Introduction Acute necrotizing encephalopathy of childhood (ANEC) is a rare and severe neurological disorder that primarily affects children, characterized by fever, altered consciousness, and seizures. Its etiology and pathogenesis remain poorly understood, and early recognition is crucial for prompt management.

Objectives To emphasize the significance of early diagnosis and management of ANEC, especially in the context of a Low- and Middle-Income Country (LMIC), given the unfavorable outcomes associated with ANEC.

Methods A retrospective chart review of six pediatric patients diagnosed with ANEC at the Aga Khan University Hospital in Karachi were enrolled. The study analyzed clinical, radiological findings, underlying etiologies, treatment approaches, and its outcomes.

Results The cases in this series exhibited varying clinical presentations, including fever, seizures, and altered consciousness. Neuroimaging consistently revealed bilateral thalamic lesions, a hallmark of ANEC, which were seen in 83% of the patients and 33% of the patients showed white matter involvement. All patients received both intravenous immunoglobulin (IVIG) and Steroids along with different combinations of therapies such as antibiotics, antiviral medications, and supportive care. As for the outcomes, 4 patients (67%) successfully recovered and discharged home while two patient died with mortality rate (33%) was determined.

Conclusion Early recognition and tailored treatment, including IVIG and steroids, can significantly impact patient outcomes. The association between ANEC and viral infections, including SARS-CoV-2, raises questions about its potential triggers. Further research and international collaboration are essential to deepen our understanding of this devastating condition and its outcome.

Prem Chand
the Aga Khan University
Pakistan

Abdullah Bin Faisal
The Aga Khan University
Pakistan

Durray Shahwar

Aliyan Jan

Jay Kumari