Esophageal electromyography in scleroderma patients with functional dysphagia.

Simultaneous recordings of myoelectric and manometric activities of the esophagus were made in two groups of patients with scleroderma. Group A consisted of patients who suffered from functional dysphagia that had appeared recently, and who had a normal size esophagus when examined radiographically. Patients in group B had complained of functional dysphagia for several years and showed a dilated hypotonic esophagus on x-ray. A control group of five normal subjects was also examined in the same manner. The study was carried out by means of a peroral probe with two pairs of suction-needle electrodes and two manometric side-hole catheters positioned at the same levels as the electrodes. Standard manometric examination of the esophagus was carried out in all patients. Normal subjects after each deglutition showed a propagated burst of spikes in correspondence with the ascending phase of the peristaltic wave, whereas, in the period between deglutitions, rare spikes and no pressure waves were recorded. Group A patients were characterized by the frequent appearance during the interdeglutitive period of spontaneous rhythmic sequences or bursts of spikes associated with pressure waves. In these patients, repetitive nonpropagated spike bursts with a higher than normal amplitude and duration were observed after deglutitions in association with high-amplitude nonpropagated repetitive pressure waves similar to those observed in diffuse esophageal spasm (DES). Five of the seven group B patients showed spike bursts and pressure waves with both amplitude and duration markedly lower than normal. The remaining two patients from group B presented no spike bursts or pressure waves at all. Standard manometry demonstrated findings equivalent to those obtained via electromyography (EMG) in all patients of group B and in only two patients of group A. In conclusion, the functional dysphagia of patients with scleroderma can be attributed to two different motor disorders. The first one is characterized by disorganized myoelectric hyperactivity and may have a manometric appearance similar to that of diffuse spasm. The second one is characterized by a marked decrease in myoelectric activity and corresponds to the classic manometric finding of scleroderma involvement of the esophagus. Both these myoelectric patterns seem to be related to subsequent stages of esophageal scleroderma involvement.