Electrophysiological evaluation of oropharyngeal swallowing in myotonic dystrophy

Objective-Oropharyngeal dysphagia is a common feature of patients with myot onic dystrophy and is not usually perceived due to their emotional deficits and lack of interest. The aim was to show the existence and frequency of s ubclinical electrophysiological abnormalities in oropharyngeal swallowing a nd to clarify the mechanisms of dysphagia in myotonic dystrophy. Methods-Eighteen patients with myotonic dystrophy were examined for orophar yngeal phase of swallowing by clinical and electrophysiological methods. Te n patients had dysphagia whereas 11 patients had signs and symptoms reflect ing CNS involvement. Four patients with myotonia congenita and 30 healthy v olunteers served as controls. Laryngeal movements were detected by means of a piezoelectric sensor. EMG activities of the submental muscle (SM-EMG) an d needle EMG of the cricopharyngeal muscle of the upper eosophageal sphinct er (CP-EMG) were also recorded during swallowing. Results-In about 70% of the patients with myotonic dystrophy, the existence of oropharyngeal dysphagia was indicated objectively by means of the techn ique of "dysphagia limit" and by clinical evaluation. Duration of the swall owing reflex as defined by the laryngeal relocation time (0-2 time interval ) and submental muscle excitation as a part of the swallowing reflex (A-C i nterval) were significantly prolonged in patients with myotonic dystrophy, especially in dysphagic patients. Triggering time of the swallowing reflex (A-0 interval) also showed significant prolongation, especially in the pati ents having both dysphagia and CNS involvement. During swallowing, CP muscl e activity was abnormal in 40% of the patients with myotonic dystrophy. Conclusion-Both myopathic weakness and myotonia encountered in oropharyngea l muscles play an important part in the oral and the pharyngeal phases of s wallowing dysfunction in myotonic dystrophy. It was also suggested that CNS involvement might contribute to the delay of the triggering of the swallow ing reflex and some abnormal EMG findings in the CP sphincter, resulting in oropharyngeal dysphagia in myotonic dystrophy.