Proposing A New Treatment Model: Early Initiation and Eeg Based Escalation of Immunotherapy In Children With Norse/fires

Objectives It is postulated autoimmune or immune mediated etiopathogenesis as the root cause of NORSE/FIRES. The observations with our cohort of children with NORSE we are proposing a new model of treatment depicting early initiation and EEG based rapidly escalation of immunotherapy.

Methods The study duration of study is from June 2019 to March 2023.During study duration out of 31 children with NORSE, 18 children were treated as per this proposed new treatment model. Their clinical, biochemical, radiological, electrophysiological and treatment details were recorded with short term neurological follow-up

Results 18 children(11 boys,8 girls) with NORSE/FIRES admitted during study period were treated with newer protocol. Their mean age was 7.4 years. History of preceding or current febrile illness was seen in 6 (33%) patients. None of 18 were positive for HSV PCR or CSF anti NMDA antibody. All 18 children had abnormal EEG with 7(38%) of them had evidence of electrical status epilepticus. All the patients were started on immunotherapy (methyl prednisolone and IV IG) within 72 hours of admission. Escalation of immunotherapy was based on EEG findings (scoring based mainly on background activity) and clinical profile. median duration for termination seizures was 8 days. No mortality was recorded and only 5(28%) children were mechanically ventilated. Out of 18 ,8(45%) children have epilepsy in follow-up.

Conclusion: This study highlighted EEG's role as biomarker of neuroinflammation in children with NORSE. Early to very early initiation of immunotherapy and EEG based escalation of the same has given superior results in our cohort.