The Clinical Characteristics and Prognosis of Children Presenting with New Onset Refractory Status Epilepticus in COVID-19-related Multisystem Inflammatory Syndrome

Introduction: MIS-C is a hyperinflammatory process leading to multiorgan failure and shock, occurring during the acute or post-infectious stage of SARS-CoV-2, and has 2 subtypes: para-infectious and post-infectious varieties. The new onset of refractory status epilepticus has rarely been described as the presenting feature of MIS-C. Methods: This retrospective study, conducted at Dr. B. C. Roy Post Graduate Institute of Pediatric Sciences, included children hospitalised between August 1, 2020 and July 31, 2021, with NORSE and subsequently diagnosed to have MIS-C. Their clinico-demographic variables, treatment courses during hospital stays, laboratory reports, radiological and electrophysiological findings, and outcomes at discharge and follow-up over 1 year were recorded. At their 12-month visits, their motor disabilities (primary) and continuation of anti-epileptic drugs, and persistence of MRI brain abnormalities (secondary) were the outcome measures. The characteristics of the patients in the para-infectious and post-infectious groups were compared using the Mann-Whitney U test for continuous variables and the Chi-square test for categorical variables. Results: There were 8 and 10 patients in groups A and B, respectively. Patients in group B had significantly higher age, more prolonged RSE, use of anaesthetics and ventilation, and longer PICU stay, while other clinical and laboratory parameters and short and long-term outcomes were not significantly different between the two groups. 8 patients developed hemiparesis, while 2 had quadriparesis in the acute stage, but 15 (83%) patients had complete recovery from their motor deficits by 1 year. At 1-year follow-up, 33% and 39% of patients, had abnormal MRI and EEG finding, respectively. Conclusion: The post-infectious group had significantly higher age, longer duration of RSE, ventilation, use of general anaesthetics, and PICU stay. Acute disseminated encephalitis and acute leucoencephalopathy were the most commonly observed MRI abnormalities in the acute phase, with prolonged persistence of cerebritis in patients in the post-infectious group, warranting long-term immunomodulation. Combined immunotherapy with IVIG and steroids was effective in the acute phase. However, long-term anti-epileptic therapy was needed in both groups.