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Journal of Neurology

, Volume 260, Issue 5, pp 1295–1303 | Cite as

Oral muscles are progressively affected in Duchenne muscular dystrophy: implications for dysphagia treatment

  • Lenie van den Engel-Hoek
  • Corrie E. Erasmus
  • Jan C. M. Hendriks
  • Alexander C. H. Geurts
  • Willemijn M. Klein
  • Sigrid Pillen
  • Lilian T. Sie
  • Bert J. M. de Swart
  • Imelda J. M. de Groot
Original Communication

Abstract

Dysphagia is reported in advanced stages of Duchenne muscular dystrophy (DMD). The population of DMD is changing due to an increasing survival. We aimed to describe the dysphagia in consecutive stages and to assess the underlying mechanisms of dysphagia in DMD, in order to develop mechanism based recommendations for safe swallowing. In this cross-sectional study, participants were divided into: early and late ambulatory stage (AS, n = 6), early non-ambulatory stage (ENAS, n = 7), and late non-ambulatory stage (LNAS, n = 11). Quantitative oral muscle ultrasound was performed to quantify echo intensity. Swallowing was assessed with a video fluoroscopic swallow study, surface electromyography (sEMG) of the submental muscle group and tongue pressure. Differences in outcome parameters among the three DMD stages were tested with analysis of variance. Oral muscles related to swallowing were progressively affected, starting in the AS with the geniohyoid muscle. Tongue (pseudo) hypertrophy was found in 70 % of patients in the ENAS and LNAS. Oral phase problems and post-swallow residue were observed, mostly in the LNAS with solid food. sEMG and tongue pressure data of swallowing solid food revealed the lowest sEMG amplitude, the longest duration and lowest tongue pressure in the LNAS. In case of swallowing problems in DMD, based on the disturbed mechanisms of swallowing, it is suggested to (1) adjust meals in terms of less solid food, and (2) drink water after meals to clear the oropharyngeal area.

Keywords

Duchenne muscular dystrophy Dysphagia Oral muscles Ultrasound Video fluoroscopic swallow study Surface electromyography 

Notes

Acknowledgments

This work was funded by the Duchenne Parent Project (The Netherlands). The authors wish to thank the patients and their parents for participating in the study. The authors also wish to thank Karen van Hulst, MSc (Radboud University Medical Centre, Nijmegen, The Netherlands, Department of Rehabilitation) for analyzing the VFSS.

Conflicts of interest

The authors declare that they have no conflict of interest.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2012

Authors and Affiliations

  • Lenie van den Engel-Hoek
    • 1
  • Corrie E. Erasmus
    • 2
  • Jan C. M. Hendriks
    • 3
  • Alexander C. H. Geurts
    • 1
  • Willemijn M. Klein
    • 4
  • Sigrid Pillen
    • 2
  • Lilian T. Sie
    • 2
  • Bert J. M. de Swart
    • 1
  • Imelda J. M. de Groot
    • 1
  1. 1.Department of Rehabilitation, Nijmegen Centre for Evidence Based PracticeRadboud University Medical Centre, NijmegenNijmegenThe Netherlands
  2. 2.Department of Pediatric NeurologyRadboud University Medical Centre, NijmegenNijmegenThe Netherlands
  3. 3.Department of Epidemiology, Biostatistics and HTARadboud University Medical Centre, NijmegenNijmegenThe Netherlands
  4. 4.Department of RadiologyRadboud University Medical Centre, NijmegenNijmegenThe Netherlands

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