Introduction:Acute necrotizing encephalopathy(ANE),a fatal subtype of infection-triggered encephalopathy syndrome(ITES),can be triggered by many systemic infections.RANBP2 gene mutations were associated with recurrent...Introduction:Acute necrotizing encephalopathy(ANE),a fatal subtype of infection-triggered encephalopathy syndrome(ITES),can be triggered by many systemic infections.RANBP2 gene mutations were associated with recurrent ANE.Case presentation:Here we report a 1-year-old girl with recurrent ITES and RANBP2 mutation.She was diagnosed with influenza-associated encephalopathy and made a full recovery on the first episode.After severe acute respiratory syndrome coronavirus 2 infection,the patient presented with seizures and deteriorating mental status.Brain magnetic resonance imaging revealed necrotic lesions in bilateral thalami and pons.Methylprednisolone,immunoglobulin,and interleukin 6 inhibitors were administered.Her consciousness level was improved at discharge.Nineteen cases of 2019 coronavirus disease-related ANE have been reported,of which 22.2%of patients died and 61.1%had neurologic disabilities.RANBP2 gene mutation was found in five patients,two of whom developed recurrent ITES.Conclusion:Patients with RANBP2 mutations are at risk for recurrent ITES,may develop ANE,and have a poor prognosis after relapse.展开更多
文摘Introduction:Acute necrotizing encephalopathy(ANE),a fatal subtype of infection-triggered encephalopathy syndrome(ITES),can be triggered by many systemic infections.RANBP2 gene mutations were associated with recurrent ANE.Case presentation:Here we report a 1-year-old girl with recurrent ITES and RANBP2 mutation.She was diagnosed with influenza-associated encephalopathy and made a full recovery on the first episode.After severe acute respiratory syndrome coronavirus 2 infection,the patient presented with seizures and deteriorating mental status.Brain magnetic resonance imaging revealed necrotic lesions in bilateral thalami and pons.Methylprednisolone,immunoglobulin,and interleukin 6 inhibitors were administered.Her consciousness level was improved at discharge.Nineteen cases of 2019 coronavirus disease-related ANE have been reported,of which 22.2%of patients died and 61.1%had neurologic disabilities.RANBP2 gene mutation was found in five patients,two of whom developed recurrent ITES.Conclusion:Patients with RANBP2 mutations are at risk for recurrent ITES,may develop ANE,and have a poor prognosis after relapse.
