The Unfortunate Fate of an Altered State: EBV CNS Infection in an Immunocompetent Patient

Introduction: Epstein Barr virus (EBV) is highly prevalent infecting more than 90% of the human population worldwide. CNS involvement, however, occurs only in 0.5%-7.5% of all EBV infections. EBV is known to promote proliferative disorders and contribute to occurrence of neurological diseases like acute cerebellar ataxia, meningitis, encephalitis, and certain brain tumors most often in the immunocompromised. We present a case of EBV CNS infection in an immunocompetent adult.


Description: A 63 year-old female with history of uterine sarcoma with resection, seizures and anemia presented to the emergency room for altered mentation due to a seizure. On arrival she was postictal, hypotensive, tachycardic, and afebrile with no nuchal rigidity. CT head/neck and MRI brain imaging were negative for acute abnormalities. Laboratory analysis revealed leukocytosis, metabolic acidosis, and was negative for HIV infection or evidence of immunocompromise. Blood cultures were drawn and empiric antibiotics with intravenous fluids were started. Despite IV fluids, vasopressors were needed leading to ICU admission. Despite seizure control during hospital stay she continued with fluctuating mentation. As leukocytosis improved and repeat blood cultures resulted negative, antibiotics were discontinued; she was weaned off vasopressors and started on corticosteroids. Given worsening mentation and repeat normal neuroimaging, lumbar puncture was done. CSF flow cytometry was negative for lymphoproliferative disorder; cultures were negative for bacterial or fungal pathogens and immunofluorescence was negative for paraneoplastic antibodies. PCR analysis detected EBV viral DNA. As she showed little neurological improvement her family opted for hospice.


Discussion: This case reinforces the utility of PCR test on CSF samples to diagnose EBV encephalitis. Given that neuroimaging is normal in most EBV associated CNS infections, MRI imaging is still recommended to rule out other causes of CNS infection. The literature describes treatment protocols including antivirals, corticosteroids, and monoclonal antibodies, however, given the rarity of presentation, treatment standardization is lacking. We encourage a high index of suspicion for EBV CNS infection in immunocompetent patients presenting with encephalopathy and normal neuroimaging.