Abstract

Epstein Barr virus (EBV) infection has an incidence of 60 to 100 per 100000 and neurologic complications occur in 1 to 5% of patients. Chronic infection and raised intracranial pressure outwith the context of hydrocephalus have been rarely been reported previously with EBV infection. GATA-2 is a highly conserved transcription factor involved in the genesis and function of haematopoetic and progenitor cells. Mutations are rare although the incidence is not well defined (10% of primary neutropenias and monocytopenias). Persistent EBV viremia has been reported previously in 11% patietns with GATA-2 deficiency. We present the case of a 21-year old woman who presented to a district general hospital with headache and meningeal irritation and bilateral abducence nerve palsy and diagnosed with viral meningitis. Her investigation showed positive EBV serology. She represented with signs and symptoms of raised intracranial pressure and then neutropenic sepsis and was found to have persistent EBV viral load. Investigation revealed a mutation in the GATA2 gene [c. 1091_1096dup p.(Ala364_Asn365dup)] which has not been previously reported. She is awaiting haematopoetic stem cell transplant. To our knowledge there have been no previous reports of EBV meningoencephalitis and subsequent raised intracranial pressure as the presenting syndrome of GATA-2 deficiency.