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Movement Disorder Emergencies pp 163–177Cite as

Sydenham’s Chorea, PANDAS, and Other Post-streptococcal Neurological Disorders

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

Abstract

Sydenham’s chorea is a post-streptoccocal movement disorder and one of the major criteria for the diagnosis of rheumatic fever. The likely pathophysiological mechanism involves induction, as a response to the infection, of antibodies that cross-react with the basal ganglia. Anti-basal ganglia antibodies are found in 45–100% of patients with Sydenham’s chorea, and their levels correlate with disease activity.

Further post-streptococcal non-rheumatic neurologic disorders include the contested condition PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection) and other diseases such as disseminated encephalomyelitis with basal ganglia lesions; acute myoclonus; isolated striatal necrosis; paroxysmal dystonic choreoathetosis; acute parkinsonism; and the opsoclonus-myoclonus syndrome. In many of these conditions, antineuronal antibodies are detected raising the hypothesis of a common autoimmune pathophysiological mechanism.

The symptoms of Sydenham’s chorea, PANDAS, and other post-streptococcal CNS disorders can evolve rapidly, often requiring prompt intervention. Management of post-streptococcal CNS disorders includes symptomatic treatment of the acute movement disorder and/or psychiatric problem; antibiotic therapy; and immunotherapy.

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Authors and Affiliations

  1. First Department of Pediatrics, Agia So fi a Hospital, University of Athens, Thivon and Lebadias, Athens, 11527, Greece

    Roser Pons M.D.

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  1. Roser Pons M.D.
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Correspondence to Roser Pons M.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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Pons, R. (2013). Sydenham’s Chorea, PANDAS, and Other Post-streptococcal Neurological 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_13

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  • DOI: https://doi.org/10.1007/978-1-60761-835-5_13

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  • Publisher Name: Humana Press, Totowa, NJ

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  • Online ISBN: 978-1-60761-835-5

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