Pediatric Movement Disorders - Chorea and Choreoathetosis

Treatment

Chorea may be difficult to treat. If the child is taking any medications that can cause or worsen chorea, these should be tapered and discontinued, if possible. The mainstay of treatment in adults is the class of "neuroleptics," including haloperidol and pimozide.

These drugs selectively enhance the function of the indirect pathway by blocking the inhibitory effect of dopamine on this pathway. However, the incidence of side effects in children from neuroleptics has been reported to be as high as 20%. Therefore, it is often safer to start with an alternative medication, such as a benzodiazepine, particularly clonazepam, diazepam, or clobazam. According to reports, certain anti-epileptic medications, such as valproate and piracetam, may improve the symptoms of chorea. In particular, valproate may be very helpful in children with Sydenham's chorea.

There is considerable debate about whether children with Sydenham's chorea due to streptococcal infection should be given long-term antibiotics. There is not yet scientific evidence to support this, although short-term treatment is certainly needed in order to prevent complications such as rheumatic fever. Many forms of chorea (including Sydenham's chorea) will gradually resolve with time even if untreated.