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Dysphagia in Myotonic Dystrophy

  • Sonoko NozakiEmail author
Chapter
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Abstract

In myotonic dystrophy type 1, all stages in the five swallowing model are disturbed.

Deglutition-related muscle weakness, as opposed to myotonia, was the most significant contributor to impairment.

In anticipatory stage, some patients show poor awareness of dysphagia. Aberrations of feeding behaviors and silent aspiration have been reported.

In oral preparatory and propulsive stage, force of masticatory and labia oris is degraded, but patients sometimes swallow foods without adequate chewing. Myotonic phenomena are seen in the circumferential musculature of the buccal capsule.

In pharyngeal stage, decreased pharyngeal peristalsis, delayed deglutition reflex, and epiglottis dysraphic are observed. The threshold of the cough reflex is high, and this is related to silent aspiration.

In esophageal stage, degradation of esophagus peristalsis and gastroesophageal reflux are present.

Myotonic phenomena reduce with hyperthermia treatment on the masseter muscle, and that induces easier mouth opening. A warm-up phenomenon is seen with repeated opening and closing of the mouth.

Dilatation of upper esophageal sphincter using balloon catheter is effective.

When drop in SpO2 is revealed during meals due to respiratory disturbance, ventilation is desirable while eating.

During percutaneous endoscopic gastrostomy, we need to keep watching for aggravation of respiratory insufficiency.

Keywords

Myotonic dystrophy Dysphagia Myotonia Silent aspiration Feeding behavior Hyperthermia treatment Respiratory failure 

Notes

Acknowledgment

We wish to thank the Ashiya Municipal Hospital and Toneyama National Hospital for providing valuable cases.

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Copyright information

© Springer Nature Singapore Pte Ltd. 2018

Authors and Affiliations

  1. 1.Department of Neurology and RehabilitationKansai Rosai HospitalAmagasakiJapan

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