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Sydenham's chorea
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Sydenham's chorea : ウィキペディア英語版
Sydenham's chorea

Sydenham's chorea or chorea minor (historically referred to as Saint Vitus Dance) is a disorder characterized by rapid, uncoordinated jerking movements primarily affecting the face, hands and feet.〔(NINDS Sydenham Chorea Information Page ) Saint Vitus Dance, Rheumatic Encephalitis from the National Institute of Neurological Disorders and Stroke. Accessed April 26, 2008〕 Sydenham's chorea (SC) results from childhood infection with Group A beta-haemolytic Streptococcus〔(Sydenham's chorea: ) Symptoms/Findings from WeMOVE.Org Accessed April 26, 2008〕 and is reported to occur in 20–30% of patients with acute rheumatic fever (ARF). The disease is usually latent, occurring up to 6 months after the acute infection, but may occasionally be the presenting symptom of rheumatic fever. Sydenham's chorea is more common in females than males and most patients are children, below 18 years of age. Adult onset of Sydenham's chorea is comparatively rare and the majority of the adult cases are associated with exacerbation of chorea following childhood Sydenham's chorea.
==Presentation==
Sydenham's chorea is characterized by the abrupt onset (sometimes within a few hours) of neurologic symptoms, classically chorea, usually affecting all four limbs. Other neurologic symptoms include behavior change, dysarthria, gait disturbance, loss of fine and gross motor control with resultant deterioration of handwriting, headache, slowed cognition, facial grimacing, fidgetiness and hypotonia.〔〔 Also, there may be tongue fasciculations ("bag of worms") and a "milk sign", which is a relapsing grip demonstrated by alternate increases and decreases in tension, as if hand milking.〔(Medscape > Pediatric Rheumatic Heart Disease Clinical Presentation > Noncardiac manifestations ). Author: Thomas K Chin, MD; Chief Editor: Stuart Berger, MD. Updated: Aug 4, 2010〕
Non-neurologic manifestations of acute rheumatic fever are carditis, arthritis, erythema marginatum, and subcutaneous nodules.〔
The PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections) syndrome is similar, but is not characterized by Sydenham's motor dysfunction. PANDAS presents with tics and/or a psychological component (e.g., OCD) and occurs much earlier, days to weeks after GABHS infection rather than 6–9 months later. It may be confused with other conditions such as lupus and Tourette syndrome.
Movements cease during sleep, and the disease usually resolves after several months. Unlike in Huntington disease, which is generally of adult onset and associated with an unremitting autosomal dominant movement disorder and dementia, neuroimaging in Sydenham's chorea is normal and other family members are unaffected. Other disorders that may be accompanied by chorea include abetalipoproteinemia, ataxia-telangiectasia, Fahr disease, glutaric aciduria, Wilson disease, Lesch-Nyhan syndrome, hyperthyroidism, lupus erythematosus, pregnancy (Chorea gravidarum), and certain anticonvulsants or psychotropic agents.

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