An autopsy case of chronic nodular encephalomyelitis simulating neuro-Behçet syndrome.

A 29-year-old man suffered from neurological symptoms such as urinary disturbance, changes in personality, gait and speech disturbances, amnesia, myoclonus, dysphagia and muscle atrophy for nine years with exacerbations and remissions. A postmortem examination revealed many inflammatory and necrotic foci in the brain and spinal cord, especially in the brainstem and cerebral cortex. Numerous minute scar foci were also scattered in the gray matter, accompanied by some demyelinating lesions in the white matter. The presence of microthrombi and plasma exudation into the vessel wall suggested the importance of vascular factors in the pathogenesis of these CNS lesions. Peripheral neuropathy was also present. The neuropathological features were almost the same as those of neuro-Behçet syndrome, but he lacked the extraneural symptoms of Behçet disease.