Therapeutic aspects of Sydenham's Chorea: an update

Greta Depietri; Niccolo Carli; Attilio Sica; Domenico  Oliviero; Giorgio  Costagliola; Pasquale Striano; Alice  Bonuccelli; Flavia  Frisone; Diego  Peroni; Rita  Consolini; Thomas Foiadelli; Alessandro  Orsini

doi:10.23750/abm.v92iS4.12663

Therapeutic aspects of Sydenham's Chorea: an update

Authors

Greta Depietri Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa
Niccolo Carli Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa
Attilio Sica Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa
Domenico Oliviero Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa
Giorgio Costagliola Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Pisa, Italy
Pasquale Striano Pediatric Neurology Unit, Dinogmi, Giannina Gaslini’s. Istitute, University of Genoa, Italy
Alice Bonuccelli Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Italy
Flavia Frisone Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Italy
Diego Peroni Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Italy
Rita Consolini Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Italy
Thomas Foiadelli Pediatric Clinic, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia
Alessandro Orsini Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Italy

Keywords:

Acute Rheumatic Fever, Beta-Hemolytic Streptococcal Pharyngitis, Hyperkinetic Movement Disorder, Sydenham’s Chorea

Abstract

Sydenham’s Chorea (SC) is a hyperkinetic movement disorder associated with neuropsychiatric manifestations. It is believed to be caused by the autoimmune response following a group A beta-hemolytic streptococcal (GABHS) pharyngitis, and it is one of the major diagnostic criteria for Acute Rheumatic Fever (ARF) diagnosis.

Despite having been known and studied for centuries, there are still no standardized therapies or official guidelines for SC treatment, so that it is necessarily left to physicians’ clinical experience. Antibiotic treatment, symptomatic therapies, and immunomodulatory treatment are the three pillars upon which SC patients’ management is currently based, but they still lack a solid scientific basis.

The aim of this writing is precisely to review the state of the art of SC’s treatment, with an overview of the advances made in the last 5 years. However, since the therapeutic uncertainties are a mere reflection of the severe gap of knowledge that concerns SC’s pathogenesis and manifestations, the importance of high-quality research studies based on homogenized methodologies, instruments, and measured outcomes will also be stressed.

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