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

  • Roser PonsEmail author
Chapter
Part of the Current Clinical Neurology book series (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.

Keywords

Attention Deficit Hyperactivity Disorder Rheumatic Fever Streptococcal Infection Streptococcal Pharyngitis Acute Disseminate Encephalomyelitis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Supplementary material

Movement_Disorders_Emergencies_C13.mov (25,945 KB)

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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.First Department of Pediatrics, Agia So fi a HospitalUniversity of AthensAthensGreece

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