Anti-NMDA receptor encephalitis - Wikipedia
Anti-NMDA receptor encephalitis is a type of brain inflammation caused by antibodies.[4] Early symptoms may include fever, headache, and feeling tired.[1][2] This is then typically followed by psychosis which presents with false beliefs (delusions) and seeing or hearing things that others do not see or hear (hallucinations).[1] People are also often agitated or confused.[1] Over time, seizures, decreased breathing, and blood pressure and heart rate variability typically occur.[1] In some cases, patients may develop catatonia.[5]
About half of cases are associated with tumors, most commonly teratomas of the ovaries.[1][4] Another established trigger is herpesviral encephalitis, while the cause in others cases is unclear.[1][4][6] The underlying mechanism is autoimmune, with the primary target being the GluN1 subunit of the N-methyl D-aspartate receptors (NMDAR) in the brain.[1][7] Diagnosis is typically based on finding specific antibodies in the cerebral spinal fluid.[1] MRI of the brain is often normal.[2] Misdiagnosis is common.[7]
Treatment is typically with immunosuppresive medication and, if a tumor is present, surgery to remove it.[1] With treatment, about 80% of cases have a good outcome.[1] Outcomes are better if treatment is begun earlier.[2] Long-term mental or behavioral problems may remain.[2] About 4% of those affected die from the condition.[2] Recurrence occurs in about 10% of people.[1]
The estimated number of cases of the disease is one in 1.5 million people per year.[8][6] The condition is relatively common compared to other paraneoplastic disorders.[2] About 80% of those affected are female.[2] It typically occurs in adults younger than 45 years old, but it can occur at any age.[4][7] The disease was first described by Josep Dalmau in 2007.[1][9]
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