Assessment of esophageal function in patients with myasthenia gravis

Abstract.

Background: Myasthenia gravis (MG) is an autoimmune disease in which impairment of neuromuscular transmission results in a pathological fatigability of striated muscles. Dysphagia is a common symptom in MG. It is caused by a weakness of the striated muscles in the pharynx and esophagus. The purpose of our study was to evaluate the role of esophageal scintigraphy in the assessment of esophageal function in MG. Methods: In 15 patients with clinically proven MG (oculopharyngeal manifestation in 6/15 patients, generalized weakness in 9/15 patients) esophageal transit was investigated scintigraphically with a multiple swallow test protocol. 10/15 patients had a history of dysphagia. Patients were studied twice: under baseline conditions, and immediately after pharmacological stimulation with 10 mg of edrophonium chloride (EC), a short-acting acetylcholinesterase inhibitor. Results: Under baseline conditions all patients showed an impaired esophageal function (emptying [%]= 58 % ± 21; normal range > 85 %). In 14/15 individuals esophageal transit improved after administration of EC (emptying [%]= 75 % ± 18; p < 0.01), reaching the normal range in 6 patients. One patient showed no effect attributable to EC. Conclusions: Esophageal transit is often compromised in MG. Functional abnormalities may be also present in patients without a history of dysphagia. Inhibition of cholinesterase positively affects striated muscles in the pharynx and upper esophagus, thus improving esophageal transit. Esophageal scintigraphy may be considered as a simple, non-invasive method for diagnosing impairment of esophageal function in MG and to monitor the changes under pharmacological stimulation.