The treatable dementia of sjögren's syndrome

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Abstract
Progressive dementia developed during a 15-month period in a 56-year-old woman with serologically and clinically documented primary Sjogren's syndrome. Findings from magnetic resonance imaging and angiography were normal, but a brain biopsy disclosed perivascular lymphocytic inflammation in leptomeningeal and parenchymal vessels. Treatment with high-dose corticosteroids produced rapid and nearly complete resolution of the dementia.