P2A218: A Rare Cause of Status Epilepticus

Introduction: Status epilepticus is a common presentation to the pediatric intensive care unit. As there are many possible underlying etiologies, early diagnosis is important to properly treat the cause. Systemic Lupus Erythematosus (SLE) and lupus cerebritis is an extremely rare cause of status epilepticus.

Case Description: A 16-year-old female presented with new-onset tonic-clonic seizure preceded by myoclonic jerks. Her physical exam was unremarkable. Basic lab studies were unremarkable except for elevated Erythrocyte Sedimentation Rate (44 mm/hr). CSF demonstrated mild elevation of white blood cells (17) but no other signs of infection by culture or PCR. Initial electroencephalogram (EEG) showed background slowing without a clear etiology. Initial MRI of the brain was normal. Further workup revealed an elevated ANA (8.2 units), Double-Stranded DNA Antibody (225 IU/ml), Ribosome P Antibody (IgG) at greater than 8 units.
The patient had progressive neurologic decline and persistent seizures despite multiple antiepileptic medications, ultimately requiring a pentobarbital infusion for burst suppression. She was initially treated for autoimmune encephalitis with pulse dose methylprednisolone for five days, four cycles of plasmapheresis, intravenous immunoglobulin (IVIg), and Rituximab without improvement. The patient was later started on cyclophosphamide after being diagnosed with fulminant lupus cerebritis. Repeat brain MRI/MRA showed edema and restricted diffusion of the entire cerebral cortex. Despite treatment, she developed signs of increased intracranial pressure and was found to have a left occipital infarction with diffuse bilateral cerebral edema. She was then transferred to a second tertiary children’s hospital at the parents’ request. There, the patient had an endoventricular drain placed for ICP management. She had continued status epilepticus without improvement in neurologic function. The patient died after parental request for removal of life-sustaining technology.

Discussion: Seizures are a common manifestation of lupus cerebritis. However, status epilepticus is an exceedingly rare complication of SLE and lupus cerebritis. While this is a rare entity, morbidity and mortality appear to be extremely high and thus providers should have a high index of suspicion for new-onset seizures, particularly in young females.

Conclusion: Status epilepticus is a rare and morbid complication of SLE and lupus cerebritis.