingual strength was 3.5-fold less and labial strength was about 7-fold less in CDM patients than in healthy controls. Eighteen of 24 children with CDM demonstrated dysarthria and an additional 11 participants were nonverbal. Dysarthria correlated moderately with lingual strength, age, and dysphagia. Strength measures correlated moderately with dysphagia. DISCUSSION:Children with CDM have impaired orofacial functioning that affects communication and swallowing. Reliability of strength measures may be useful for future therapeutic trials. Muscle Nerve 58: 413-417, 2018.
journal_name
Muscle Nervejournal_title
Muscle & nerveauthors
Berggren KN,Hung M,Dixon MM,Bounsanga J,Crockett B,Foye MD,Gu Y,Campbell C,Butterfield RJ,Johnson NEdoi
10.1002/mus.26176subject
Has Abstractpub_date
2018-09-01 00:00:00pages
413-417issue
3eissn
0148-639Xissn
1097-4598journal_volume
58pub_type
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