Early Infantile Developmental and Epileptic Encephalopathy: A Catastrophic Complication of COVID-19-Associated Multisystem Inflammatory Syndrome in Neonates

Abstract Multisystem inflammatory syndrome in children (MIS-C) is a severe complication of SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2) infection. While children are commonly affected by MIS-C, reports have described MIS in neonates (MIS-N) following maternal coronavirus disease 2019 (COVID-19) infection. We describe a case of early infantile developmental and epileptic encephalopathy (EIDEE) secondary to MIS-N in a day 18 old neonate secondary to maternal COVID-19. A term-born male neonate with a history of maternal COVID-19 at 35 weeks of gestation presented on day 18 of life with seizures, encephalopathy, pneumonitis, transaminitis, and elevated inflammatory markers. Magnetic resonance imaging (MRI) brain showed extensive cortical laminar necrosis. He was managed for MIS-N with antiseizure medications, antibiotics, and intravenous immunoglobulin. COVID-19 reverse transcriptase-polymerase chain reaction was negative, and anti-COVID immunoglobulin G antibody was positive. Near continuous multifocal myoclonic seizures were associated with a suppression burst pattern in the EEG. Follow-up MRI showed cystic encephalomalacia and loss of periventricular white matter. The EIDEE remained drug refractory with poor neurological outcome in follow-up. Cytotoxin-mediated neuronal injury in MIS-N can lead to a catastrophic complication of EIDEE, resulting in drug-refractory epilepsy, microcephaly, and adverse neurological outcome.