Chorea and rapidly progressive subcortical dementia in antiphospholipid syndrome

Abstract

Antiphospholipid antibodies are associated with a variety of neurologic manifestations, both in patients with and without concomitant systemic lupus erythematosus. We report a patient in whom chorea and rapidly progressive subcortical dementia developed in the setting of persistently high titers of antiphospholipid antibodies. While some manifestations of antiphospholipid syndrome can be clearly linked to vascular thrombosis, it is not known whether this is also true for patients affected with chorea, dementia, or both. In our patient, serial magnetic resonance imaging showed the progressive development of deep white matter lesions but no cortical infarcts. The development of widespread pulmonary arterial thrombosis and acute cerebral ischemia, evidenced clinically and on diffusion-weighted magnetic resonance imaging of the brain, provided indirect evidence for a thrombotic pathogenesis for this patient's neurologic disease. Anticoagulation should be considered as an adjunct to the treatment of patients with antiphospholipid antibodies and chorea or subcortical dementia.