View source for Sydenham's chorea

==Background==
*Also known as rheumatic chorea
*One clinical manifestation of acute [[rheumatic fever]]
*Most common type of acquired chorea in childhood
*Movement disorder characterized by chorea, emotional lability, and hypotonia

===Epidemiology===
*More common in resource limited settings
*Chorea reported in 18-36% of cases of [[rheumatic fever]] in the US <ref> Veasy LG, Tani LY, Hill HR. Persistence of acute rheumatic fever in the intermountain area of the United States. J Pediatr. 1994;124(1):9-16. doi:10.1016/s0022-3476(94)70247-0 </ref> <ref> Centers for Disease Control (CDC). Acute rheumatic fever--Utah. MMWR Morb Mortal Wkly Rep. 1987;36(8):108-115. </ref> <ref> Stollerman GH. Rheumatic fever. Lancet. 1997;349(9056):935-942. doi:10.1016/S0140-6736(96)06364-7 </ref> <ref> Carapetis JR, Currie BJ. Rheumatic chorea in northern Australia: a clinical and epidemiological study. Arch Dis Child. 1999;80(4):353-358. doi:10.1136/adc.80.4.353 </ref>
*Greatest incidence in patients aged between 5-13 <ref> Zomorrodi A, Wald ER. Sydenham's chorea in western Pennsylvania. Pediatrics. 2006;117(4):e675-e679. doi:10.1542/peds.2005-1573 </ref> <ref> Demiroren K, Yavuz H, Cam L, Oran B, Karaaslan S, Demiroren S. Sydenham's chorea: a clinical follow-up of 65 patients. J Child Neurol. 2007;22(5):550-554. doi:10.1177/0883073807302614 </ref>

==Clinical Features==
*[[Chorea]]: rapid, random, irregular movements of face and extremities, restlessness of the truck
*Emotional lability
*Hypotonia
*Symptoms usually continuous while awake, worse with action, and improved with sleep
*Typically generalized, but may favor one side
*Typically gradual onset
*Onset usually between 1-8 months after inciting infection <ref> Eshel G, Lahat E, Azizi E, Gross B, Aladjem M. Chorea as a manifestation of rheumatic fever--a 30-year survey (1960-1990). Eur J Pediatr. 1993;152(8):645-646. doi:10.1007/BF01955239 </ref>

==Differential Diagnosis==
{{Chorea DDX}}

==Evaluation==
===Physical Exam===
*Typical neurological exam findings 
**Normal mental status
**Normal cranial nerve and sensory exam
**Motor exam tests and findings
***Spooning - patient seated with arms fully extended forward from shoulders with pronated hands and fingers spread. Typical finding is symmetric hyperextension of the MCP joint (so the arms and hands look like spoons)
***Touchdown - patient raises arms and hands up fully extended with palms facing one another (as if making the "touchdown" sign). Typical finding is pronation of one or both hands and flexion of the elbows
***Milkmaid's grip - patient grips examiners index and middle fingers. Typical finding is partial release of grip and re-gripping, as if "milking the cow 
***Darting tongue - patient sticks tongue out from mouth. Typical finding is tongue alternately withdrawing and protruding because patient cannot maintain the motor command
*Exam should also search for other manifestations of acute rheumatic fever including carditis, migratory polyarthritis, subcutaneous nodules, and erythema marginatum

===Workup===
*Group A strep testing (i.e. with throat culture)
*Serum testing for antistreptolysin O and antideoxyribonuclease B 
*CRP, ESR
*Cardiac workup including EKG and echo
*Consider LP and/or neuroimaging if considering alternative diagnoses

==Management==
*Chronic antibiotic therapy to minimize recurrence and risk of heart disease
**Length of therapy depending on risk of recurrence and severity of disease
**Penicillin G is preferred agent (usually given IM every 21-28 days) <ref> Gerber MA, Baltimore RS, Eaton CB, et al. Prevention of rheumatic fever and diagnosis and treatment of acute Streptococcal pharyngitis: a scientific statement from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research: endorsed by the American Academy of Pediatrics. Circulation. 2009;119(11):1541-1551. doi:10.1161/CIRCULATIONAHA.109.191959 </ref>
*Chorea suppressing medication
**Typically low dose dopamine 2 receptor blocking agent such as fluphenazine, haloperidol, pimozide
**Other options include carbamazepine, clonidine, or guanfacine
*Immune-suppresing treatment may be considered
**Options ranging from prednisone to IVIG and plasmapheresis in severe cases


==Disposition==
*Neurology consultation or referral 
*Decision regarding final disposition should be made based on overall clinical picture

==See Also==
*[[Chorea]]
*[[Acute rheumatic fever]]

==External Links==

==References==
<references/>

[[Category:Pediatrics]]
[[Category:ID]]
[[Category:Neurology]]