Sydenham's Chorea in Western Pennsylvania

Chorea is characterized by involuntary, fleeting, irregular, nonrhythmic movements that flow from 1 body region to another. There are many causes of childhood chorea, including cerebrovascular accidents, collagen vascular diseases, drug intoxication, hyperthyroidism, Wilson's disease, Huntington's disease, and infectious agents. Although Sydenham's chorea (SC), a nonsuppurative sequela of group A streptococcal infection, is known to be a common cause of chorea, multiple laboratory and radiographic studies are often obtained to determine the cause of pediatric chorea. We conducted a retrospective chart review to determine the causes of childhood chorea seen in a large children's hospital in an area endemic for acute rheumatic fever (ARF). The utility of neuroimaging in establishing a final diagnosis of SC is discussed. Patients who received a diagnosis of chorea between 1980 and 2004 at the Children's Hospital of Pittsburgh were identified from databases that are maintained by the divisions of Infectious Diseases and Cardiology and from the hospital's medical records department. Charts were abstracted retrospectively. All patients who had new-onset chorea and did not have any underlying neurologic disorders were included in this study. Patient demographic, clinical, laboratory, and imaging information was analyzed. Follow-up information was not found consistently and therefore was not included. Charts of patients with questionable diagnoses were reviewed with a neurologist. A total of 144 patients met the search criterion. Eleven patients had incomplete charts, and 6 charts could not be located. Thirty patients were excluded because they had preexisting neurologic diagnoses, eg, cerebral palsy. Fifteen patients were excluded because they were miscoded as having chorea. Eighty-two patients had new-onset chorea. The cause was SC (n = 79), postoperative cerebral ischemia (n = 1), and basal ganglion infarct (n = 2). Seventy-six (71%) children with SC were female. The mean age of presentation was 9.8 years (range: 5-14.5 years). Chorea was unilateral in 23 (30%) patients. Family history of ARF existed in 30% of patients. Neurologic symptoms of SC included behavior change (46%), dysarthria (67%), gait change (51%), deterioration of handwriting (29%), and headache (11%). Nonneurologic manifestations of ARF were carditis (44%), arthritis (11%), erythema marginatum (3%), and subcutaneous nodules (0%). Antecedent group A streptococcal infection was documented in 99%