Antiphospholipid antibody-associated chorea

To describe the clinical features, treatment, and outcomes of patients with antiphospholipid antibody (aPL)-associated chorea.

The study cohort consisted of consecutive patients with chorea evaluated between 1990 and 2005 with documented aPL at time of their neurologic diagnosis.

Eighteen patients were identified, 4 with systemic lupus erythematosus (SLE). The 14 non-SLE patients experienced 1.6 vascular thromboses/pregnancy losses per person, while patients with SLE experienced 0.5 events/person. Four non-SLE patients (29%) and no SLE patients met criteria for antiphospholipid antibody syndrome (APS). None of these 4 tested positive for IgM anticardiolipin antibody (aCL). In contrast, 10 (71%) non-APS patients tested positive for IgM aCL. Chorea was most often bilateral, mild to moderate, and occurred once with a median age at onset of 44 and 33 years in non-SLE and SLE patients, respectively. Therapy included immunosuppression in 3 (21%) non-SLE patients and in all SLE patients. Antidopaminergic agents were used in 7 (39%). All patients responded to treatment. Five patients received anticoagulation for thrombosis and 2 died of bleeding complications, both non-SLE patients.

aPL-associated chorea occurs most often in women and severity is mild to moderate. Clinical expression of chorea does not differ between those with and without SLE. Anticoagulation should be reserved for thrombosis treatment and not simply for chorea in the presence of aPL, as 2 patients died of bleeding. The absence of IgM aCL in patients with APS supports prior evidence that IgG aCL and lupus anticoagulant may be the more clinically relevant antibodies for thrombosis. However, IgM aCL may be important in patients with chorea.