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Semin Neurol. 2014 Sep;34(4):458-66. doi: 10.1055/s-0034-1390394. Epub 2014 Nov 4.

Neuronal surface antibody-mediated autoimmune encephalitis.

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Department of Neurology, Massachusetts General Hospital, Boston, Massachussetts.
Department of Neurology, Hospital Clínic /Institut d'Investigació Biomèdica August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
Centre de Recerca Biomèdica CELLEX, Lab Neuroimmonologia P3A, Barcelona, Spain.


In the past few years, many autoimmune encephalitides have been identified, with specific clinical syndromes and associated antibodies against neuronal surface antigens. There is compelling evidence that many of these antibodies are pathogenic and most of these encephalitides are highly responsive to immunotherapies. The clinical spectra of some of these antibody-mediated syndromes, especially those reported in only a few patients, are evolving. Others, such as anti-N-methyl-D-aspartate (NMDA) receptor encephalitis, are well characterized. Diagnosis involves recognizing the specific syndromes and identifying the antibody in a patient's cerebrospinal fluid (CSF) and/or serum. These syndromes are associated with variable abnormalities in CSF, magnetic resonance imaging, and electroencephalography. Treatment is often multidisciplinary and should be focused upon neutralizing the effects of antibodies and eliminating their source. Overlapping disorders have been noted, with some patients having more than one neurologic autoimmune disease. In other patients, viral infections such as herpes simplex virus encephalitis trigger robust antineuronal autoimmune responses.

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