Picture 1. Chorea Sydenham


CHOREA SYDENHAM,
A CLINICAL MANIFESTATION OF ACUTE RHEUMATIC FEVER

Vicka Farah Diba, Noormanto, E.S Herini
Pediatric Health Department, Medical Faculty, Gadjah Mada University
Dr Sardjito General Hopital, Yogyakarta.

Background

Acute rheumatic fever is a multisystem inflammatory disease which occurs as delayed sequelae to group A streptococcal pharyngitis. Although the acute illness causes considerable morbidity, and some mortality, the major clinical and public health effects derive from long-term damage to the heart valves, i.e., rheumatic heart disease (RHD). Over the past century, as living conditions have become more hygienic and less crowded, and nutrition and access to medical care have improved, acute rheumatic fever (ARF) and RHD have become rare in developed countries. But, rheumatic fever or rheumatic heart disease is the commonest cardiac disease in children and young adults and remains a major public health problem in developing countries.2 The important clinical manifestations are migratory polyarthritis, carditis, chorea, subcutaneous nodules and erythema marginatum occurring in various combinations. Sydenham’s chorea still often unrecognized as a clinical manifestation of acute rheumatic fever so patients got late diagnosis and therapy 5 Key word : Chorea Sydenham, Acute Rheumatic Fever

Case An eight-year old girl came to the doctor with chief complain all extremity moved involuntarily. The symptom started since two months before hospitalized. Involuntary movement began in III and V fingers with dancing movement and then worsen to lower extremity. But it disappeared when she slept. There was also an appearance of red spot in extremity that already resolve, but she didn’t suffer from subcutan nodules or swelling joint. She also has history of fever and sore throat a year ago.

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Fig.1 involuntary movement in 8 YO girl

First she was taken to private hospital in Yogyakarta and performed EEG. The result was an epileptiform appearance and she was diagnosed as focal epilepsy dd hemichorea then got therapy valproic acid 7 mg/bw/day. After a month of treatment, the involuntary movement get worsen, the child also become unable to walk but still able to speak, then the parents bring the child to pediatric neurology subdivison in General hospital Jakarta, here she was diagnosed as involuntary movement and got therapy Haloperidol 0,01 mg/bw/day, the epilepsy drug from the previous hospital has been stopped. After two weeks of treatment the complain of involuntary movement get worsen, the child become unable to walk and to speak. The parents then bring the child to Dr Sardjito General Hospital Yogyakarta. From physical examination found the girl was afebril, chorea in all extremity, inability to speak and to walk. Fast pulse rate, normal 1st and 2nd heart sound, pansystolitic murmur 1-2/6 degree in apex. Neurological status within normal limit. There wasn’t macular rash in the skin, sub cutan nodules or sign of arthritis i.e pain and swelling of ankle joint. Laboratory findings showed increased blood sedimentation rate (38 mm/hour), CRP (18) and positive of ASO. The throat swab showed negative growth for streptococcus.

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Fig. 2 ECG : Sinus tachycardia and prolonged P-R interval (0,16 ms)

From Electrocardiograph showed sinus tachycardia and 1st degree AV block.

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From Chest X-Ray showed cardiomegaly

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Fig 4. Echocardiography : Anterior mitral valve prolaps

From Echocardiograph showed mild anterior mitral valve prolapse and severe mitral regurgitation. Based on modification of Jones criteria, she was fullfilled the diagnosis of Acute Rheumatic Fever and got treatment with penicillin, prednisone, aspirin, captopril and haloperidol. After two weeks of treatment, involuntary movement diminished, she was able to speak normally and walk by herself and there was a decreasing of blood sedimentation rate.

