J Rheum Dis.  2013 Oct;20(5):336-341. 10.4078/jrd.2013.20.5.336.

Primary Sjogren's Syndrome Presenting with Rapidly Progressive Cognitive Impairment

• Chung HW
• Yoo JJ
• Choi BY
• Cho HJ
• Kang EH
• Song YW
• Lee YJ

• KMID: 2223013
• DOI: http://doi.org/10.4078/jrd.2013.20.5.336

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.

Keyword

Sjogren's syndrome; Cognitive impairment; Central nervous system

MeSH Terms

Central Nervous System
Cyclophosphamide
Depression
Dysarthria
Female
Follow-Up Studies
Gait
Humans
Inflammation
Memory
Middle Aged
Neurologic Manifestations
Peripheral Nervous System Diseases
Recurrence
Sjogren's Syndrome*
Cyclophosphamide

Figure

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  Figure 1. Brain MRI demonstrated increased signal intensity on axial T2-weighted images in both cerebral white matter, internal capsules, and cerebellar white matter. These lesions have low signal intensity on axial T1-weighted images and ADC (apparent diffusion coefficient) maps.

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  Figure 2. Brain SPECT (single positron emission computed tomography), taken 2 weeks before her admission, reveals resting hypoperfusion of the left frontal, temporal, and parietal cortex and left basal ganglia (arrows).

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  Figure 3. Salivary gland scan reveals decreased uptake in the right submandibular gland and excretory dysfunction in both submandibular glands.

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  Figure 4. Biopsy of a minor salivary gland showing focal lymphocytic infiltration with a focus score 1 (original magnification, ×400).

Reference

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