Refractory status epilepticus secondary to Neurexin-3a encephalitis: A case report

摘要

New–onset refractory status epilepticus (NORSE) is a rare and challenging disease entity that is associated with high morbidity and mortality. Among the NORSE cases with a proven aetiology, autoimmune encephalitis has been identified as a leading cause. Here we describe a young man who presented with NORSE and was eventually found to have serum autoantibodies against Neurexin-3a, a synaptic molecule that has recently been implicated as a target in the pathogenesis of a novel form of autoimmune encephalitis. The patient’s seizures responded to plasmapheresis, though the patient eventually died from sepsis. This case shows that Neurexin-3a encephalitis is a possible cause of NORSE, and plasmapheresis may be an effective treatment.