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Purpose of Review: Paraneoplastic neurologic syndromes target specific areas of the nervous system with pathogenic autoantibodies or T-cell responses. Each syndrome conveys a risk of particular tumors. Expanded paraneoplastic antibody testing has led to improved diagnosis but created challenges involving appropriate interpretation of test results.Recent Findings: Peripheral nervous system paraneoplastic disorders such as myasthenia gravis and Lambert-Eaton myasthenic syndrome involve pathogenic autoantibodies. Recently, the pathogenic mechanisms and antigens of these disorders have been further elucidated. Paraneoplastic syndromes associated with onconeuronal antibodies, such as anti-Hu, have strong cancer associations and limited response to treatment. Autoimmunity to central nervous system membrane proteins, such as the N-methyl-D-aspartate (NMDA) receptor or leucine-rich, glioma inactivated 1 (LGI1), defines an expanding group of disorders with better prognosis and more variable cancer associations. In these diseases, the autoantibodies are either proven to be or are potentially pathogenic. An animal model of anti-NMDA receptor encephalitis will allow novel treatments to be developed. Autoantibodies to intracellular synaptic antigens, such as glutamic acid decarboxylase 65 (GAD65), are associated with diverse disorders such as stiff person syndrome, and the pathophysiology of these diseases is unclear.Summary: Paraneoplastic disorders have diverse clinical manifestations, including weakness, sensory neuronopathy, encephalitis, epilepsy, and psychosis. Proper use of antibody testing may assist with diagnosis. Treatment may require immunotherapy and tumor treatment.