During this acute phase, most patients require treatment in an intensive care unit to stabilize breathing, heart rate, and blood pressure. As the disease progresses, the symptoms become medically urgent and often include autonomic dysfunction, hypoventilation, cerebellar ataxia, loss of feeling on one side of the body, loss of consciousness, or catatonia. In many cases, this leads to the illness going undiagnosed. The symptoms usually appear psychiatric in nature, which may confound the differential diagnosis. Some other symptoms typical during the disease onset include impaired cognition, memory deficits, and speech problems (including aphasia, perseveration or mutism). Other common manifestations include seizures and bizarre movements, mostly of the lips and mouth, but also including pedaling motions with the legs or hand movements resembling playing a piano. These changes often include agitation, paranoia, psychosis, and violent behaviors. However, behavior changes are a common first symptom within both groups. During the initial stage of the disease, symptoms vary slightly between children and adults. Beyond the prodromal symptoms, the disease progresses at varying rates, and patients may present with a variety of neurological symptoms. These symptoms may be present for weeks or months prior to disease onset. Prior to the development of a symptom complex that is specific to anti-NMDA receptor encephalitis, people may experience prodromal symptoms, including headaches, flu-like illness, or symptoms similar to an upper respiratory infection. The disease was first described by Josep Dalmau in 2007. It typically occurs in adults younger than 45 years old, but it can occur at any age. The condition is relatively common compared to other paraneoplastic disorders. The estimated number of cases of the disease is one in 1.5 million people per year. Recurrence occurs in about 10% of people. About 4% of those affected die from the condition. Long-term mental or behavioral problems may remain. Outcomes are better if treatment is begun earlier. With treatment, about 80% of people have a good outcome. Treatment is typically with immunosuppresive medication and, if a tumor is present, surgery to remove it. Diagnosis is typically based on finding specific antibodies in the cerebral spinal fluid. The underlying mechanism is autoimmune, with the primary target being the GluN1 subunit of the N-methyl D-aspartate receptors (NMDAR) in the brain. Another established trigger is herpesviral encephalitis, while the cause in others cases is unclear. In some cases, patients may develop catatonia Ībout half of cases are associated with tumors, most commonly teratomas of the ovaries. Over time, seizures, decreased breathing, and blood pressure and heart rate variability typically occur. People are also often agitated or confused. 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). Early symptoms may include fever, headache, and feeling tired. Viral encephalitis, acute psychosis, neuroleptic malignant syndrome Ĭorticosteroids, intravenous immunoglobulin (IVIG), plasma exchange, azathioprine Īnti-NMDA receptor encephalitis is a type of brain inflammation caused by antibodies. Specific antibodies in the cerebral spinal fluid Later: Seizures, decreased breathing, blood pressure and heart rate variability NMDA receptor antibody encephalitis, anti-N-methyl-D-aspartate receptor encephalitis, anti-NMDAR encephalitisĮarly: Fever, headache, feeling tired, psychosis, agitated Medical condition Anti-NMDA receptor encephalitis
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