Supporting Data doc 3.9MB

Novel treatment approach to NORSE (new onset refractory status epilepticus) in children: Skipping the pharmacological coma

Objectives: NORSE is defined as a clinical condition, with new onset status epilepticus without a clear etiology in a patient without any pre-existing neurological disorder or epilepsy. In majority of the cases of NORSE proven or possible either autoimmune or immune mediated etiopathogenesis is suspected. Our aim of study is to discuss the implications of early and timely escalation of immunotherapy in children with NORSE. Methods: study duration was from january 2019 to january 2022. Total 11 children with NORSE were included and clinical, biochemical, radiological, electrophysiological and treatment details were recorded with short term follow up Results: Out of total 116 children with status epilepticus 11 were presented with NORSE/FIRES. Mean age was 4.7 years. History of preceding or current febrile illness was seen in 4 (36%) patients. All CSF sample were negative for HSV PCR and autoimmune antibody profile. All 11 children had abnormal EEG with 7(64%) of them had evidence of electrical status epilepticus. All the patients were given immunotherapy and it was timely escalated as per combined clinical and EEG findings. The aim is to achieve clinical seizure freedom with normal physiological EEG background activity(unlike burst suppression with pharmacological coma) as per age. Seizure termination was achieved with median duration of 5 days. With this approach 10(91%) children didn’t require ventilation ,none was given pharmacological coma with zero mortality. Conclusion: Early Immunotherapy has beneficial role in management of children with NORSE. Our approach to treatment of NORSE/FIRES in children seems promising but multi-centre study is needed.
Keywords: NORSE,status epilepticus,immunotherapy,pharmacological coma