Primary Antiphospholipid Syndrome Presenting Dementia and Brain Atrophy

ABSTRACT

Antiphospholipid antibody syndrome (APS) has been considered as a distinct clinical entity, usually associated with systemic lupus erythematosus (SLE) or with other connective tissue disease. Prominent clinical features include venous thrombosis, arterial occlusions, thrombocytopenia, hemolytic anemia, recurrent fetal loss, livedo reticularis and neurologic manifestations. Antiphospholipid antibodies (aPL) represent a spectrum of phospholipid-binding antibodies, including the lupus anticoagulant(LA), anticardiolipin antibody (aCL), and the biologic false-positive serologic test for syphilis (BFP-STS). Primary antiphospholipid syndrome (PAPS) which has been recently described is a complication of autoimmune aPL with lack of serologic and clinical features of SLE. We present a young female patient with PAPS who showed progressive dementia, diffuse cerebral atrophy, renal arteriolar occlusions, livedo reticularis, and valvular heart disease. To our knowledge, our patient is the first described with the clinical and mangetic resonance imaging(M-RI) findings in this entity.