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January 25, 2019Article

Effects of weakness of orofacial muscles on swallowing and communication in FSHD

View ORCID ProfileKarlien Mul, Kiera N. Berggren, Mattie Y. Sills, Ayla McCalley, Baziel G.M. van Engelen, Nicholas E. Johnson, View ORCID ProfileJeffrey M. Statland
First published January 25, 2019, DOI: https://doi.org/10.1212/WNL.0000000000007013
Karlien Mul
From the Department of Neurology (K.M., M.Y.S., A.M., J.M.S.), University of Kansas Medical Center, Kansas City; Department of Neurology (K.N.B., N.E.J.), Virginia Commonwealth University, Richmond; and Department of Neurology (K.M., B.G.M.v.E.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands.
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Kiera N. Berggren
From the Department of Neurology (K.M., M.Y.S., A.M., J.M.S.), University of Kansas Medical Center, Kansas City; Department of Neurology (K.N.B., N.E.J.), Virginia Commonwealth University, Richmond; and Department of Neurology (K.M., B.G.M.v.E.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands.
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Mattie Y. Sills
From the Department of Neurology (K.M., M.Y.S., A.M., J.M.S.), University of Kansas Medical Center, Kansas City; Department of Neurology (K.N.B., N.E.J.), Virginia Commonwealth University, Richmond; and Department of Neurology (K.M., B.G.M.v.E.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands.
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Ayla McCalley
From the Department of Neurology (K.M., M.Y.S., A.M., J.M.S.), University of Kansas Medical Center, Kansas City; Department of Neurology (K.N.B., N.E.J.), Virginia Commonwealth University, Richmond; and Department of Neurology (K.M., B.G.M.v.E.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands.
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Baziel G.M. van Engelen
From the Department of Neurology (K.M., M.Y.S., A.M., J.M.S.), University of Kansas Medical Center, Kansas City; Department of Neurology (K.N.B., N.E.J.), Virginia Commonwealth University, Richmond; and Department of Neurology (K.M., B.G.M.v.E.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands.
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Nicholas E. Johnson
From the Department of Neurology (K.M., M.Y.S., A.M., J.M.S.), University of Kansas Medical Center, Kansas City; Department of Neurology (K.N.B., N.E.J.), Virginia Commonwealth University, Richmond; and Department of Neurology (K.M., B.G.M.v.E.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands.
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Jeffrey M. Statland
From the Department of Neurology (K.M., M.Y.S., A.M., J.M.S.), University of Kansas Medical Center, Kansas City; Department of Neurology (K.N.B., N.E.J.), Virginia Commonwealth University, Richmond; and Department of Neurology (K.M., B.G.M.v.E.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands.
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Citation
Effects of weakness of orofacial muscles on swallowing and communication in FSHD
Karlien Mul, Kiera N. Berggren, Mattie Y. Sills, Ayla McCalley, Baziel G.M. van Engelen, Nicholas E. Johnson, Jeffrey M. Statland
Neurology Jan 2019, 10.1212/WNL.0000000000007013; DOI: 10.1212/WNL.0000000000007013

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Abstract

Objective This study explores the use of quantitative data on strength and fatigability of orofacial muscles in patients with facioscapulohumeral muscular dystrophy (FSHD) and assesses the frequency of swallowing and communication difficulties and their relationship to orofacial muscle involvement.

Methods We included 43 patients with FSHD and 35 healthy controls and used the Iowa Oral Performance Instrument (IOPI) to obtain quantitative measurements of strength and endurance of lip compression, cheek (buccodental) compression, and tongue elevation. For the assessment of swallowing and communication difficulties, we used the dysphagia-specific quality of life (SWAL-QOL) and Communicative Participation Item Bank questionnaires.

Results Cheek compression strength was reduced in patients with FSHD compared to healthy controls. Dysphagia and difficulty with verbal communication were reported by 25% and 35% of patients, respectively, and correlated to cheek compression strength and endurance and to anterior tongue elevation endurance. Prolonged cheek compression or anterior tongue elevation endurance (decreased fatigability) made swallowing or speech problems less likely to occur.

Conclusion Cheek compression strength is the most sensitive IOPI measure for orofacial weakness in FSHD. Orofacial weakness contributes to dysphagia and speech difficulties in FSHD, which are both common, though generally mild. Higher endurance of orofacial muscles was associated with a lower chance of dysphagia or speech problems. More research is required for further refinement of the pattern of facial muscle involvement in FSHD and to provide new insights for improvement of speech and language therapy.

  • Received August 8, 2018.
  • Accepted in final form October 25, 2018.
  • © 2019 American Academy of Neurology

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