Sydenham's chorea: clinical and evolutive characteristics

Abstract

CONTEXT: During the last 12 years we have observed an increase in the frequency of Sydenham's chorea in our country. We have observed that some of our patients have presented recurrence of the chorea despite regular treatment with benzathine penicillin. OBJECTIVE: The aim of our study was to evaluate clinical and evolutive characteristics of Sydenham's chorea in a group of patients followed in our Pediatric Rheumatology Unit. TYPE OF STUDY: Retrospective study. SETTING: Section of Pediatric Rheumatology - Discipline of Allergy, Clinical Immunology and Rheumatology - Department of Pediatrics - UNIFESP - EPM. PARTICIPANTS: Two hundred and ninety patients with rheumatic fever followed between 1986 and 1999. METHODS: We reviewed the records of 290 patients with rheumatic fever followed between 1986 and 1999. All patients were diagnosed according to the revised Jones criteria (1992). We included 86 patients that presented Sydenham's chorea as one of the major criteria (one or more attacks) and evaluated their clinical and evolutive characteristics as well the treatment. RESULTS: Fifty-five patients were girls and 31 were boys. The mean age at onset was 9.7 years and mean follow-up period was 3.6 years. The 86 Sydenham's chorea patients presented 110 attacks of chorea. We observed isolated chorea in 35% of the patients, and 25 (29%) presented one or more recurrences. We included only 17 of the 25 patients for further analysis, with a total of 22 recurrences of which 14 were attacks of chorea, because it was not possible to precisely detect the interval between attacks in the other patients. The approximate interval between the attacks ranged from 4 to 96 months. In 71% of the patients there was no failure in the secondary prophylaxis with benzathine penicillin, which was performed every 3 weeks. CONCLUSION: Despite the regular use of secondary benzathine penicillin prophylaxis, children with rheumatic fever have a high risk of Sydenham's chorea recurrence.

Apresentação e desfecho da febre reumática em uma série de casos

“…Frequency of chorea (28%) was higher than that reported in the international literature, 2,4,6 with 15%-20% estimates, but was similar to the results reported in the Brazilian series, 11,14,22 nearly 30%. Chorea is characterized by involuntary movements of extremities, muscular hypotonia, dysartria, gait disturbance, and tics, as well as behavioral manifestations.…”

The Immunobiology of Tourette's Disorder, Pediatric Autoimmune Neuropsychiatric Disorders Associated withStreptococcus, and Related Disorders: A Way Forward

“…By examining the scant literature on using antibiotics to prevent SC recurrences, the complications in determining efficacy become apparent. Although prophylactic antibiotic therapy in patients with SC appears successful in the prevention of neuropsychiatric exacerbations (Gebremariam 1999), other investigators report that about a third will continue to have a recurrence (Terreri et al 2002). Studies in which SC patients received monthly prophylactic injections of benzathine penicillin G showed that not all SC recurrences appear to be GAS triggered (Korn-Lubetzki et al 2004) and that recurrences may occur after infections that are too mild or too brief to be easily detected (Berrios et al 1985).…”

Como a coréia de Sydenham é tratada no Rio de Janeiro?

“…Apesar de ter sua incidência reduzida nos últimos anos, principalmente em países desenvolvidos, ainda constitui, no Brasil, a principal causa de coréia adquirida na infância 6,7 . A incidência de cS em países desenvolvidos é de 0,2 a 0,8 por 100000 por ano, o que representa 10 a 40% dos pacientes com FR 3,[8][9][10][11] . A taxa de recorrência é de 10 a 42% [10][11][12] .…”

“…A incidência de cS em países desenvolvidos é de 0,2 a 0,8 por 100000 por ano, o que representa 10 a 40% dos pacientes com FR 3,[8][9][10][11] . A taxa de recorrência é de 10 a 42% [10][11][12] . cerca de 60 a 71% dos pacientes com cS apresentam lesões cardíacas valvares 3,10,13 .…”

“…A taxa de recorrência é de 10 a 42% [10][11][12] . cerca de 60 a 71% dos pacientes com cS apresentam lesões cardíacas valvares 3,10,13 . Esta última é a principal responsável pela morbidade crônica e mortalidade dos pacientes 2,4,14 .…”