LOKESWARİ BALLEDA, SRAVANİ KOLLA, CHANDRA SEKHARA REDDY THİMMAPURAM
The Journal of Pediatric Research - 2025;12(2):108-113
Acute necrotizing encephalopathy of childhood (ANEC) is a rare, rapidly progressive neurological disorder predominantly affecting children. It is often triggered by viral infections such as influenza. Dengue virus may also be a trigger. The hallmark of ANEC is cytokine storm-induced inflammation, leading to symmetrical necrotizing lesions in the thalami, brainstem, and cerebellum. Tocilizumab, an anti-interleukin 6 (IL-6) receptor antibody, has emerged as a promising therapy for cytokine-mediated damage. Here, we present 2 cases of dengue/flavivirus encephalopathy, “with magnetic resonance imaging findings suggestive of the classic features of ANEC”, the symmetric bilateral lesions affecting the thalami. Two previously healthy children presented with acute febrile illness and neurological symptoms, requiring admission to a paediatric intensive care unit. The first case was a 5-year-old girl with a 3-day history of high fever, followed by seizures for 2 days and subsequent unconsciousness. Upon admission, she was critically ill, unconscious with a Glasgow Coma Scale score of E1M2V1, and in respiratory failure. Her acute necrotizing encephalitis severity score (ANE-SS) was 7/9. She was managed with ventilatory support, intravenous (IV) fluids, IV antibiotics, and other supportive care. The second case involved an 8-year-old boy with a 2-day history of fever, accompanied by headache, vomiting, and drowsiness. On admission, he was lethargic and drowsy, showing signs of meningeal irritation. His ANE-SS was 4/9. Both cases were treated with tocilizumab, an IL-6 receptor inhibitor, as a single dose of 12 mg/kg infusion over 1 hour, along with antiepileptics, corticosteroids, IV immunoglobulins, and other supportive management. Both patients recovered dramatically. Repeat neuroimaging on day 5 showed significant reductions in the sizes and severities of the lesions in both cases. The efficacy of tocilizumab in these two cases highlights its potential as a targeted therapy for cytokine-mediated neurological damage in ANEC.
