Refractory status epilepticus secondary to atypical Rasmussen encephalitis successfully managed with aggressive immunotherapy

A 19-year-old right-handed man with focal epilepsy from age 11 years secondary to Rasmussen encephalitis (RE) was admitted for management of refractory status epilepticus (SE). His seizure types included left arm somatosensory aura, left-sided motor seizures, and complex partial seizures with automatisms. Serial video EEG confirmed seizures arising from right temporal or parietal regions. Serial brain MRIs showed progressive right hemispheric atrophy, affecting the perisylvian region, consistent with RE (figure 1, A-C). Brain biopsy at age 14 showed inflammatory features as shown in figure 1D. Clinical, radiologic, and biopsy features were consistent with atypical RE, atypical due to an indolent course. The patient had frequent seizures at a rate of 2-3 per week, despite multiple antiepileptic drugs (AEDs) and monthly IV immunoglobulin (IVIg) treatments (0.5 g/kg) since age 14. He did not develop focal neurologic deficits. He was attending college and playing basketball.