Dilshan Wimalasena¹, Dilshan Wimalasena¹, Nimasha Wimalaguna¹, Prasadi Walpola¹, Udari Ruvanpathirana¹, Shakila Dharmasiri¹, Thilina Munasinghe¹
1- National Hospital Galle, Sri Lanka
Abstract Body
Introduction: Acute necrotizing encephalopathy of childhood (ANEC) is a rare, rapidly progressive encephalopathy characterized by symmetric necrotic lesions in the thalami and other deep brain structures and Influenza virus is the commonest etiology . It is believed to result from a cytokine storm causing blood–brain barrier disruption and secondary neuronal injury .
Case presentation: We describe three children diagnosed with influenza A–associated ANECduring a seasonal outbreak in Southern SL. All three presented with acute febrile illness, followed by seizures and altered consciousness within 24–72 hours. Case 1: A 1-year-old girl developed refractory seizures and dystoni a requiring mechanical ventilation, high-dose methylprednisolone (IV MPNL), intravenous immunoglobulin (IVIG), and Tocilizumab. She had significant motor dysfunction requiring prolonged rehabilitation, to which she showed a dramatic response. Case 2: A 5-year-old girl developed early encephalopathy with thalamic and dentatehypodensities on neuroimaging. She showed rapid clinical improvement after IV MPNL and IVIG. Her residual truncal ataxia and right lower limb spasticity significantly improved with an intense rehabilitation programme. Case 3:A 6-year-old girl presented with seizures and posturing, and the MRI Brain showedclassic bilateral thalamic lesions. She was initially given IV MPNL and IVIG. As she had a very poor response to the initial immune therapy combination, her anagement was escalated to 7 cycles of plasma exchange followed by IV Tocilizumab. Then she had intense inward rehabilitation programme resulting in improving her functional outcomes.
Conclusion: Timely identification, rapid immunomodulatory treatment and intense rehabilitation results in better outcomes of ANEC.