Anti-NMDA receptor encephalitis
Rare disease which results in brain inflammation / From Wikipedia, the free encyclopedia
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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]
Anti-NMDA receptor encephalitis | |
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Other names | NMDA receptor antibody encephalitis, anti-N-methyl-D-aspartate receptor encephalitis, anti-NMDAR encephalitis |
A schematic diagram of the NMDA receptor | |
Specialty | Neurology |
Symptoms | Early: Fever, headache, feeling tired, psychosis, agitated[1][2] Later: Seizures, decreased breathing, blood pressure and heart rate variability[1] |
Complications | Long term mental or behavioral problems[2] |
Usual onset | Over days to weeks[3] |
Risk factors | Ovarian teratoma, unknown[1][4] |
Diagnostic method | Specific antibodies in the cerebrospinal fluid[1] |
Differential diagnosis | Viral encephalitis, acute psychosis, neuroleptic malignant syndrome[2] |
Treatment | Immunosuppresive medication, surgery[1] |
Medication | Corticosteroids, intravenous immunoglobulin (IVIG), plasma exchange, azathioprine[2] |
Prognosis | Typically good (with treatment)[1] |
Frequency | Rare [2] |
Deaths | ~4% risk of death[2] |
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]