British Surveillance study of SC

The first prospective surveillance study of Sydenham’s chorea in the UK and Republic of Ireland, which was partly funded by us, has been published here –

Archives of Disease in Childhood 2023;108:736-741

Over a 24 month period, 40 eligible cases were identified by reports made by general paediatricians in the UK & Republic of Ireland.  

This works out as about 1 new cases every 2-3 weeks. No reports from Ireland! No obvious geographical clustering.

The mean age of cases was 9 years, and 60% were female. The majority were judged as ‘moderate’ or ‘severe’ chorea (72% and 14% respectively).

The most common neurological presenting features (apart from chorea) were loss of fine motor skills, walking problems, and speech difficulties.

But this is probably  an underestimate, given that half of children (51.2%) had severe impairment due to chorea on at least one of the six domains (hygiene, handwriting, dressing, speech, walking, and handling utensils) [using a special rating scale, called the UFMG Sydenham’s chorea rating scale].

86% also presented with emotional and/or behavioural symptoms. Emotional lability (wild swings) was seen in 76%, anxiety in 51%, tics in 37%.  More unusual features included episodic confusion with visual hallucination.

 Roughly 15% answered “not known” for these kinds of features.

20.9% had SC as part of rheumatic fever, but only 12% were reported as having carditis (heart involvement) and other rheumatic fever criteria are rare, so it is not clear on what basis this diagnosis was made.

And 17% didn’t get an echo of the heart, so hard to know if they did or didn’t.  

These children were more likely to get an MRI scan of the head than a throat swab! (86 vs 76%).

Only 1 had a  family history of SC.

76% were admitted to hospital, with mean length of stay 6 days (up to 22).  Only 30% saw a neurologist.

Almost all cases had evidence of prior streptococcal infection, and were prescribed courses of antibiotics of varying duration.

23% (10) were started on long term prophylaxis.

Other treatments used included symptomatic treatment with anticonvulsants and neuroleptics (53%). Approximately a quarter of cases (27.9%) received immunomodulatory treatment in the form of steroids or immunoglobulins. Since only 30% saw a neurologist, this makes it look as if neurologists pretty much always use immune treatments, and the majority of children who didn’t see a neurologist didn’t get offered it.  Potentially of course only severe cases were referred to neurology.

51% were referred to occupational therapy or physiotherapy (the ones who most needed it, hopefully, given that 51% were rated as having severe impairment of function) but definitely less than the 93% of children described as having moderate to severe chorea.  14% were referred to psychology.