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 [1] [2] [3] [4]
  • Greatest incidence in patients aged between 5-13 [5] [6]

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 [7]

Differential Diagnosis

Causes of Chorea

Behavioral

  • Tourette
  • Behavioral or emotional disorders

Autoimmune or inflammatory

Cerebrovascular

Infectious

  • AIDS-related
  • Cruetzfeld-Jakob disease or other prion disease
  • Diphtheria
  • Legionnaire disease
  • Lyme disease
  • Malaria
  • Neurocysticercosis
  • Neurosyphilis
  • Progressive multifocal leukoencephalopathy
  • Toxoplasmosis
  • Tuberculosis

Metabolic/Endocrine

Drugs & Toxins

Paraneoplastic

  • Neoplasm with basal ganglia involvement
  • Small-cell lung cancer

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) [11]
  • 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

External Links

References

  1. ↑ 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
  2. ↑ Centers for Disease Control (CDC). Acute rheumatic fever--Utah. MMWR Morb Mortal Wkly Rep. 1987;36(8):108-115.
  3. ↑ Stollerman GH. Rheumatic fever. Lancet. 1997;349(9056):935-942. doi:10.1016/S0140-6736(96)06364-7
  4. ↑ 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
  5. ↑ Zomorrodi A, Wald ER. Sydenham's chorea in western Pennsylvania. Pediatrics. 2006;117(4):e675-e679. doi:10.1542/peds.2005-1573
  6. ↑ 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
  7. ↑ 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
  8. ↑ Miranda M, et al. Oral contraceptive induced chorea: another condition associated with anti-basal ganaglia antibodies. J Neurol Neurosurg Psychiatry 2004; 75(2): 327-328
  9. ↑ Bordelon YM, et al. Movement disorders in pregnancy. Semin Neurol 2007; 27(5):467-475
  10. ↑ Chang MH, et al. Non-ketotic hyperglycaemic chorea: a SPECT study. J Neurol neurosurg Psychiatry 1996; 60(4): 428-430
  11. ↑ 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
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