Esophageal motility disorders in Mexican patioents with Duchennes muscular dystrophy

ABSTRACT

Objetive Myopathies are entities tahat mainly involve strieted muscle. In Duchennes muscular dystrophy (DMD) there have been reported smooth muscle alterations in the pre-oral phase of swallowing, in gastric emptying, and pseudoobstruction. Nevertheless, esophageal motility alterations are not concluding. The objetive of this work was to determine if there are motor esophageal alterations is this patients, and if this alterations are related to the clinical manifestations of disease. Study design: nine consecutive patients with DMD (mean age 8, range 6-11 years; males) were evaluated, comparing clinical and manometric findings. Results: esophageal manometry alterations were found in all patients, mainly simultaneous non-peristaltic waves (60.86 percent) of diminished amplitude, in both striated and smooth muscle. Seventy seven percent presented with upper and lower gastrointestinal symptoms (dysphagia, regurgitation, epigastric pain, constipation, and distention). No correlation was found between esophageal motility alterations and gastrointestinal symptoms, nor with the clinical stage of disease in accordance to Brook (r=0.27). Conclusion: these results show that patients with DMD present esophageal motor disorders in both striated and smooth muscle, as well as upper and lower gastrointestinal symptoms. Specialized motility studies, could yield a better understanding of disease, and, possibly with adequate treatment, provide for a better quality of life in children with DMD. (AU)