O3209: EBV Infection Presenting with Concurrent Encephalitis and GBS in an Immunocompetent Adolescent

Introduction: Neurological manifestations of Ebstein-Barr virus (EBV) infection include encephalitis, aseptic meningitis, transverse myelitis and Guillain-Barré syndrome (GBS). These may occur with or without symptoms of infectious mononucleosis. Here we present a rare case of EBV resulting in encephalitis with polyneuropathy, a phenomenon that has not been well reported.

Case Description: A 16-year-old girl presented to the emergency room with 1-day of abdominal pain, headache, and vomiting. She had symptoms of an upper respiratory infection about 3 weeks earlier. Over the next 6 hours she became obtunded and then had a brief seizure. Her examination revealed minimal response to pain, hypotonia, and absent deep tendon reflexes in all extremities, as well as absent movement in her left upper and lower extremities. Cerebrospinal fluid opening pressure was 33 cm H2O, protein was 2,046 mg/dL, glucose was normal, and WBC count was 33 cells/µL. EEG revealed severe diffuse encephalopathy and subclinical seizures. MRI brain and spine revealed leptomeningeal and cauda equina enhancement. Broad-spectrum antimicrobials, acyclovir and levetiracetam were started. An extensive work up, including leptomeningeal biopsy, was done to evaluate for infectious, autoimmune and oncological causes. Empiric steroids were initiated for possible central nervous system hemophagocytic lymphohistiocytosis and she received IVIG for possible GBS. Her work up was negative except for positive EBV PCR in CSF, serological evidence of active EBV infection and FDG uptake in the posterior nasopharynx, tonsils and multiple cervical lymph nodes on PET scan. The patient's mental status returned to baseline, but she had persistent quadriplegia with no improvement following IVIG. Plasma exchange was done. Electromyography about 7 weeks after presentation confirmed severe axon loss in a generalized subacute sensorimotor polyneuropathy pattern. The patient was admitted to a rehabilitation center and showed mild improvement of her motor and sensory function about 3 months after presentation.

Discussion: Concurrent presentation of encephalitis and GBS is rare and EBV should be considered as a possible etiology. A combination of serologic and molecular techniques should be used to make the diagnosis of EBV. Treatment should not be delayed while awaiting results.

Conclusion: Encephalitis and GBS secondary to EBV is a rare presentation, but clinicians should keep it on their differential as early management is key.