PP08.5 – 2322: Acute necrotizing encephalitis of children (ANEC)

Abstract

Objective Acute necrotizing encephalitis is a catastrophic disease presenting as encephalopathy and complicated by motor and cognitive disability. Liver affection is a common association. MRI is diagnostic showing bilateral thalamic involvement and differentiates ANEC from other viral and autoimmune encephalitis. This work aims at analyzing outcome of this condition in relation to neuron-imaging. Methods This study comprises 3 boys and 2 girls, aged between 10 and 22 months. They presented in emergency room with fever and disturbed sensorium. MRI, EEG, CFS analysis, liver function and coagulation profile were done for all cases. Results Seizures were present in three cases two focal and one generalized. Hypotonia and sluggish tendon reflexes were evident in four cases with inability to sit or stand. Bulbar manifestations were present in two cases, one case had asymmetrical facial palsy and one had ophthalmoplagia. MRI showed bilateral symmetrical thalamic necrosis in all cases. Subcortical white matter lesions in parasagital and parietal areas were present in four cases and brain stem and tegmentem signals in two. Elevated ALT and prolonged prothrombin time were found in all cases. CSF showed increased proteins in four cases. EEG in the two comatose cases showed hypoactivity and featureless background. One case recovered completely after hydrocortisone therapy. Two cases with brain stem and white matter lesion developed psychomotor retardation and were assigned on intervention program. The two cases showing hemorrhagic necrosis in thalami died in ICU. Conclusion Despite thalamic necrosis in all ANEC cases, those with hemorrhagic necrosis have the worst prognosis. Brain stem and white matter lesions are associated with neurological dysfunction and intellectual retardation.