ABSTRACT
Introduction: Since April 2020, Multisystem inflammatory syndrome in children (MIS-C) has been reported worldwide and associated with a different spectrum of symptoms. Although mild neurological manifestations in SARS-Cov2 infection and MIS-C have been reported, severe involvement with brain abnormalities, is rare 1. Objectives: Describe a child with MIS-C presenting non-convulsive status epilepticus associated with abnormal cerebral magnetic resonance (MR), never previously reported. Methods: Case report. Results: A previously healthy 19-month-old girl presented to our emergency department after a prolonged febrile seizure involving the right side of her body lasting about 25 minutes. She presented with fever lasting more than 24 hours. On physical examination, abdominal distention and tenderness and altered mental status with irritability were detected. RT-PCR for SARS-CoV-2 on nasal swab was negative but her parents had SARS-CoV-2 infection four weeks earlier. Laboratory showed elevated CRP (35 mg/L), while all microbiological analyses in blood, urine and CSF were negative. Computerized tomography (TC) showed a doubtful left temporal hypointensity, and cerebral MRI displayed cytotoxic oedema in left temporal mesial area of the brain on diffusion-weighted imaging (DWI). Few day later, her clinical conditions worsened with irritability and drowsiness associated with persistent abdominal distention, diarrhoea, and high fever. The EEG revealed a pattern suggestive for non-convulsive status epilepticus responsive to benzodiazepines and loading dose of Levetiracetam. Consensually, an increase of inflammatory markers (CRP 153 mg/L, procalcitonin 114 ug/L) was observed. Chest X-ray, EKG, troponin and BNP levels were normal, whereas echocardiogram demonstrated left ventricular diastolic dysfunction and mild pericardial effusion. In the suspicion of MIS-C with abdominal, cardiac and neurological involvement, she was treated with intravenous immunoglobulin (2g/kg), methylprednisolone (2 mg/kg) and acetylsalicylic acid (5mg/kg). Serum SARS-Cov2 antibody test resulted positive for previous infection, confirming the diagnosis of MIS-C. Neuronal antibodies for immune-mediated CNS disorders tested negative. Within 36 hours from therapy start, a significant improvement in general conditions, along with stable apyrexia and decreasing in inflammatory markers were observed. She was discharged two weeks later on oral steroids, ASA and Levetiracetam;the physical examination was normal, and EEG showed a global improvement in brain electrical activity. Conclusion: Neurological symptoms secondary to SARS-Cov2 infection and MIS-C have been reported in children (1) but only a few present severe neurological complications such as status epilepticus. Non-convulsive status epilepticus has been previously described in an adult with acute COVID192 but has never been reported as presenting sign of MIS-C. The current case illustrates the need of a careful neurological evaluation in children with MIS-C, as CNS involvement can represent the main clinical presentation thus underlining the need of an appropriate diagnostic and therapeutic approach.