DOCUMENT

Headlines 11 T he complication from a sore throat that causes inflammation of the heart can also appear in the brain, resulting in a condition called Sydenham’s chorea. Sydenham’s chorea targets the movement control system in the brain’s basal ganglia. Around seven to eight children are admitted to hospitals in the Auckland region with the condition each year, which is about 12% of rheumatic fever cases overall. Dr Hannah Jones is a consultant paediatric neurologist at Starship and recipient of the Senior Clinical Research Fellowship from the Neurological Foundation. She is about to embark on a $1.4 million HRC-funded study to assess if a high-dose steroid is effective as a treatment. “Sydenham's chorea is rare, but unfortunately New Zealand and Australia have some of the highest rates in the world,” she says. “Children develop uncontrollable movements of their face and limbs, which they try to mask with purposeful activity. Some children do not have the heart or joint symptoms, and it can take weeks for parents and teachers to recognise that their children are not just fidgety,” says Hannah. “The children frequently also have psychiatric symptoms such as volatile emotions, anxiety, hyperactivity and obsessive-compulsive symptoms. Thankfully it has a very distinctive presentation, and most New Zealand and Australian doctors would recognise the condition.” For her study, Hannah is recruiting children with Sydenham's chorea in both New Zealand and Australia to achieve a larger sample size. In Australia, Aboriginal and Torres Straight Islanders are overrepresented in cases. She aims to enrol 80 children – with 40 children to be given a three- day course of high-dose steroids and 40 to receive a placebo. “High-dose dexamethasone has been chosen as the intervention because it is used in other neuro-immunological disorders in children. It is taken orally three times a day for three days, and a short course is rarely associated with side-effects. We have been using this medication in some children in Australia and New Zealand but it needs further research to know if it’s the best treatment moving forward.” Without treatment, the symptoms of Sydenham’s chorea usually resolve within six months. “Six months is a significant period for children to be facing difficulties with daily activities, having time off school and dealing with neuropsychiatric symptoms which we may be able to treat promptly. In overseas studies the psychiatric symptoms have been shown to persist for years, with long-term difficulties continuing into adulthood.” Hannah hopes her work will help to make a real difference in vulnerable children’s lives. “While more needs to be done to reduce rheumatic fever rates, it is also important to find treatments to reduce suffering and improve the quality of life for those that are unfortunate enough to develop the condition.” hidden Dr Jones examines a three-month-old boy at Starship, admittedwith paroxysmal movements. “Some children do not have the heart or joint symptoms, and it can take weeks for parents and teachers to recognise that their children are not just fidgety.”