Acute Necrotizing Encephalopathy of Childhood: A Report of Two Cases

Acute necrotizing encephalopathy of childhood (ANEC) is a rare, severe disorder in previously healthy infants and children, associated with high morbidity and mortality. Early recognition is essential for timely management. We report two pediatric cases. A three-month-old female infant presented with hypotonia, feeding refusal, and lethargy; MRI revealed bilateral, symmetrical thalamic lesions with edema. A four-year-old female developed status epilepticus following a febrile illness; imaging showed diffuse, asymmetrical cortical and subcortical lesions, including hemorrhagic changes in the thalami and brainstem. Both patients received aggressive supportive care, immunomodulation with corticosteroids, and empiric antimicrobial therapy. The second patient also required intensive management for status epilepticus. ANEC presents with nonspecific symptoms such as fever, seizures, and altered consciousness. MRI is crucial for diagnosis, demonstrating characteristic lesions that may vary in distribution and severity. Early neuroimaging, prompt supportive care, and awareness of lesion variability are essential for accurate diagnosis and optimizing outcomes in children with ANEC.