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Autoantibodies against NMDA receptors and other neuronal targets: the widening spectrum of antibody-mediated CNS disorders

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Autoantibodies against the neuromuscular acetylcholine receptors in myasthenia gravis were first discovered almost 40 years ago, but the existence of autoantibodies that cause CNS disease has only recently been appreciated. Three main targets for the autoantibodies are components of the voltage-gated potassium channel complex, LGI1 and CASPR2 , and NMDA receptors. The diseases are usually quite sudden in onset with combinations of seizures, psychiatric and cognitive dysfunction, and the patients progress rapidly, unless treated with immunotherapies to reduce the levels of the antibodies, although some do improve spontaneously over time. Although, there are many questions outstanding, current knowledge of the cellular targets of the antibodies and the mechanisms involved will be discussed.

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