Acute necrotizing encephalopathy - A diagnosis of Doom". A report on two cases.

Background: Acute necrotizing encephalopathy (ANE) is a rare subtype of post infectious encephalopathy generally occurring in children below the age of five. Adult cases are rare and carry guarded prognosis. CSF profile showing increased proteins and MRI brain showing multifocal, symmetric brain lesions affecting bilateral thalami, cerebral periventricular white matter, brainstem tegmentum, or cerebellum are the characteristic features. Case: Case 1: 50-year-old gentleman developed progressive altered sensorium with right sided weakness and tremulousness. Within few days he developed acute onset severe bradycardia, hypotension bulbar dysfunction and quadriparesis. MRI showed extensive brain stem and diencephalic lesions with hemorrhages indicating necrotizing encephalopathy. CSF showed elevated protein with lymphocytic pleocytosis. He was treated with Intravenous immunoglobulins and methylprednisolone with no significant improvement and succumbed to hospital acquired infection. Case 2: 30-year-old gentleman with no previous comorbidities, presented with high-grade fever and altered sensorium. The initial MRI brain was normal. CSF analysis showed high-protein, low glucose with normal cells .Blood culture was positive for Staphylococcus hominis. He was treated as bacterial meningitis with antibiotics and steroids. His sensorium suddenly deteriorated during hospital stay and became deeply comatose. A repeat MRI showed brainstem and thalamic lesions with hemorrhagic changes suggestive of necrotizing encephalopathy. He was managed conservatively with methylprednisolone. He developed severe bradycardia progressing to asystole from which he could not be revived. Conclusion Characteristic MRI findings help diagnose necrotizing encephalopathy early. It helps patient management and prognostication, but improved treatment strategies are required.