Primary Antiphospholipid Syndrome Presenting Dementia and Brain Atrophy

J Korean Neurol Assoc > Volume 11(4); 1993 > Article

Journal of the Korean Neurological Association 1993;11(4): 561-566.

치매와 뇌위축을 보인 원발성 항인지질항체 증후군 (Primary Antiphospholipid Syndrome)

김현각, 이재홍,이명종

울산대학교 신경과. 서울중앙병원 신경과.

Primary Antiphospholipid Syndrome Presenting Dementia and Brain Atrophy

Hyeon-Gak Kim, M.D., Jae-Hong Lee, M.D., Myoung-Chong Lee, M.D.

Department of Neurology, College of Medicine Asan University

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.