Chronic persistent nodular polioencephalomyelitis presenting with brain atrophy

A 61-year-old male had a recurrent fever (approx. 38 degrees C) and one month later, disorientation and forgetfullness became gradually apparent. His mental and somatic status deteriorated progressively with the recurrence of fever; 3 years later he was in a persistent vegetative state accompanied by status epilepticus, and died after 5 years. Mononuclear cytosis (8-20/microliters) and increased protein (20-70 mg/100 ml) were observed in the cerebro-spinal fluid (CSF) at each examination. There was no increase of antibody titers against various viruses, chlamydia, mycoplasma and toxoplasma in the serum and CSF. Neuropathologically, the prominent microscopic finding was patchy scarred foci consisting of neuronal loss replaced by glial tissue throughout the gray matter. A few gliomesenchymal nodules were found. In addition, rod-shaped macrophages were found to varying degrees in all areas of the brain and spinal cord. No viral inclusion bodies were identified. Electron microscopic examination revealed no viral particles in the affected areas. Suspensions of the frozen brain and CSF were inoculated into primary neuron cultures derived from fetal mice and into mouse brains, but the results were negative. There had been five reported cases of chronic nodular encephalitis up until 1971; this case seems to differ from the others by the presence of persistent inflammation and by neuropathologic findings.