Encephalomyeloneuropathy with ganglionitis of the myenteric plexuses in the absence of cancer.

A 55-year-old woman presented with rapidly progressive brainstem dysfunction which led to death within a month. She also had constipation for three weeks, and barium enema showed ileus. Subacute encephalomyelitis predominantly involving the medulla and pons correlated with the patient's initial symptoms. In addition, ganglionitis of the myenteric plexuses explained the constipation and ileus. Ganglioradiculoneuropathy was another finding. The presence of abundant neuronophagia in the brainstem, dorsal root ganglia, and myenteric plexuses raised the speculation that a putative virus, toxic agent, or immune reaction possessed special affinity for neurons and ganglion cells. The neuropathological findings were similar to paraneoplastic changes, but no neoplasm was found.