Orofacial strength, dysarthria, and dysphagia in congenital myotonic dystrophy.

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 Nerve

journal_title

Muscle & nerve

authors

Berggren KN,Hung M,Dixon MM,Bounsanga J,Crockett B,Foye MD,Gu Y,Campbell C,Butterfield RJ,Johnson NE

doi

10.1002/mus.26176

subject

Has Abstract

pub_date

2018-09-01 00:00:00

pages

413-417

issue

3

eissn

0148-639X

issn

1097-4598

journal_volume

58

pub_type

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