Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Epstein-Barr virus-associated encephalitis presenting as refractory status epilepticus that persisted for nearly 2 months with exellent recovery

Makoto Nishie, M.D., Masaya Ogawa, M.D. and Kozo Kurahashi, M.D.

Department of Neurology, Aomori Prefectural Central Hospital

A 37-year-old woman presented with Epstein-Barr virus (EBV) -associated encephalitis that developed into refractory status epilepticus ten days after the onset of headache and fever, without signs suggestive of infectious mononucleosis. An electroencephalogram showed definite epileptogenic changes, including diffuse slow wave bursts with paroxysmal generalized bilateral sharp waves. The patient required general anesthesia for nearly two months, but had completely improved 18 months later. The patient developed Klüver-Bucy syndrome four months after the onset: bilateral frontal hypoperfusion was detected with SPECT at this time, but also improved after 18 months. MRI showed a 2∼3 mm lesion of the cerebellar white matter, which was suggestive of a small demyelinaed focus. The patient's serum was positive for EBV DNA within two weeks of onset, but negative thereafter. However, the CSF was positive for EBV DNA for more than five months, with a four-fold increase in the titers of IgG antibody for EBV-viral capside antigen in the CSF. Given the patient's good recovery from her critical condition, her severe encephalitis-/encephalopathy with persistent status epilepticus probably resulted from an EBV-associated immune-response after the reactivation of EBV, rather than from a direct infiltration of EBV into the brain.

(CLINICA NEUROL, 43: 422|426, 2003)
key words: Epstein-Barr virus, status epilepticus, viral encephalitis, EBV DNA in the CSF, Polymerase chain reaciton, EBV reactivation

(Received: 1-Apr-03)