Treatment Options for Anti-N-methyl-D-aspartate Receptor Encephalitis

Anti-NMDAR encephalitis is an autoimmune limbic disorder with an unknown cause, which affects mostly young women. Behavioral changes, seizures, orofacial muscle contractions, and memory impairment are usually observed. The variability of the symptoms depends on which part of the brain is inflamed. Treatment options aim to reduce serious neurological damage. Early diagnosis combined with the proper treatment favorably impacts the outcome. In our facility, weekly induction therapy is started pending an investigation into an associated ovarian or other tumor. Two case studies are discussed with favorable outcomes. The first is an outstanding case study with refractory status epilepticus caused by anti-NMDA receptors with benign teratoma which improved following combination therapy with rituximab and cyclophosphamide. There was no response to benzodiazepines and to an abundant number of antiepileptic drugs. Brain magnetic resonance imaging clearly indicated severe brain atrophy in the first case. Serial EEG monitoring showed a multitude of abnormalities. Delta brush activity was interictal and distinctive of anti-NMDAR encephalitis.