Brainstem lesions due to granular ependymitis in symptomatic diffuse esophageal spasm: a case report

An 80 year-old man had symptomatic diffuse esophageal spasms for a few months. They were documented by radiographic, endoscopic and manometric findings. The post-mortem neuropathological examination showed a granular ependymitis of the fourth ventricle involving the dorsal region of the motor dorsal nuclei of the vagus nerve which showed frank neuronal loss. The muscular wall of the esophagus, its myenteric plexus and the fasciculi of the vagus nerve were histologically normal. This is the first published case of such a brainstem lesion associated with symptomatic diffuse esophageal spasms. These pathological data are compared with those already published on achalasia.