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.
METHODS: 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.
RESULTS: 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% of patients who were tested by an elevated
antistreptolysin O titer (n = 53), an elevated anti-
deoxyribonuclease B titer (n = 7), a positive streptozyme (n = 53), or acute throat
infection with Streptococcus pyogenes (n = 19). A total of 52 neuroimaging tests were obtained from 46 patients with SC. In patients with SC, brain MRI was abnormal in 8 of 32 patients, and brain computed tomography was abnormal in 1 of 20 patients. Abnormalities did not aid in diagnosis and included nonspecific increased signal (n = 2), nonspecific punctate lesions (n = 2), asymmetry of the hippocampal fissures, unrelated petrous bone anomaly,
Arnold Chiari malformation, and
medulloblastoma in a macrocephalic patient. Three patients with
chorea that was not attributed to ARF had atypical presentations: 1 developed
chorea after a perioperative hypoxic/ischemic central nervous system insult; 1 had an episode of disorientation,
aphasia, and transient facial droop (angiography showed basal ganglia
infarct); and 1 with hemichorea had basal
ganglion infarct seen on MRI.
CONCLUSIONS: Ninety-six percent of children who had acute
chorea and presented to a large children's hospital in an area that is endemic for ARF had SC. These patients had characteristic demographic and clinical features of SC. The most common concurrent major Jones criterion was
carditis.
Arthritis,
erythema marginatum, and subcutaneous nodules were uncommon in this population. Neuroimaging was obtained in 58% of patients with SC and did not aid in any of their diagnoses. The 3 patients with
chorea that was not caused by SC had histories that were atypical for SC and warranted neuroimaging. SC can be readily diagnosed on the basis of history, physical examination, and laboratory evaluation; neuroimaging is not necessary and should be reserved for patients who have an atypical presentation, including hemichorea.