Report of a Child With Febrile Status Epilepticus and Post-COVID Multi-System Inflammatory Syndrome
Holly C Appleberry1, Alexis Begezda2, Helen Cheung3
1Department of Neurology, Penn State Milton S. Hershey Medical Center, Hershey, PA, USA.
Insights
Multi-system Inflammatory Syndrome in Children (MIS-C) can trigger Febrile Infection Related Epilepsy Syndrome (FIRES) in infants. Early recognition of MIS-C is crucial for managing this rare neurological complication post-COVID.
Area of Science:
- Pediatric Neurology
- Infectious Diseases
- Neuroimmunology
Background:
- Multi-system Inflammatory Syndrome in Children (MIS-C) is a rare post-infectious complication following COVID-19.
- Central nervous system (CNS) involvement in MIS-C is uncommon and presents with diverse clinical and imaging findings.
- Febrile Infection Related Epilepsy Syndrome (FIRES) is a distinct clinical entity characterized by refractory status epilepticus.
Observation:
- A case of an infant presenting with refractory febrile status epilepticus is described.
- The infant later met clinical criteria for MIS-C, with significant multi-organ involvement.
- Brain MRI revealed diffuse restricted diffusion, predominantly in anterior regions.
Findings:
- The patient exhibited a heterogeneous presentation suggestive of CNS involvement in MIS-C.
- Genetic testing revealed multiple variants of uncertain significance.
- Treatment with pulse steroids and intravenous immunoglobulin (IVIg) led to clinical improvement.
Implications:
- This case underscores the importance of considering MIS-C in the differential diagnosis of FIRES, especially after COVID-19 exposure.
- Early identification and management of MIS-C can be critical for neurological outcomes in affected children.
- Further research is needed to elucidate the pathophysiology linking COVID-19, MIS-C, and FIRES.
Abstract:
Multi-system Inflammatory Syndrome in Children (MIS-C) is a post infectious inflammatory syndrome following COVID infection. Previous case series have demonstrated that CNS involvement is less common and presents heterogeneously. The following case describes an infant with an initial presentation of refractory febrile status epilepticus. Genetic testing later showed multiple variants of uncertain significance. The patient met clinical criteria for MIS-C and had a markedly abnormal brain MRI with bilateral diffuse restricted diffusion (anterior > posterior). Clinically, the patient improved with pulse steroids and IVIg. This case highlights the importance of maintaining MIS-C in the differential as a trigger of Febrile Infection Related Epilepsy Syndrome (FIRES) with multi-organ involvement presenting 2-4 weeks after infectious symptoms and COVID exposure.
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