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KMID : 0960920020010010064
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Dementia and Neurocognitive Disorders
2002 Volume.1 No. 1 p.64 ~ p.67
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Two Cases of Young Adults with Antiphospholipid Antibody Syndrome Manifesting as Progressive Dementia and Cortical Atrophy
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Lee Chang-Seon
Kim Heyn-Kak
Lee Jae-Hong
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Abstract
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Primary antiphospholipid syndrome(PAPS) is a complication of autoimmune antiphospholipid antibodies with lack of serologic and clinical features of systemic lupus erythematosus or other collagen vascular disorders. We have observed progressive dementia in two patients who had antiphospholipid antibodies without any other cause of dementia. One patient is 31-year-old female who presented with progressive cognitive impairment of 2 year’s duration and decreased hand dexterity. Her Mini-Mental State Exam(MMSE) score was 20. Livedo reticularis was noted on both forearms and knees. She was tested positive for VDRL, lupus anticoagulant and anti-cardiolipin an tibody. FTA-ABS and all other antibodies for collagen vascular disease were negative. Brain MRI showed mild to moderate cortical atrophy with no definite parenchymal lesion. Her dementia deteriorated despite corticosteroid therapy and follow-up MRI revealed marked brain atrophy with ventricular dilatation. The other patient, a 36-year-old male, presented with a one-year history of memory impairment and mental slowing. Laboratory tests were significant only for lgG anti-cardiolipin antibody. His MMSE score was 26. Brain MRI showed mild atrophy with some small T2 signal intensities in the centrum semiovale. Cerebral angiogram was unrevealing. We suggest that PAPS may produce progressive dementia mainly by causing cortical atrophy. PAPS should be suspected in the diffrential diagnosis of young adult dementia.
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KEYWORD
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Antiphospholipid antibody, Dementia, Cortical atrophy
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