Primary Sjogren's Syndrome Presenting with Rapidly Progressive Cognitive Impairment

ABSTRACT

Primary Sjogren's syndrome (pSS) is characterized by chronic inflammation and dysfunction in exocrine organs; however, it also has protean clinical features, including neuropsychiatric symptoms. A major neurological manifestation is peripheral neuropathy and involvement of the central nervous system is uncommonly described in pSS. A 52-year-old female was admitted because of depression, dysarthria, gait abnormality, and memory disturbance, which had developed over two months, and was diagnosed as pSS. She was treated successfully with high-dose glucocorticoid and cyclophosphamide pulse therapy without recurrence during the follow-up period of two years. Herein, we describe the first Korean case of pSS presenting with rapidly progressive cognitive impairment along with a review of the literature.