Sydenham’s chorea (SC) is an autoimmune movement disorder characterised by uncoordinated involuntary movements (chorea), emotional instability and hypotonia. It is one of the major Jones diagnostic criteria of rheumatic fever caused by group A streptococcus bacterium (GAS).
Report on the incidence of SC diagnosis in Ireland (including Northern Ireland) July 2006–July 2013.
Describe the clinical presentation, investigation and management.
Methods A retrospective observational study was undertaken of all children with a clinical diagnosis of SC or “SC like” presentation attending the paediatric neurology departments in Ireland. A questionnaire and chart review was performed on all cases.
Results 16 cases were documented by paediatric neurologists from 2006- 2013 giving an incidence of 0.21/100,000. 13 occurred in females with a median age of 10 years. 75% presented predominantly with a hemichorea.
All had functional impairment with 11/16 having behavioural or mood change. 5 had carditis diagnosed by echo. 13 had serological evidence of previous GAS infection. All had a normal MRI brain.
15/16 received medication. valproate was the first choice in 11 cases, steroids in 2 and valproate in combination with steroids in 2. 8 were treated with penicillin for >11 months.
Conclusion The incidence of Sydenhams chorea appears to be rising. It is a clinical diagnosis and important to recognise. In our cohort we used a combination of steroids and valproate which gave a good response. There was a trend towards long-term penicillin.
Acknowledgements Collaborators: David Webb (Crumlin), Tony Bouldin (Crumlin), Deidre Peake (Belfast), Sanjay Tirupathi (Belfast), Niamh Mc Sweeney (Cork).