Practice Current: How do you treat anti-NMDA receptor encephalitis?

Anti–NMDA receptor (anti-NMDAR) encephalitis was first described in 20071 and is now recognized as one of the most common forms of encephalitis.2 Anti-NMDAR encephalitis is considered a multistage disease, characterized by nonspecific prodromal flu-like symptoms, followed by acute onset of psychiatric manifestations such as psychosis, delusions, hallucinations, anxiety, insomnia, repetitive behaviors, echolalia, and mutism. Patients at this stage generally present to either neurology or psychiatry services. This phase is usually followed by a change in level of alertness with periods of extreme agitation and catatonia along with the appearance of the classic orofacial and lingual dyskinesias or other movement disorders and pronounced autonomic instability. The combination of autonomic storms and coma often leads to a prolonged intensive care unit (ICU) admission. Patients—children in particular—can also develop focal or generalized seizures. Anti-NMDAR encephalitis can be associated with a tumor, especially ovarian teratomas in female patients older than 12 years. Brain MRI is normal in up to 67% of patients, whereas EEG is abnormal in 90% of patients.3 EEG findings are nonspecific and can include slowing, disorganization of the background, and electrographic seizures. The diagnosis is confirmed by the presence of NMDAR antibodies in the CSF.