We analyzed the clinical, radiologic, and immunologic characteristics of 50 patients with
chorea and the
antiphospholipid syndrome (APS) (6 from our clinics and 44 from a MEDLINE computer-assisted review of the literature from 1985 through 1995). Forty-eight (96%) patients were female and 2 (4%) were male. Twenty-nine (58%) patients had defined
systemic lupus erythematosus (SLE), 6 (12%) had "lupus-like" syndrome, and 15 (30%) patients had "primary" APS. Mean age of patients in this series was 23 +/- 12 years (range, 6-77 yr); mean age at presentation of
chorea was 21 +/- 12 years (range, 6-77 yr). In 11 (22%) patients, the onset of
chorea was in childhood (6-14 yr), and in 2 (4%) patients it presented at 60 years or more. Six (12%) patients developed
chorea soon after they started taking
estrogen-containing
oral contraceptives, 3 (6%) developed
chorea gravidarum, and 1 (2%) patient developed
chorea shortly after delivery. Most patients (66%) presented only 1 episode of
chorea.
Chorea was bilateral in 55% of patients. Computed tomography and magnetic resonance imaging scans reported
cerebral infarcts in 35% of patients. The following
antibodies were detected:
lupus anticoagulant (92%),
anticardiolipin antibodies (91%),
antinuclear antibodies (82%), anti-
DNA (59%), anti-Ro (10%), anti-RNP (8%), anti-La (2%), and anti-Sm (2%). The
chorea in these patients responded to a variety of medications, for example,
steroids,
haloperidol, antiaggregants,
anticoagulants, or a combination of
therapy, usually prescribed in the presence of other manifestations of APS or SLE. However, many patients responded well to
haloperidol and to the discontinuation of
oral contraceptives if this was the precipitating factor.