Introduction: New onset refractory status epilepticus can have various reasons as etiology. Autoimmune encephalitis/encephalopathy is becoming more and more recognizable. This entity includes anti-neuronal surface protein antibodies, such as anti-VGKC, anti-NMDAr, anti-AMPAr; and intracellular antibodies, such as anti-GAD and onconeural antibodies.

Case Description: A 13-year-old boy presented with confusion accompanied by fever and focal seizures. MRI of brain showed abnormal signals from temporal lobes and CSF showed mild pleocytosis. HSV encephalitis was highly suspected. The patient was treated with acyclovir, but seizures were difficult to be controlled despite multiple anti-seizure medications. Eventually the patient was placed in a pentobarbital-induced coma, which necessitated a high dose to eliminate the seizures. PCRs for HSV twice were negative as well as other viral searching. Repeat MRI of brain did not show any progressive abnormalities and demonstrated stable signals from temporal lobes. Immune-modulating treatment was initiated 10 days after admission. Autoimmune work-up showed positive anti-VGKC antibody. The treatment included IVIG, high dose of steroids and plasmapharesis. Now, 7 weeks after admission, the patient’s seizures are still not well-controlled.

Discussion: Anti-VGKC antibodies associated intractable seizures are rare. Encephalitis associated with VGKC antibodies presented with status epilepticus and focal epilepsy can be confused with herpes encephalitis. Early recognition of autoimmune epilepsy is critical. Since most of the cases are non-paraneoplastic, more systematic and aggressive immune-modulating treatment should be initiated as soon as possible in order to improve outcomes.