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Anti-NMDA Receptor Encephalitis and Other Autoimmune and Paraneoplastic Movement Disorders

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Movement Disorder Emergencies

Part of the book series: Current Clinical Neurology ((CCNEU))

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Abstract

A substantial number of movement disorders are mediated by immunological mechanisms. In some instances the immune response is triggered by the presence of a tumor that ectopically expresses a neuronal protein, leading to a brain autoimmune response or paraneoplastic syndrome. Other immune-mediated movement disorders may be post-infectious, likely triggered by molecular mimicry or other, as yet unknown, mechanisms. There is a new and expanding group of syndromes that are associated with antibodies against cell surface or synaptic proteins and may cause early and prominent movement disorders. Anti-NMDA receptor encephalitis is the most frequent of these disorders that may occur with or without tumor association, affect children and adults, and can be severe but responsive to treatment. Recognition of this and other immune responses to synaptic proteins is important because, different from classical paraneoplastic syndromes, they often respond to immunotherapy. Because the presentation and clinical course of immune-mediated syndromes often develop very quickly, and because failure to recognize and treat these disorders may lead to morbidity or even mortality, we believe that this qualifies these syndromes as movement disorder emergencies. This chapter focuses on anti-NMDAR encephalitis and other autoimmune or paraneoplastic movement disorders, with emphasis on their clinical presentations, differential diagnoses, immunological associations and antigens, and treatment strategies.

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Authors and Affiliations

  1. Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA

    Jessica Panzer M.D., Ph.D. & Josep Dalmau M.D., Ph.D.

  2. Institució Catalana de Recerca i Estudis Avançats (ICREA) at Institut d’Investigació Biomèdica August Pi i Sunyer (IDIBAPS), Hospital Clinic, University of Barcelona, Villarroel, 170, Barcelona, 08036, Spain

    Josep Dalmau M.D., Ph.D.

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  1. Jessica Panzer M.D., Ph.D.
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Correspondence to Josep Dalmau M.D., Ph.D. .

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  1. , Neurology, Columbia University Medical Center, West 168th St. Suite 310 710, New York, 10032, USA

    Steven J. Frucht

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Panzer, J., Dalmau, J. (2013). Anti-NMDA Receptor Encephalitis and Other Autoimmune and Paraneoplastic Movement Disorders. In: Frucht, S. (eds) Movement Disorder Emergencies. Current Clinical Neurology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60761-835-5_22

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  • DOI: https://doi.org/10.1007/978-1-60761-835-5_22

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