Autoimmune Encephalitis and Related Disorders of the Nervous System
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"About 20 years ago the group of diseases currently known as "autoimmune encephalitides" or "antibody-mediated encephalitides" was unknown and the field of "Autoimmune Neurology" inexistent. Since then, 18 autoimmune encephalitides and the corresponding syndromes have been described including 16 in which the antigens are expressed on the cell surface of neurons and two on the surface of glial cells.1-3 In many of these diseases several immunological triggers have been identified - usually tumors or viral infections -, and in some, a genetic susceptibility linked to distinct human leukocyte ant...
"About 20 years ago the group of diseases currently known as "autoimmune encephalitides" or "antibody-mediated encephalitides" was unknown and the field of "Autoimmune Neurology" inexistent. Since then, 18 autoimmune encephalitides and the corresponding syndromes have been described including 16 in which the antigens are expressed on the cell surface of neurons and two on the surface of glial cells.1-3 In many of these diseases several immunological triggers have been identified - usually tumors or viral infections -, and in some, a genetic susceptibility linked to distinct human leukocyte antigen (HLA) has been shown.1, 4 These findings along with the availability of specific diagnostic tests and clinical guidelines,5 have remarkably changed the diagnostic and treatment approach to patients with encephalitis of unclear etiology which until recently comprised 45-50% of all cases of encephalitis and now represent only 20-25% of all cases.6 Neurologic disorders once considered idiopathic, or ascribed to possible infections, or defined with descriptive terms, are now recognized as autoimmune diseases that may lead to psychosis, catatonia, abnormal movements, seizures, memory impairment, cognitive decline, dysautonomia, or spinal cord dysfunction. Due to the severity and duration of these symptoms, up to 50% of patients require and receive prolonged intensive care support that not long ago may have been considered futile.7, 8"--
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