ESOPHAGEAL MOTOR FUNCTION IN PATIENTS WITH MYOTONIC-DYSTROPHY

To investigate pharyngeal and esophageal motor function in myotonic dy strophy (MD), and its relationship to esophageal symptoms, we used low -compliance, high-fidelity esophageal manometry and videofluorography to evaluate 14 consecutive MD patients. Patients exhibited a consisten t, typical motor pattern, involving a marked reduction in resting tone of both the upper and lower esophageal sphincters, and a reduction in contraction pressure in the pharynx and throughout the esophagus, Rad iology showed hypotonic pharynx with stasis and a hypo- or amotile, of ten dilated, esophagus. These findings were nonspecific, however, bein g present in patients both with and without dysphagia, which suggests that MD patients have valid compensatory mechanisms. Dysphagia only co rrelated to the pharyngeal impairment at manometry. Furthermore, the r esults of our study suggest that not only the proximal, striated part of the gullet, but also the distal part (in which smooth muscle domina tes) is involved in the disease. The latter leads to the impairment of the LES resting tone and competence, highlighting the risk of gastroe sophageal reflux disease in these patients.