Esophageal Dysmotility Confirmed by Esophageal Manometry in a Patient with Stiff-Man Syndrome and Dysphagia

Abstract

Stiff-man syndrome (SMS) is a rare autoimmune condition characterized by “tin soldier” rigidity of axial muscles. Nearly 150 cases have been reported with 6 reports of pharyngeal or esophageal dysphagia. We report the first case of esophageal dysmotility documented by esophageal manometry in a patient with SMS and dysphagia. A 37 year old African American male with SMS was referred for progressive regurgitation and dysphagia to solids and liquids since the diagnosis 2 years prior. The dysphagia was less noticeable with thickened liquids or soft foods. The patient denied odynophagia, pyrosis, early satiety or constipation. Esophageal manometry revealed low amplitude contractions to wet swallows with no significant antegrade propulsion. A few secondary peristaltic waves of adequate amplitude were seen. [figure1] The patient was unable to tolerate upper endoscopy. pH-metry with Bravo revealed pathologic reflux on day #2 with regurgitation symptoms strongly correlated with acid reflux events. A barium esophogram showed a normal contour, however motility was not assessed as the procedure was terminated secondary to coughing and aspiration of contrast. A gastric emptying study was normal. Dysphagia and regurgitation did not improve with high dose proton pump inhibitor therapy. As benzodiazepines did not improve symptoms in the past, the patient was treated with steroids and intravenous immune globulin (IVIG), which resulted in transiently improved axial muscular rigidity and dysphagia. SMS is a rare disorder characterized electromyographically by continuous motor discharge. Several cases of dysphagia have been described which were responsive to benzodiazepine therapy. To our knowledge, this is the first report of esophageal manometric findings in a patient with SMS. Our findings suggest not only striated muscle, but also smooth muscle involvement. Smooth muscle involvement has been previously reported in the form of delayed gastric emptying. The lack of acid reflux on day #1 of pH-metry raised a question of possible residual effect from conscious sedation, however the patient's symptoms have not sustained a response to medical therapy.Table