えん下障害を呈したミトコンドリアミオパチー２例のえん下動態―消化管内圧計を用いて

Abstract

We evaluated a swallowing pressure in two cases of mitochondrial myopathy of oculopharyngeal muscle atrophy type (case 1) and facio-scapulo-humeral atrophy type (case 2). A catheter with three diode transducers each 5 cm apart was swallowed through the nose into the upper esophagus. Firstly a patient was requested to drink 1 ml of water at about five-second intervals, and swallowing pressures were recorded under the condition that the speed of paper recording and catheter pull-through was the same 1 mm/sec. Secondly swallowing pressures were recorded under the condition that the middle transducer was fixed at the level of the upper esophageal sphincter and the speed of paper recording was 5 mm/sec. Thirdly resting pressure of the upper esophageal sphincter was recorded under the same condition of the first recording. The results were as follows: 1) swallowing pressure in the pharynx was low in case 1 and lower in case 2, 2) relaxation of the upper esophageal sphincter was complete in case 2 and incomplete in case 1, 3) resting pressure of the upper esophageal sphincter was rather low in two cases. And the degree of subjective dysphagia was higher in case 1 than case 2. These facts lead to the conclusion that relaxation of the upper esophageal sphincter was more important than peristaltic movement of the pharynx at swallowing.