Dr. Jankovic, Director of the Parkinson's Disease Center and Movement Disorders Clinic at Baylor College of Medicine, has received research and/or training grants from Adamas, Allergan, Biotie, Civitas/Acorda, Hoffmann-La Roche, Medtronic, Merz, Neurocrine , Nuvelution, Pfizer, Prothena, Psyadon, Revance, and Teva; and has served as a consultant or as an advisory committee member for Adamas, Allergan, Merz, Prothena, Revance, and Teva.)
Movement disorders are prominent in the clinical presentation of many autoimmune disorders (Baizabal-Carvallo and Jankovic 2018). They are common in over 50% of cases in children, but less so in adults (Dash et al 2018). Nonetheless, the subacute onset of a new movement disorder in a subject older than 50 years old should prompt the suspicion of a possible autoimmune disorder. This article is not a comprehensive review of autoimmune encephalitis. Rather, it will focus on disorders in which movement disorders are an integral part of disease symptomatology.
• Autoimmune encephalitis should be suspected in the context of new-onset movement disorders in an acute/subacute presentation and an accompanying neuropsychiatric syndrome.
• Early diagnosis and treatment are associated with a better prognosis and decreased risk of relapse.
• Treatment of the underlying disorder usually improves the movement disorders, but symptomatic treatment may be necessary as well.
Historical note and terminology
The first identification of paraneoplastic neurologic syndromes occurred in the late 1940s, when neurologic symptoms were associated with a systemic cancer, but without an identifiable lesion in the nervous system (Dalmau and Rosenfeld 2008). Later, specific antibodies to onconeural intracellular antigens (Hu, Yo, Ri, Ma2, Tr, CV2, etc.) were identified, usually manifesting as a paraneoplastic cerebellar degeneration or limbic encephalitis (Heine et al 2015). In 2005, the identification of antibodies to neuronal surface antigens as a causal agent, often, but not always, associated with malignancies, revolutionized the field (Ances et al 2005). Whereas “classical” paraneoplastic syndromes have a poor response to treatment, the autoimmune encephalitis with antibodies to cell surface or synaptic proteins are treatable, and clinical improvement can be observed even months after the initial episode (Leypoldt et al 2015).
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