MANOMETRIC CHARACTERISTICS OF THE PHARYNX, UPPER ESOPHAGEAL SPHINCTER, ESOPHAGUS, AND LOWER ESOPHAGEAL SPHINCTER IN PATIENTS WITH OCULOPHARYNGEAL MUSCULAR-DYSTROPHY

Improved techniques in esophageal manometry have made this test an attractive option for investigating pharyngeal or esophageal disorders in patients with dysphagia. We studied esophageal as well as upper esophageal sphincter/pharyngeal (UES/P) pressure dynamics in 11 patients with an established diagnosis of oculopharyngeal muscular dystrophy with modern solid-state manometric techniques and then compared manometric and clinical findings. Esophageal manometric abnormalities were found in 10/11 patients, with the most common being simultaneous contractions and incomplete lower esophageal relaxation. 9/11 patients showed abnormal UES/P manometrics, with the most common abnormalities found in the pharynx. The presence of manometric abnormalities closely paralleled clinical assessment of degree of disease severity. Modern manometric techniques offer an opportunity for a quantitative assessment of swallow abnormalities.