Subacute Sclerosing Panencephalitis

Why It Matters

This case highlights the critical public-health implications of measles infection and under-vaccination: preventable childhood measles can lead to delayed, fatal neurodegenerative disease years later, stressing the importance of vaccination and surveillance.

Summary

A 7-year-old boy in the U.S. developed three months of cognitive decline and seizures and was found to have signs of diffuse encephalopathy on MRI, periodic high-amplitude discharges on EEG, and markedly elevated measles-specific IgG in cerebrospinal fluid. He had measles as an infant in Afghanistan and was never vaccinated thereafter. Clinical, imaging, EEG, and CSF findings led to a diagnosis of subacute sclerosing panencephalitis (SSPE), a rare but fatal progressive neuroinflammatory disease caused by persistent measles infection. The case underscores the long latency and severe neurologic sequelae of measles infection acquired in early life.

Original Description

Subacute sclerosing panencephalitis (SSPE) is a progressive neuroinflammatory disease associated with persistent measles virus infection and can have a latency of many years. In this video, NEJM Editorial Fellow Katerina Lin, MD, describes a clinical case involving a 7-year-old boy.

Search "Subacute Sclerosing Panencephalitis" at NEJM.org to learn more.

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