The clinical, laboratory, etiological profile and outcome of acute necrotising encephalitis of childhood in tertiary care centre from western India

Abstract

Acute necrotising encephalopathy of childhood (ANEC) is an encephalopathy that presents with viral prodrome accompanied by episodes of seizures and rapid alteration of consciousness. Many patients had a previous history of febrile illness before neurological worsening. Though there is no definite genetic cause, few genes are involved in familial forms of the disease. Geographically this disease is seen in East Asian countries involving infantile and childhood age. Radiological evaluation classically shows lesions involving thalami, cerebellum, brainstem, and white matter. We hereby report 4 cases hospitalized at a tertiary health care hospital with classical presentation, hallmark radiographic, and hematological picture. All four patients presented with varied neurological involvement including altered consciousness, status dystonicus, trismus, and status epilepticus following an acute febrile illness. Radiological involvement is classical in all with damage to the thalami, cerebellum, brainstem, and white matter, though the neurological presentation, duration, and outcome of all varied. All these 4 patients were thoroughly investigated and out of them 3 were managed with IVIG and MPS, the rest one with an immunosuppressive state managed symptomatically in a conservative manner, and all four were discharged with varied neurological morbidities. All of them offered symptomatic medical management, and physiotherapy from experts. ANEC is an acute deteriorating neurological disorder with a prior history of febrile illness. Neurological illness has hallmark clinical presentation with classical symmetrical brain involvement including thalami, cerebellum, brainstem, and white matter. Though having a varied clinical spectrum, it has responded well to immunotherapy by anti-inflammatory agents including IVIG and MPS.