Treatment of Sydenham's Chorea with Haloperidol

Abstract

SUMMARY 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. RÉSUMÉ Traitement de la chorée de Sydenham par l'halopéridol Quatre enfants présentant un tableau manifeste de chorée de Sydenham ont été traités par l'halopéridol, agent neuroleptique de la série des butyrophénones. L'halopéridol fut donnéà des doses journalières de 1 à 3mg et les mouvements anormaux disparurent rapidement dans les quatre cas. II n'y eut pas de retour des mouvements anormaux durant le traitement. Chez un sujet les mouvements choréiques apparurent à nouveau après l'arrêt d'un traitement de deux mois: la reprise du traitement régularisa rapidement la situation. L'halopéridol est proposé comme une forme de traitement symptomatique des mouvements anormaux de la chorée de Sydenham. ZUSAMMENFASSUN Behandlung der Sydenhamschen Chorea mit Haloperidol Vier Kinder mit einem Syndrom, das der Sydenhamschen Chorea entspricht, wurden mit Haloperidol behandelt, einem neuroleptischen Mittel der Butyrophenongruppe. Haloperidol wurde in täglichen Dosen von 1 bis 3mg gegeben, worauf die abnormen Bewegungen bei allen vier Patienten sofort verschwanden. Während der Behandlungsperiode traten die abnormen Bewegungen nicht wieder auf. Bei einem Patienten wurde die Haloperidoltherapie nach zwei Monaten unterbrochen und die Chorea trat wieder auf. Dies konnte schnell behoben werden, indem die Therapie wieder aufgenommen wurde. Haloperidol wird zur symptomatischen Behandlung der Sydenhamschen Chorea empfohlen. RESUMEN Tratamiento de la corea de Sydenham con haloperidol Cuatro niños con un síndrome de corea de Sydenham fueron tratados con haloperidol, un agente neuroléptico de la serie de la butirofenona. El haloperidol se dio diariamente a dosis oscilantes entre 1 y 3mg y los movimientos anormales en los cuatro pacientes desaparecieron rápidamente. No hubo reaparición de movimientos anormales en el curso del tratamiento. Cuando en un paciente se interrumpió el haloperidol durante dos meses reapareció la corea, la cual se resolvió rápidamente al reinstaurar el tratamiento. Se propone el haloperidol como una forma de tratamiento sintom'tico de los movimientos anómalos de la corea de Sydenham.

Treatment of Sydenham’s Chorea: A Review of the Current Evidence

“…For example, reports varied on whether they provided the time to improvement of symptoms (10 out of 44),19,25,27,30,35–38,42,55 the time to full remission of symptoms (two out of 44),21,28 or both (13 out of 44) 20,22–24,26,32,33,35,41,44–46,52,53. Nine out of 38 studies reviewed improvement in a chorea score at fixed weekly, monthly, or yearly intervals, rather than reporting the time to an observed improvement or recovery 29,40,47–51,54,56.…”

“…However, this literature review identified significant diversity of antibiotic treatment and/or reporting. Twenty-one studies failed to comment on whether primary or secondary penicillin treatment was utilized19–21,23,25,26,29,31,33–36,39,45,46,48,51,54–56 and 10 reports did not comment on the length of treatment or method of delivery 22,24,28,35,40,42–44,51,53. One study reported primary but not secondary penicillin treatment 47.…”

“…In 1972 haloperidol (4–5 mg daily, divided twice a day or three time a day) was successfully used in two patients that had previously failed phenothiazine, diazepam, sedation, and/or amantadine 39. Four additional patients responded within several days to haloperidol 1–3 mg 33. In a retrospective analysis of 100 SC patients, haloperidol was successfully used in 82 out of 87 and was ineffective in five; the latter were subsequently treated with prednisone 31.…”

“…Data from these studies were incorporated into our total number, recognizing that negative case reports usually go unpublished and the deficiency of placebo-controlled trials. Thus, taking all evaluated studies into consideration, the results are as follows: for haloperidol, 133 out of 159 patients were successfully treated solely with this medication;10,20,25,31,33–35,39,40,42,44 treatment was ineffective in 119,10,19,20,25,31,35,48,52,55 and was discontinued because of side effects in seven 10,19,20. Of note, these numbers do not include 20 patients treated with either haloperidol or haloperidol plus IVIG (discussed later),50 since it was difficult to incorporate the results of this dual therapy trial into a discussion of haloperidol efficacy.…”

An update on the treatment of Sydenham’s chorea: the evidence for established and evolving interventions

“…Dopamine receptor antagonists include haloperidol, pimozide and risperidone. Haloperidol is an effective symptomatic medication but it must be titrated slowly to reach maximum effect with minimal toxic manifestations [Shenker et al 1973]. Table 1 summarizes the larger cases series which have addressed the efficacy and safety of treatment options for SC.…”