Discussion ARF is an auto-immune response to bacterial infection with group A streptococcus (GAS). People with ARF are often in great pain and require hospitalisation. Recurrences of ARF may cause further valve damage, leading to steady worsening of RHD. ARF is predominantly a disease of children aged 5–14 years, The prevalence of RHD peaks in the third and fourth decades. Therefore, although ARF is a disease with its roots in childhood, its effects are felt throughout adulthood, especially in the young adult years when people might otherwise be at their most productive. 1The diagnosis of ARF is guided by the Jones criteria, that were introduced in 1944. The criteria divide the clinical features of ARF into major and minor manifestations, based on their prevalence and specificity. Arthritis is the most common manifestation, present in 80% of patients. It is described as painful, migratory and transient. Knees and ankles are more frequently affected. Carditis occurs in 40-75% of patients in the first 3 weeks of the illness. Death may occur in the acute phase. Erythema marginatum and subcutaneous nodules are rare, less than 10% of patients are affected. Sydenham’s chorea is a rare presentation, affecting less than 5% of patients. 5 Table 1. Jones criteria (revised) Guidelines for the diagnosis of acute rheumatic fever 3 Diagnosis is established if we found : two major manifestation or one major + two minor manifestation.3 But because Sydenham’s chorea may occur after a prolonged latent period following group A streptococcus (GAS) infection, the diagnosis of ARF under these conditions does not require the presence of other manifestations or elevated plasma streptococcal antibody titres. During recent outbreaks of ARF in the USA, up to 71% of patients with chorea had carditis. Approximately 25% of patients with “pure” chorea also eventually develop RHD. This is explained by the finding that over 50% of patients with chorea, but without cardiac murmurs, have echocardiographic evidence of mitral regurgitation. 1 In our patient found Sydenham’s chorea with carditis, elevated of ESR, CRP, Prolonged PR Interval and positive ASO and from echocardiography found mild anterior mitral valve prolapse and severe mitral regurgitation. Sydenham’s chorea was described by Thomas Sydenham in 1686 and it has been associated with rheumatic fever since 1956. It is a major manifestation of rheumatic fever and according to the latest modification of the Jones criteria in 1992, a criterion sufficient even by itself for the diagnosis of the disease. 4The main feature in Sydenham’s chorea is involuntary movements. These can be generalised or unilateral. These movements occur at rest, may start suddenly or gradually, and are exacerbated by stress. They disappear during sleep. Usually the patient has abnormal neurological signs with hypotonia and motor restlessness which can lead to coordination problems, gait disturbances and speech impairment. As a result, the activities of daily living can be severely disrupted. The differential diagnoses of Sydenham’s chorea include atypical seizures, tics disorders, degenerative or neurometabolic causes like Huntington disease, Wilson’s disease, autoimmune diseases like systemic lupus erythematosis, drugs (phenytoin, amintriptyline), hormonal-induced causes like oral contraceptive pills, pregnancy/chorea gravidum 5The majority of patients are between 5-15 years of age, with girls predominating over the age of 11 years, thereby suggesting important hormonal influences. The duration of Sydenham’s chorea can last for 4-6 months, however, it can range from as little as one week up to over 2 years. It is believed that Sydenham’s chorea occurs secondary to an autoimmune reaction towards the host’s central nervous system, particularly the basal ganglia and its circuits. 5 There are four main step for the treatment of Acute rheumatic fever. 3 Step I : Primary prevention, The goal relies on the correct diagnosis of group A streptococcal infection and effective treatment for eradicating the organisms with appropriate antimicrobial agent. Group A beta-haemolytic streptococcus remains exquisitely sensitive to penicillin, which is still the antibiotic of first choice. 3 Step II – Anti inflammatory treatment. For arthtritis only can be given aspirin 75 – 100 mg/bw/day and for carditis can be given prednisolon 2 mg/ bw/ day for two weeks than tapp off for two weeks and while tappering off added aspirin 75 mg/bw/day for two weeks than continue aspirin alone 100 mg/ bw/day for another 4 weeks. 3Step III- Supportive management & management of complications. For example Rest to joints & supportive splinting, treatment of congestive cardiac failure (digitalis,diuretics).3 Treatment of chorea (diazepam or haloperidol), Haloperidol acts by blocking the dopamine receptors and it has been found to decrease the severity of choreic movements. Most patients respond to an initial dose of 0.5-1 mg/day with increments of 0.5 mg-1 mg/day every 3 days up to a maximum of 5 mg/day. In our patient, she responded to the haloperidol quite quickly and there were no observable side-effects. 5 Step IV- Secondary prevention (prevention of recurrent attacks) 3 *In high-risk situations, administration every 3 weeks is justified and recommended There is no consensus on the duration of antibiotic prophylaxis. In developed countries where the incidence of rheumatic fever is low, life-long prophylaxis is probably not necessary. It is reasonable to stop prophylaxis in patients who have reached their early 20s, more than 5 years past their last attack, and are free of rheumatic heart disease. The American Heart Association recommends prophylaxis to be continued at least 10 years after the last episode of rheumatic fever or until the patients are well into adulthood. 5 ConclusionSydenham’s chorea is a rare presentation of acute rheumatic fever, early detection as clinical manifestasion of Acute Rheumatic Fever is important for early management

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4. Terreri, MTRAs, Roja SC, Len CA. Sydenham’s chorea – clinical and evolutive characteristics. Sao Paulo Med J/Rev Paul Med 2002;120(1):16-9.5.
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