Unusual cause of fever, vision loss and super refractory status epilepticus in association with simian virus 40 (SV40)

Abstract

We present a case of a 23-year-old man with history of fever followed by painless complete vision loss, with subsequent new-onset refractory status epilepticus (NORSE). He initially developed bilateral retinitis. A few days later, he started having focal seizures, and subsequently developed super-refractory status epilepticus, requiring anaesthetic agents. MRI brain revealed multifocal cortical and subcortical hyperintensities in occipital and temporoparietal regions without contrast enhancement. MRI repeated a month later showed new lesions with non-visualisation of some previous lesions. Finally, a brain biopsy was done which revealed presence of lymphocytic infiltrate with SV40 inclusions in oligodendrocyte. We propose the affliction of an atypical virus affecting the retina and brain grey and white matter, presenting with NORSE in our patient. Future similar cases and isolation of the virus may help in establishing the conclusive diagnosis.