[Fulminant cerebello-brainstem encephalitis with polyradiculitis following probable Epstein-Barr virus infection]

Epstein-Barr virus (EBV) infection is occasionally accompanied by acute neurological impairment. The pathogenesis of neurological manifestations with EBV infection consists of primary inflammations of EBV infection, and secondary immunologic reactions. However, their clinical course and prognosis are usually favorable. Here we report a patient with fulminant neurological involvement in association with EBV infection. The patient was a 44-year-old man. One morning he developed ataxic gait and speech following flu-like symptoms. He noticed double vision in the afternoon. He had disturbance of consciousness, bilateral ptosis with mydriasis, opthalmoplegia, facial diplegia, bulbar palsy, and weakness of muscles in extremities and respiratory system on the next day. He required mechanical ventilatory support for a month. His symptoms began to improve gradually two weeks after the onset. Two month later, neurological examinations disclosed severe cerebellar ataxia of the four extremities and ocular movement, cerebellar speech, and moderate weakness in his limbs. Moderate cerebellar ataxia and diminished deep tendon reflexes remained for 8-months. Although he had no physical manifestations of infectious mononucleosis, DNA of EBV was identified in the cerebrospinal fluid (CSF) by the polymerase chain reaction method. From these results, we diagnosed his condition as a cerebello brainstem encephalitis with polyradiculitis associated with EBV infection. The cell counts and protein content of CSF gradually normalized in the early stage of his illness, but CSF protein increased again, and had the peak of 275 mg/dl in about one month. In spite of normalized CSF cell counts, his neurological symptoms persisted. CT scan and MRI studies of the brain and the spinal cord were repeated, but demonstrated no significant abnormalities. Clinical course and CSF findings revealed that his fulminant neurological symptoms were most likely produced by the secondary immunologic reactions following the primary inflammations by EBV infection.