An update on the treatment of Sydenham’s chorea: the evidence for established and evolving interventions

Abstract

Over 320 years after Thomas Sydenham described the condition labelled Sydenham's chorea, it remains poorly understood. The disorder is an antineuronal antibody-mediated neuropsychiatric disorder caused by a poststreptococcal, autoimmune condition affecting control of movement, mood, behaviour and potentially the heart. The treatment remains empirical, and is less than optimal. There are few large clinically controlled trials. Recommendations for optimal management remain inconsistent and are hampered by the side effects from pharmacotherapy. Care for patients should be targeted at primary treatment (penicillin and bed rest), secondary palliation (symptomatic medication) and supportive (social) care. Small studies have demonstrated trends to support the use of immunoglobulins and steroids as therapeutic interventions for children affected by Sydenham's chorea.

Neuropsychological manifestations in children with Sydenham’s chorea after adjunct intravenous immunoglobulin and standard treatment

“…We included a non-SC control group for comparison. Similar to results reported by Walker et al (Walker and Wilmshurst 2010), we hypothesized that children with SC who had received IV immunoglobulins in addition to the standard treatment (IVIG+STDT) would demonstrate less pronounced impairments 6 months after initiating treatment compared with children who received standard treatment alone.…”

“…Immunomodulatory therapies, including corticosteroids, plasma exchange (PE) and intravenous immunoglobulins (IVIG), have also been effectively used to treat PANDAS and SC (Van Toorn et al 2004;Cardoso et al 2003;Garvey et al 2005;Van Immerzeel et al 2010;Walker et al 2012). However, there is a paucity of controlled studies measuring the efficacy of these various treatments (Cardoso 2011;Bonthius and Karacay 2003;Walker and Wilmshurst 2010). Recommendations for treatment are mostly Boff label^; i.e., there are no guidelines indicating the optimal duration of treatment.…”

“…In addition to the administration of penicillin, neuroleptics (such as risperidone and haloperidol) or sodium valproate are used to correct neurochemical imbalances in the basal ganglia (Cardoso 2011;Bonthius and Karacay 2003;Davutoglu et al 2004;Walker and Wilmshurst 2010). Immunomodulatory therapies, including corticosteroids, plasma exchange (PE) and intravenous immunoglobulins (IVIG), have also been effectively used to treat PANDAS and SC (Van Toorn et al 2004;Cardoso et al 2003;Garvey et al 2005;Van Immerzeel et al 2010;Walker et al 2012).…”

Conventional and intravenous immunoglobulin therapy in paediatric antiphospholipid antibodies-related chorea

“…The treatment should last no more than four to eight weeks to avoid the risk of tardive dyskinesia and Parkinsonism. 12 Risperidone is also used in young patients affected by a severe type of chorea. 13 In adult patients other atypical neuroleptics and xenazine could represent effective alternatives, but there have been no controlled studies in children.…”

“…12 Dopamine receptor antagonists mainly used in children include haloperidol, risperidone and primozide (the last one used in children 12 years or older). Haloperidol is the first-line therapy used in children at a dose of 0.025 mg/kg/day in divided doses up to a maximum of 0.15 mg/kg/day.…”