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Published in: BMC Neurology 1/2021

Open Access 01-12-2021 | Guillain-Barré Syndrome | Case report

Acute motor and sensory axonal neuropathy in association with primary Sjögren’s syndrome: a case report

Authors: Yu-Ming Chen, Kuei-Ying Su

Published in: BMC Neurology | Issue 1/2021

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Abstract

Background

Primary Sjögren’s syndrome is a chronic, autoimmune, connective tissue disorder that results from the infiltration of exocrine glands, especially the lacrimal and salivary glands, by autoantibodies. Patients with Sjögren’s syndrome commonly present with dry eyes (xerophthalmia) and dry mouth (xerostomia). However, the clinical manifestations of Sjögren’s syndrome can be complicated and variable due to involvement of extraglandular organ systems, such as the nervous system. The neurological manifestations of this disorder often precede those of other exocrine gland symptoms. Hence, early diagnosis of Sjögren’s syndrome remains a challenge.

Case presentation

We report the case of a 63-year-old woman with primary Sjögren’s syndrome who presented with acute motor and sensory axonal neuropathy (AMSAN). Treatment with glucocorticoids and immunosuppressants partially improved her muscle weakness and paresthesia.

Conclusions

This case demonstrates the importance of early recognition and diagnosis of AMSAN in association with primary Sjögren’s syndrome to achieve a favorable clinical outcome. Primary Sjögren’s syndrome may be underdiagnosed because of vague symptoms of the sicca complex. Comprehensive immunological testing to evaluate this condition may be performed in patients presenting with variants of Guillain-Barré syndrome.
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Metadata
Title
Acute motor and sensory axonal neuropathy in association with primary Sjögren’s syndrome: a case report
Authors
Yu-Ming Chen
Kuei-Ying Su
Publication date
01-12-2021
Publisher
BioMed Central
Published in
BMC Neurology / Issue 1/2021
Electronic ISSN: 1471-2377
DOI
https://doi.org/10.1186/s12883-021-02190-z

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