The patient is a previously healthy 29-year-old female who presented with a one-week prodromal headache and low-grade fever admitted in convulsive status epilepticus. MRI brain revealed nonspecific basal ganglia and subcortical FLAIR hyperintensities. Work-up, including serum and CSF viral/autoimmune/paraneoplastic encephalitis panels, CT chest/abdomen/pelvis, cerebral angiogram, and brain biopsy were all unremarkable.
Initial EEG showed severe slowing of the background, abundant bilateral independent periodic discharges, and numerous subclinical seizures of left frontotemporal onset. Seizures still occurred on a combination of midazolam and ketamine drip, and pentobarbital was initiated. She was treated for a presumed autoimmune etiology with IV methylprednisolone, plasmapheresis, IVIG, and anakinra. Ketogenic diet was stopped due to acidosis. She was maintained on 7 antiepileptic drugs (AEDs): clobazam, lacosamide, levetiracetam, oxcarbazepine, perampanel, phenobarbital, and phenytoin. Attempts to taper AEDs were complicated by re-emergence of bilateral independent focal seizures. On admission day 40, a VNS was implanted. The initial setting was 0.25 mA, 30 Hz, pulse-width 500 mS, cycle 3 seconds on, 3 minutes off. The current was up-titrated by 0.25 mA daily to 1.0 mA, and then every 3-4 days to 2.0 mA. Perampanel and oxcarbazepine were then weaned, and clobazam was tapered down. On day 66, patient began following one step commands. On day 86, patient was transferred out of the intensive care unit with intact mental status. There were no noted complications with VNS.