Description, Causes and Risk Factors:
A postinfectious chorea appearing several months after a Streptococcal infection with subsequent rheumatic fever. The chorea typically involves the distal limbs and is associated with hypotonia and emotional lability. Improvement occurs over weeks or months and exacerbations occur without associated infection recurrence.
For many years Sydenham disease has been considered the prototype of an autoimmune disorder triggered by an infectious agent. Its de?ned association with rheumatic fever in 1838, and linkage to a preceding group A ?-hemolytic streptococcal (GABHS) infection, there are numerous unanswered questions about the underlying Pathology & pathophysiology of Sydenham disease. Recently, clari?cation of the underlying mechanism in Sydenham disease has assumed, renewed importance as the disorder has been proposed as a model for a spectrum of childhood neurobehavioral disorders, including tics and obsessive-compulsive disorder, termed PANDAS (Pediatric autoimmune neuropsychiatric disorders associated with Streptococcal infection).
Much of the literature concerning Sydenham disease is very old. The disease itself appears to have changed over time: it is substantially less common than it was but also symptoms tend to be less severe and relapses less frequent. This may be partially due to penicillin, but improved social conditions and a natural reduction in the virulence of the Streptococci have also played a role.
Pay careful attention to children's complaints of sore throats and get early treatment to prevent acute rheumatic fever. If there is a strong family history of rheumatic fever, be especially watchful, because your children may be more likely to develop this infection.
The symptoms of Sydenham disease range in severity from mild to completely incapacitating. A vague deterioration in the ability to perform everyday tasks is replaced by involuntary jerking movements that are most obvious in the extremities and face but are also present in the trunk. Twitching movements are more noticeable on the limbs of one side of the body. The muscles of speech and swallowing may also be affected. Irritability, anxiety, and emotional instability, chiefly episodes of crying initiated by trivial incidents, are also common symptoms.
Diagnosis of Sydenham disease must be done clinically. Patients presenting with Sydenham disease often show no signs of a fever and have little evidence of acute inflammation. While testing for a recent infection may seem like a promising diagnostic tool, a recent group A beta hemolytic streptococcal infection may not always be present. Streptococcal antibodies are useful in that they can indicate whether or not the patient is truly infected with the group A streptococcus and thus has acute rheumatic fever or if the infection is temporary. Unfortunately antibodies to group A streptococcus are only seen in 80% of patients.
Blood count may be normal and ESR and CRP returned to normal.
ASO titer and other serological tests described with rheumatic fever may still be helpful.
Antineuronal antibodies have been described, but they do not form part of routine diagnosis. The antibody titer falls as the disease improves and rises again in relapse.
MRI studies have shown enlargement of caudate, putamen and globus pallidus in patients with the diseaseand changes in the basal ganglia.
By the time that chorea presents it is unusual to be able to isolate GABHS from the pharynx.
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 (HaldolSM) but valproate is now most specialists' first-line choice. Dopamine receptor blocking drugs such as pimozide (OrapTM) 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.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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