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Abstract
Primary Sjögren'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 re-currence during the followup 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.
References
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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.
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).
Figure 3.
Salivary gland scan reveals decreased uptake in the right submandibular gland and excretory dysfunction in both submandibular glands.
Figure 4.
Biopsy of a minor salivary gland showing focal lymphocytic infiltration with a focus score 1 (original magnification, ×400).
Table 1.
Cases of Sjögren's syndrome presenting cognitive dysfunction responsive to immunosuppresive therapy
| Case 1 (3) | Case 2 (4) | Case 3 (5) | Case 4 (6) | The present case | |
|---|---|---|---|---|---|
| Age (years old) | 56 | 48 | 29 | 57 | 52 |
| Gender | Female | Male | Female | Female | Female |
| Classification criteria of pSS described∗ | Dry mouth (+), Dry eye (+) Schirmer test (+), Anti-Ro/La (+/+) | Dry mouth (+), Dry eye (+) Schirmer test (+), Anti-Ro/La (+/-), MSG† biopsy (+) | Dry mouth (+), Dry eye (+) Schirmer test (+), Anti-Ro/La (+/+), MSG biopsy (+) | Dry mouth (+), Dry eye (+) MSG biopsy (+) | Schirmer test (+), Salivary scan (+), Anti-Ro/La (+/+), MSG biopsy (+) |
| Time from exocrinopathy to CSN symptoms | 15 years | ND‡ | 17 months | ND | - |
| Presenting neurological symptoms | Forgetfulness, Visual hallucination | Lassitude, Forgetfulness, Sterotypic speech | Dissociation, Memory disturbance | y Ataxia, Hypoesthesia, Memory difficulty, Depression | Depression, Dysphagia, Gait disturbance, Memory disturbance |
| MRI | Normal | T2 hyperintense small lesions in the periventricular white matter | Normal | T2 hyperintense lesions in the spinal cord and left frontal white matter | T2 hyperintense lesion, Both cerebellum, cerebrum, internal capsule |
| Treatment | High dose of PD§ (120 mg/day) | High dose of PD (40 mg/day) | High-dose of PD (40 mg/day) | Methylprednisolone pulse and then high dose of PD (1 mg/kg) | Methylprednisone 1 g/day IV and then high dose of PDL¶ (1 mg/kg), CTX∗∗ pulse |
| Follow up | Improvement after 1 month | An episode of relapse after 11 months during PD tapering | Two episodes of relapse during 6 months of Pd tapering | ND | Improvement after 6 weeks No relapse during 2 years |
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