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Sydenham Chorea - A neurological disorder of childhood resulting from infection via Group A beta-hemolytic streptococcus (GABHS), the bacterium that causes rheumatic fever. Sydenham chorea is characterised by rapid, irregular, and aimless involuntary movements of the arms and legs, trunk, and facial muscles.

Affects girls more often than boys and typically occurs between 5 and 15 years of age.

Some will have a sore throat several weeks before the symptoms begin, but the disorder can also strike up to 6 months after the fever or infection has cleared.  

Symptoms can appear gradually or all at once and may include:

 

The symptoms of sydenham chorea can vary from:

A halting gait and slight grimacing.

To

Involuntary movements that are frequent and severe enough to be incapacitating.  

Random, writhing movements of chorea are created by an auto-immune reaction to the bacterium that interferes with the normal function of a part of the brain (the basal ganglia) that controls motor movements.  

The use of antibiotics to treat streptococcus infections, rheumatic fever, and consequently sydenham chorea , has helped to lessen the problem in North America and Europe. However, the problem remains prevalent across what is considered to be developing (ignored) areas of the world .

There is no specific treatment for sydenham chorea. However, those with the mildest form, bed rest during the period of active movements may be considered. However if severity of movements interferes with rest, drugs may be used as a controlling mechanism.

 

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It may recommended that children who have had sydenham chorea  be prescribed penicillin over the course of 10 years to prevent additional manifestations of rheumatic fever. However, long term use of antibiotics may create other problems.

Most may recover completely from, although a small number will continue to have disabling, persistent chorea despite treatment.  

The duration of symptoms varies, generally from 3 to 6 weeks, but some will have symptoms for several months.  Cardiac complications may occur in a small minority of children, usually in the form of endocarditis.  In many of the those with sydenham chorea the disease will recur, typically 1 ½ to 2 ½ years after the initial attack.  

There is noted an association between recurrent sydenham chorea and the later development of the abrupt onset forms of:

Known as paediatric autoimmune neuropsychiatric disorders associated with streptococcus infection.

 

Question

What is the nature of the association and the biological pathways that connect streptococcal infection, autoimmune response, and the later development of these specific behavioural disorders?

 

Chorea Disease - Site Page

Chorea Gravidarum - Site Page

Wilson’s Disease - Site Page

Inflammation - Site Page

 

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Sydenham Chorea