Anakinra in Severe RSV-Associated Autoinflammatory Encephalopathy With Delayed ADEM-Like Changes

Respiratory syncytial virus (RSV)-associated encephalopathy is an uncommon but potentially devastating complication in children. Increasing evidence suggests that infection-triggered neuroinflammation rather than direct viral invasion underlies neurologic injury in a subset of patients. We describe 2 previously healthy, unrelated children who developed rapidly progressive encephalopathy after RSV infection. Initial brain magnetic resonance imaging scan results were normal, but follow-up imaging results showed multifocal white matter lesions consistent with an acute disseminated encephalomyelitis-like pattern. Both patients were given refractory to high-dose corticosteroids, intravenous immunoglobulin, and interleukin (IL)-6 blockade. Markedly elevated serum IL-1β levels were documented in one patient. Treatment with the IL-1 receptor antagonist, anakinra, was temporally associated with neurologic and radiologic recovery in both cases. These observations suggest that an IL-1–driven autoinflammatory phenotype may contribute to disease severity in selected children with RSV-associated encephalopathy and highlight the importance of repeat neuroimaging and consideration of targeted immunomodulation in refractory cases.