Anti-NMDA receptor encephalitis is a relatively recently-described syndrome, first reported in four young women with ovarian teratomas in 2005. The syndrome is characterized by autoantibodies to the N-methyl D-aspartate receptor. Patients often present with prodromal headaches, fever, and URI symptoms, followed by psychiatric symptoms such as anxiety, agitation, decreased levels of consciousness, bizarre behavior, disorganized thinking, and memory deficits. Symptoms often degenerate to reduction of verbal output, seizures, and autonomic instability. Approximately 80% of patients with NMDA-R encephalitis are women, often younger than 45 years of age, and 50-60% of these women will have an ovarian teratoma expressing neurologic tissue. Men are less often affected by NMDA-R encephalitis, and less often have an associated tumor. Approximately 75% of patients will have substantial regression of symptoms with initiation of treatment, which includes steroids, IVIG, plasmapharesis, and removal of the associated tumor. However, 25% of patients will have severe neurological impairment or die as a result of the disease. Factors that predict improved outcome include early treatment with appropriate immunotherapy, ovarian teratoma detection, and lower severity of symptoms.
References: Anti NMDA Receptor Encephalitis