Subacute sclerosing panencephalitis with prominent neuro-ophthalmological manifestation at onset

Abstract

Subacute sclerosing panencephalitis (SSPE) is a rare disease caused by the measles virus, which gives rise to clinical symptoms between two and 20 years after the primary infection. The course of SSPE is generally fatal and includes progressive intellectual and behavioral decline followed by relentless neurological deterioration. No treatment is currently available.We report the case of a seven-year-old boy presenting with nausea and decreased visual acuity, showing a clinical evolution consistent with SSPE. The interval between the first symptoms and death was less than one year.At autopsy, findings consistent with viral encephalitis and the presence of intranuclear inclusion bodies in the brainstem ganglions supported the diagnosis of SSPE.