|Paul J. Myers, DO||Lehigh Valley Health Network/USF MCOM, Department of Emergency Medicine, Allentown, Pennsylvania|
|Kathleen E. Kane, MD||Lehigh Valley Health Network/USF MCOM, Department of Emergency Medicine, Allentown, Pennsylvania|
|Bernadette G. Porter, BS||Lehigh Valley Health Network/USF MCOM, Department of Emergency Medicine, Allentown, Pennsylvania|
|Richard J. Mazzaccaro, MD, PhD||Lehigh Valley Health Network, Department of Pediatrics, Allentown, Pennsylvania|
Sydenham Chorea (SC) is an acute rheumatic fever complication. SC is the most common acquired childhood chorea, usually affecting children five to fifteen years of age.1 It occurs following an untreated group A streptococcal infection and a latent period of one to six months.1,2 Despite rheumatic fever diminishing, 18% to 36% of acute rheumatic fever patients develop SC.3 Full recovery often takes several months; some patients suffer permanent neurologic sequelae.1
An 11-year old male presented to the Emergency Department with two days of uncontrolled body twitching. The movements affected his right arm and leg, with occasional lip twitches; he experienced intermittent confusion and hyperactivity. The patient denied recent illness, but recalled a fever with headache and vomiting several months prior. Besides the above findings, his physical examination was normal.
The patient’s rapid streptococcal antigen test was negative, but his throat culture was positive for group A beta hemolytic Streptococcus. An anti-streptolysin O (ASO) titer resulted at 503 (reference range, <240 IU/mL). Symptoms progressed to include slurred speech, head jerking, awkward gait, and decreased right eye vision.
Several motor manifestations are pathognomonic: “Milkmaid’s grip” occurs when patients are unable to clench their fists, displaying as intermittent relaxation and tightening of hand grip.1 “Choreic hand” is “spooning” of the hand by wrist flexion and extension of the digits.1 The linked video demonstrates this patient’s motor manifestations (Video).
Evaluation of chorea in pediatric patients should include testing for group A streptococcal infection with throat culture and ASO titers. SC is key to diagnosing rheumatic fever and should prompt evaluation for rheumatic heart disease. This patient’s brain magnetic resonance imaging and electrocardiogram were normal, but his echocardiogram showed mild mitral regurgitation. Treatment included penicillin for ten days, instructions to get monthly bicillin injections through age twenty-one and a recommendation for lifelong antibiotic prophylaxis.
Supervising Section Editor: Sean O. Henderson, MD
Full text available through open access at http://escholarship.org/uc/uciem_westjem
Address for Correspondence: Bernadette Porter, EM Residency Suite, 2545 Schoenersville Road, Bethlehem, PA, 18017. Email: Bernadette.Gl-Porter@lvhn.org.
Submission history: Submitted July 9, 2014; Accepted August 20, 2014
Conflicts of Interest: By the WestJEM article submission agreement, all authors are required to disclose all affiliations, funding sources and financial or management relationships that could be perceived as potential sources of bias. The authors disclosed none.
- Weiner S, Normandin P. Sydenham chorea: a case report and review of the literature. Ped Emerg Care. 2007;23(1):20-4.
- Peña J, Mora E, Cardozo J, et al. Comparison of the efficacy of carbamazepine, haloperidol and valproic acid in the treatment of children with Sydenham’s chorea: Clinical follow-up of 18 patients. Arq Neuropsiquiatr. 2002; 60(2-B):374-77.
- van Immerzeel TD, van Gilst RM, Hartwig NG. Use of immunoglobulins in the treatment of Sydenham chorea. Eur J Pediatr. 2010; 169(9):1151–54.