An inflammatory axonopathy of the myenteric plexus producing a rapidly progressive intestinal pseudoobstruction.

A previously well 39-yr-old man presented with a 4-wk history of abdominal pain, nausea, vomiting, and weight loss. An upper gastrointestinal examination showed retained food in the stomach and duodenal dilatation. A radioisotope meal showed little gastric emptying; esophageal manometry was normal. Because of persistent symptoms, a duodenojejunostomy was done. However, the patient remained symptomatic and after an episode of profuse vomiting, aspirated and died 10 wk after initial presentation. At autopsy, no tumor was found. Hematoxylin and eosin stains throughout the gastrointestinal tract showed many lymphocytes and plasma cells within the myenteric plexus. Silver stains showed the argyrophilic and argyrophobic neurons to be normal, but axons showed beading, fragmentation, and dropout in all areas. We therefore concluded the following: intestinal pseudoobstruction can be caused by an inflammatory neuropathy of the myenteric plexus, not associated with a distant carcinoma, and this process produced an axonopathy while sparing neuron bodies.