2002

Majority of patients (79%) were given drug treatment for the chorea. The drugs used
were: phenobarbitone 30-60 mg three times daily (50%), haloperidol 0.75-1.5 mg twice
daily (29%), chlorpromazine 25 mg twice to thrice daily (13%), enzhexol 2 mg twice to
thrice daily (8%) and erphenazine 2 mg twice to thrice daily (8%). he percentage of good
clinical response to these rugs was: haloperidol (100%), benzhexol(100%),
perphenazine (100%), phenobarbitone(83%) and chlorpromazine (67%). Only one
p a t i e n t h a d t o s t o p d r u g t r e a t m e n t(phenobarbitone) due to
drowsiness. Except thepatient with persistent isolated chorea, drug
therapy could be successfully withdrawn in allother patients. There was no difference in
theclinical response of chorea to drugs in patientswith isolated chorea compared with
mixed chorea

2003
Despite treatment with valproic acid and neuroleptics, a significant proportion of patients
with Sydenham chorea (SC) remain with chorea. We evaluated the effect of intravenous
methyl-prednisolone followed by oral prednisone in patients with SC refractory to
conventional treatment. Patients were enrolled in the study if they failed to improve with
conventional treatment, despite the development of side effects. Chorea was rated on a
0 to 4 score. Five patients, 3 of them women, were included in the study. The median
pretreatment rating score of the chorea was 3 (range, 3–4) and dropped to 1 (range, 0–
2) after a median follow-up of 7 months (range, 3–7 months). Two patients developed
Cushing syndrome. Our data suggest that intravenous methyl-prednisolone followed by
oral prednisone is an effective and well-tolerated treatment of refractory SC. © 2003
Movement Disorder Society

2005
Sydenham's chorea has been established as a postinfectious autoimmune
neuropsychiatric disorder. Corticosteroids have been used to treat patients with severe
disease but are not always effective, and relapses are frequent after cessation. Eighteen
subjects were entered into this randomized-entry controlled trial designed to determine if
intravenous immunoglobulin or plasma exchange would be superior to prednisone in
decreasing the severity of chorea. Mean chorea severity for the entire group was
significantly lower at the 1-month follow-up evaluation (overall 48% improvement).
Although the between-group differences were not statistically significant, clinical
improvements appeared to be more rapid and robust in the intravenous immunoglobulin
and plasma exchange groups than in the prednisone group (mean chorea severity
scores decreased by 72% in the intravenous immunoglobulin group, 50% in the plasma
exchange group, and 29% in the prednisone group). Larger studies are required to
confirm these clinical observations and to determine if these treatments are cost-
effective for this disorder. (J Child Neurol2005;20:424—429).
2008
Four adolescents with a syndrome consistent with Sydenham's chorea were treated with
haloperidol, a neuroleptic agent of the butyrophenone series. Haloperidol was given in
daily dosages ranging between 1 and 3mg and the abnormal movements in all four
patients rapidly disappeared. There were no recurrences of abnormal movements during
the course of treatment, but chorea reappeared in one patient when the drug was
discontinued after a two-month course. This quickly resolved when treatment was
resumed. Haloperidol is proposed as a form of symptomatic treatment for the abnormal
movements of Sydenham's chorea.

2009
Rest is usually advocated, as traditional teaching is that this reduces the risk of
rheumatic heart disease, although this is not evidence-based.

The most important consideration is secondary prophylaxis, usually with penicillin, to

prevent further streptococcal infections.

A number of drugs may help control the chorea, although many of these can have
extrapyramidal effects of their own. Probably the best documented of these is
haloperidol but valproate is now most specialists' first-line choice.1

Dopamine receptor blocking drugs such as pimozide are used for non-responders or
those with chorea paralytica.

Immune therapies, e.g. plasma exchange, intravenous immunoglobulins, may play a role
in selected patients.18

See the separate article Rheumatic Fever for discussion of the treatment of cardiac
complications.

2010
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.