New‐onset refractory status epilepticus as an early manifestation of multisystem inflammatory syndrome in adults after COVID‐19

Abstract

Multisystem inflammatory syndrome in adults (MIS‐A) is a rare hyperinflammatory complication with multi‐organ involvement that manifests a few weeks after recovering from a typically mild coronavirus disease 2019 (COVID‐19) infection. Although encephalopathy and seizures can occur in the acute phase of COVID‐19, the nervous system is infrequently involved in patients with MIS‐A. Herein, we describe the case of a young woman who presented with new‐onset refractory status epilepticus (NORSE) following a mild COVID‐19 infection associated with symptoms, signs, and laboratory findings that satisfy the updated Centers for Disease Control and Prevention (CDC) definition of MIS‐A. Magnetic resonance imaging of the brain revealed symmetric T2‐signal increase involving both orbitofrontal lobes, insulae, and hippocampi. One of the notable findings in our patient was the quick response and significant clinical recovery that occurred following initiation of treatment with intravenous methylprednisolone and intravenous immunoglobulin. Our case expands the clinical spectrum of MIS‐A and documents the occurrence of NORSE as one of its early clinical manifestations. A routine comprehensive clinical and laboratory assessment is needed to screen for this underdiagnosed condition, especially in patients with post‐COVID‐19 inflammatory complications.

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

“…Although NORSE has not been reported as a presenting symptom in MIS-C, it has been reported in adults with multisystem inflammatory syndrome (MIS-A). 9 Nawfal et al 10 reported the first case of NORSE in MIS-A, in a 21-year-old woman with cardiac, gastrointestinal dysfunction, and encephalopathy, occurring 5 weeks after her COVID-19 infection. Leelamani et al 11 reported another young lady with NORSE 2 days after developing mild fever and cough and having a positive nasal swab PCR.…”

Multisystem inflammatory syndrome in adults: Characteristics, treatment, and outcomes

“…16 Finally, systemic capillary leak syndrome, spontaneous coronary artery dissection, severe inflammatory myopathy, cortical unihemispheric brain edema, thyroiditis, refractory status epilepticus, ischemic stroke, and subconjunctival hemorrhage have been rarely reported as clinical manifestations of MIS-A. 28,30,31,[61][62][63] Overall, the clinical features of MIS-A, including fever, gastrointestinal, and cardiovascular involvement, are similar to those of MIS-C. 16,19,53 These findings were consistent with the comparisons between Belay et al 53,60 report about MIS-A and previous surveillance data about MIS-C. Compared to patients with MIS-C (n = 1733) from previously published surveillance data, a higher proportion of the patients with MIS-A (n = 20) had shock (75% vs. 37%), cardiac dysfunction (75% vs. 31%), myocarditis (70% vs. 17%), and pericardial effusion (45% vs. 23%).…”

“…Uncommonly, 9 (5%) of 195 patients had arterial or venous thrombosis, and 1 had severe mononeuritis multiplex, affecting the right median and facial nerves and both ulnar, tibial, peroneal, and sural nerves 16 . Finally, systemic capillary leak syndrome, spontaneous coronary artery dissection, severe inflammatory myopathy, cortical unihemispheric brain edema, thyroiditis, refractory status epilepticus, ischemic stroke, and subconjunctival hemorrhage have been rarely reported as clinical manifestations of MIS‐A 28,30,31,61–63 …”