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BMC Neurology

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Open Access 01-12-2022 | Magnetic Resonance Imaging | Case report

A rare case report of primary Sjögren’s syndrome with clinical characteristics similar to those of CLIPPERS

Authors: ChenLing Lv, FeiYan Zhu, Chao Chen, YunLing Wang, DengJun Guo, ZhenZhong Zhang

Published in: BMC Neurology | Issue 1/2022

Abstract

Background

Primary Sjögren’s syndrome (pSS) is an autoimmune inflammatory disease characterized by dryness of the eyes, mouth and other mucous membranes. Patients with pSS can also present with extraglandular manifestations, such as pulmonary, kidney and nervous system involvement. Central nervous system (CNS) manifestations have rarely been described in pSS.

Case presentation

A 33-year-old man was admitted with a one-month history of dizziness, speech disturbance, and walking instability. His brain enhanced magnetic resonance imaging (MRI) showed symmetrical, enhanced “salt-and-pepper-like” speckled lesions in the brainstem, basal ganglia, and subcortical regions, and his diagnosis was considered possible chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Further examination revealed that anti-SSA antibody was positive, and the Schirmer test and labial salivary gland histopathology were abnormal, which supported the diagnosis of pSS.

Conclusion

pSS is a chronic systemic autoimmune disease that involves neurological complications. This case suggests that CNS lesions of pSS can present with clinical and MRI findings similar to those of CLIPPERS.

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Title: A rare case report of primary Sjögren’s syndrome with clinical characteristics similar to those of CLIPPERS
Authors: ChenLing Lv
FeiYan Zhu
Chao Chen
YunLing Wang
DengJun Guo
ZhenZhong Zhang
Publication date: 01-12-2022
Publisher: BioMed Central
Keywords: Magnetic Resonance Imaging
Magnetic Resonance Imaging
Glucocorticoid
Published in: BMC Neurology / Issue 1/2022
Electronic ISSN: 1471-2377
DOI: https://doi.org/10.1186/s12883-022-02945-2

Keynote webinar | Spotlight on medication adherence

Springer Medicine

A rare case report of primary Sjögren’s syndrome with clinical characteristics similar to those of CLIPPERS

Abstract

Background

Case presentation

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Case presentation

Conclusion

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