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KMID : 0388220130200050336
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Journal of the Korean Rheumatism Association
2013 Volume.20 No. 5 p.336 ~ p.341
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Primary Sjogren’s Syndrome Presenting with Rapidly Progressive Cognitive Impairment
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Chung Hee-Won
Yoo Jong-Jin
Choi Byoong-Yong
Cho Hyun-Jung
Kang Eun-Ha
Song Yeong-Wook
Lee Yun-Jong
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Abstract
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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 mani-festation 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 gluco-corticoid and cyclophosphamide pulse therapy without re-currence 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 re-view of the literature.
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KEYWORD
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Sjogren’s syndrome , Cognitive impairment , Central nervous system
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