Dysphagia in duchenne muscular dystrophy versus myotonic dystrophy type 1

摘要

Introduction: In this study we aimed to demonstrate the distinctive features of dysphagia in patients with myotonic dystrophy type 1 (DM1) and Duchenne muscular dystrophy (DMD). Methods: Diet-type score (DTS) and maximum tongue pressure (MTP) were measured in 20 DM1 and 24 DMD patients; all patients were also examined by videofluoroscopy (VF). We used VF to measure the range of hyoid bone excursion (RHBE) during pharyngeal transit time and the area of pharyngeal residue (APR) after the first swallow. Results: RHBE and APR values for DM1 patients were significantly greater than those for DMD patients. DTS and MTP did not differ between patients. A significant correlation was observed between DTS, MTP, and RHBE in DMD patients, but not in DM1 patients. Conclusions: Our findings reveal the differences in the distinctive features of dysphagia in DM1 and DMD. Adjustments in the diet of DMD patients in accordance with swallowing ability could be useful.