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Download Citation on ResearchGate | Corea de Sydenham: presentación de un caso tratado con carbamazepina con excelente respuesta clínica | Sydenham’s. Request PDF on ResearchGate | On Dec 1, , S. Fernández Ávalos and others published Corea de Sydenham: un pasado aún presente. Corea de Sydenham: presentación de un caso tratado con carbamazepina con excelente respuesta clínica. Sydenham’s chorea: Report of a case treated with.

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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 restsecondary palliation symptomatic medication and supportive social care. Despite some progress in understanding the pathogenesis and pathophysiology, the treatment remains largely symptomatic and not evidence based.

Medline search extended from until From these, articles with relevance to the review topic were selected and 86 of these were in languages other than English and no abstracts were available. One hundred and nine articles were studied in more detail and 59 articles were referenced in this review. Twenty articles which focused on the management are summarized in Table 1. There were no Cochrane reviews. A search in NLM Gateway http: Case reports, clinical trials and review articles were selected and if available were studied and referenced.

Relevant articles in English were reviewed in full and those in other languages were assessed based on the abstracts if available. Rheumatic fever is currently the major cause of acquired heart disease in children [ Manyemba and Mayosi, ].

Hence, when SC is diagnosed, treatment strategies must include the prevention of rheumatic heart disease. The incidence and prevalence of acute rheumatic fever and rheumatic heart disease in industrialized countries with market economies has been reduced significantly. In resource-poor countries, the burden of disease remains high. A minimum of The prevalence of rheumatic heart disease in children aged 5—14 years is highest in sub-Saharan Africa 5—7 perthe Pacific and indigenous populations of Australia and New Zealand 3—5 per and South—Central Asia 2.

The World Health Organization WHO criteria using an echocardiogram to diagnose subclinical rheumatic heart disease is suboptimal when compared with the combined criteria of valve morphology in addition to the assessment of regurgitation [ Marijon et al. As such, prevalence data based on the WHO criteria alone will underestimate the true figures.

SC is the commonest form of acquired chorea. This is supported by case series from Tunisia, Iran and Turkey [ Demiroren et al. Large series over prolonged periods have noted a decline in the number of cases [ Nausieda et al. SC is an antineuronal antibody-mediated neuropsychiatric disorder [ Husby et al.

Antibodies which arise in response to group A beta-haemolytic streptococcus GABAS infection cross react with epitopes on neurons within the basal ganglia, frontal cortex and other regions. Children with SC have elevated serum antineuronal antibody titres [ Swedo, ]. Immunoglobulin G from patients with SC cross reacts specifically with neuronal cytoplasmic antigens in subthalamic and caudate nuclei [ Wolf and Singer, ]. A cerebral arteritis with cellular degeneration occurs.

Hyperaemia, endothelial swelling, perivascular round cell infiltration and petechial haemorrhage are found on histological cofea [ Aron et al. These changes are a result of the autoimmune process and result in dopaminergic dysfunction [ Nausieda et al. These insights are leading to targeted therapeutic interventions rather than symptomatic management alone.

Movements are controlled by two main systems: The second system b is relevant to movement disorders and it consists of three main pathways [ Comings, ]. These core are mainly dopamine controlled and there is significant cross over between them. The basal ganglia is best considered as a relay station containing neurones with many different neurotransmitters that regulate and integrate sensory, emotional and voluntary inputs controlling motor activities.


In addition, the limbic system and prefrontal cortex are sycenham in the ckrea of attention and emotion. In summary, movements, attention and emotion all result from a complex interaction of neurotransmitters in the basal ganglia, limbic systems sydenhqm prefrontal cortex [ Comings, ]. Gamma aminobutyric acid GABA dopamine, noradrenaline and serotonin all play a role. Symptomatic treatments act via these neurotransmitters [ Edgar, ]. Certain medications act on the dopamine system, for example haloperidol, tetrabenazine and pimozide block dopamine receptors.

sydeham Benzodiazepines facilitate the action of GABA. Valproate enhances the action of GABA. Carbamazepine modifies sodium channels which increase neuronal stability and it also acts by blockage at the level of dopaminergic postsynaptic receptors [Carapetis et al. Carbamazepine can increase GABA levels or decrease glutamatergic activity, restoring disrupted interplay between basal ganglia and the cerebral cortex ee further explains its role in the management of chorea [ Feigin et al.

The clinical features of SC include both neurological abnormalities and psychiatric coea. The former comprise involuntary choreatic movements, voluntary movement incoordination, muscular weakness and hypotonia [ Gowers, ].

Psychiatric disorders include emotional lability, hyperactivity, distractibility, obsessions and compulsions [ Garvey and Swedo, ]. This constellation of features results in difficulty in the execution of activities of daily living with the result that the condition impacts negatively on the quality of life of children.

