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Status epilepticus (SE) has different causes, one of which is autoimmune encephalitis (AIE). The SE on AIE can progress to refractory status epilepticus (RSE), despite being on anti-seizure medications (ASMs). If AIE is suspected, early confirmation with MRI and CSF and initiation of immunosuppressive therapies, like intravenous methylprednisolone (IVMP), plasma exchange or intravenous immunoglobulins(IVIG) in addition to ASMs, is recommended. Literature on RSE in AIE suggests high morbidity and mortality, when presented being stupor, status epilepticus lasting beyond 10 days and autoantibodies against GABA-B receptors in AIE (GABIE).  We report a patient with GABIE whose RSE lasted greater than 10 days but ultimately returned to her neurologic baseline.

49-year-old female with PMH of hypertension who presented for two days of dizziness, memory loss and progressive difficulty functioning followed by multiple seizures. During admission, seizures became more frequent and progressed into SE despite use of multiple ASMs. MRI brain revealed progressively intensifying T2 signal in the bilateral mesial temporal and occipital lobes. Lumbar puncture showed elevated protein and WBC with positive GABA-B confirming GABIE. EEG confirmed refractory status epilepticus originating from bilateral independent right and left temporal-occipital areas despite being on multiple ASMs and completing 5 days of daily IVMP 1000mg and 5 sessions of plasmapheresis. Persistent florid electrographic seizures, persuaded the team to start daily IVIG and methylprednisolone 1000mg in combination for 5 days. The seizures began to improve and abated within 15 days.

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Early and aggressive treatment has been recommended for AIE. GABIE with RSE, however, has been found to have a poor response to immunomodulating therapies. We report a patient to the contrary. This case indicates that aggressive treatment with immunomodulating therapies may still be beneficial even for patients with RSE GABIE, in addition to conventional ASMs. Further, combination IVIG and IVMP should be considered.