At the peak of their illness children may become totally dependent on their families. Choreatic movements are involuntary, irregular, purposeless, non-rhythmic, abrupt, rapid and unsustained [ Edgar, ]. Movements disappear with sleep and rest [ Edgar, ]. Voluntary movements make the chorea worse and are themselves incoordinated making activities such as writing, dressing and eating difficult [ Garvey and Asbahr, ].

The hypotonia and weakness have a range of severity from mild to severe. The severe form is termed chorea mollis or chorea paralytica and vorea be confused with dd clinical appearance of a stroke [ Garvey et al. Such children d be mute and confined to a wheel chair [ Garvey and Swedo, ].

Obsessions may include causing harm to loved ones, separation anxiety and fear of contamination, resulting in compulsive washing [ Swedo, ]. A change in behaviour may precede the chorea [ Asbahr et al. Classical descriptions of SC indicate that it is benign and self-limiting [Carapetis et al.

At best the condition lasts for 6 months but more usually it has a relapsing course for up to 2 years [ Paz et al. At worst it may evolve into a chronic movement disorder [ Paz et al. Variation in the duration of chorea and a lack of methods to quantify the severity together with the lack of a therapeutic index has made evaluation of therapy difficult [ Aron et al.

Notably this scoring system does not include the neuropsychiatric implications of the disorder. Based on the aetiology, pathology, pathophysiology and clinical presentation of SC, the treatment has four main tenets: Evidence of recent streptococcal infection as reflected by a raised antistreptolysin titre or a raised anti-D-Nase titre varies in different regions, but was demonstrated in studies from Dydenham and Australia [ Demiroren et al.

Sydenham’s chorea – Wikipedia

Thus, treatment with penicillin is mandatory to eliminate the streptococcus. Secondary prophylaxis with long-term penicillin is primarily given to protect the heart; whether sydenhwm prevents relapses of SC is still debatable [ Berrios et al. This is largely based on anecdotal evidence but it is common clinical practice.

Physical activity should be restricted until the acute phase reactants have normalized and then restarted gradually [ Cilliers, ]. Adverse outcomes and a chronic relapsing course of SC were more common in children who sdyenham not receive 10 days of penicillin, hospitalization and bed rest [ Walker et al. Prevention of recurrent attacks of rheumatic fever is the most cost-effective way of preventing rheumatic heart disease.


Penicillin remains the antibiotic of choice [ Cilliers, ]. Patients must also be empowered to seek primary treatment for future syddenham sore throats.


Secondary sydejham prophylaxis is given to protect the heart and does not necessarily prevent relapses of abnormal movements [ Berrios et al. Despite more than three centuries of experience there is still clrea globally accepted protocol to treat SC.

Treatments have ranged from bleeding, purging, hyperthermia, anti-inflammatory agents, sedation and currently a variety of oral pharmacotherapy is prescribed [ Gordan, ]. The use of sedation was based on the observation that excitement and stress aggravated symptoms whilst sleep abolishes them.

Barbiturates, bromides and chloral hydrate were therefore utilized in the s [ Aron et al. The increased understanding of the pathophysiology has lead to the use of agents which affect the neurotransmitters dopamine and GABA.

Dopamine receptor antagonists include ed, 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. Table 1 summarizes the larger cases series which have addressed the efficacy and safety of treatment options for SC.

In a review of 65 children with SC from Turkey, comparisons of treatments showed that haloperidol was superior sydengam pimozide for controlling chorea, both in time to onset of recovery and time to complete remission, but haloperidol was associated with wydenham side effects [ Demiroren et al. In a retrospective study of 42 patients with SC from South Africa, 39 were treated with haloperidol.

Twenty five of these patients reported side effects severe enough to cause the physician or parent to discontinue treatment or to reduce the dose [ Walker et al. In a retrospective study, patients with SC had an increased susceptibility to develop drug-induced Parkinsonism on neuroleptics [ Teixeira et al. Underlying nigrostriatal dysfunction in SC was hypothesised. Pimozide has a more selective antidopaminergic action with fewer side effects and as such is cotea [ Demiroren et al.

However, in many resource-poor countries haloperidol is first-line therapy as it is cost effective and readily available. Cardoso recommended that patients who present with severe chorea particularly coorea paralytica, in which the muscle tone is so decreased that patients are bedridden should be treated with risperidone [ Cardoso, ]. The efficacy of this agent would be based on its role as a dopamine D2 receptor blocker [ Edgar, ]. GABA is a neurotransmitter which inhibits dopaminergic corex [ Edgar, ].

The intervention was considered croea and safe in the treatment of SC [ Sabui and Pant, ]. Dorea small case series reported similar results [ Davutoglu et al. However, other researchers did not find valproic acid to be effective [ Appleton and Jan, ]. Valproic acid is recommended as the first-line agent in the treatment of SC [ Cardoso, ], especially in severe cases of SC where trials with haloperidol and diazepam have failed [ Alvarez and Novak, ].

Carbamazepine is used in some institutions to treat SC [Carapetis et al. A group from Venezuela compared the efficacy of carbamazepine, haloperidol and valproic acid in the treatment of 18 patients with SC.