Sjögren’s syndrome with rapidly progressive motor neuron disease: a case report

Sjögren’s syndrome is an autoimmune disease that can affect multiple systems. Sjögren’s syndrome with motor neuron disease is rarely reported. Herein, we describe a patient with rapidly progressive motor neuron disease secondary to Sjögren’s syndrome. A 42-year-old woman was admitted to our hospital...

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Main Authors: Huijia Yang, Xiaozhong Jing, Jinhua Yan, Dihui Ma
Format: Article
Language:English
Published: SAGE Publishing 2020-11-01
Series:Journal of International Medical Research
Online Access:https://doi.org/10.1177/0300060520974465
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author Huijia Yang
Xiaozhong Jing
Jinhua Yan
Dihui Ma
author_facet Huijia Yang
Xiaozhong Jing
Jinhua Yan
Dihui Ma
author_sort Huijia Yang
collection DOAJ
description Sjögren’s syndrome is an autoimmune disease that can affect multiple systems. Sjögren’s syndrome with motor neuron disease is rarely reported. Herein, we describe a patient with rapidly progressive motor neuron disease secondary to Sjögren’s syndrome. A 42-year-old woman was admitted to our hospital with a 2-month history of progressive limb weakness. Neurological assessment revealed fasciculation in the lower limbs and amyotrophy in the bilateral supraspinatus, interosseous, and thenar muscles. Serological examination and labial gland biopsy revealed Sjögren’s syndrome. In addition, electromyography demonstrated neurogenic damage to the upper and lower limbs. The patient received a short course of high-dose corticosteroids, intravenous immunoglobulins, and immunosuppressant treatment, including a weekly dose of 0.4 g cyclophosphamide and a daily dose of 0.2 g hydroxychloroquine. However, the patient’s limb weakness was further aggravated and her respiratory function was compromised. Electromyography re-examination demonstrated extensive neurogenic damage, and she was diagnosed with Sjögren’s syndrome with motor neuron disease. The patient died of respiratory failure after 2 months. We suggest that more effective maintenance treatments should be sought. Further investigation is required to elucidate the association between autoimmune motor neuron disease and Sjögren’s syndrome.
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spelling doaj.art-5d3b8b6562da42828b11af0d1b541f1e2022-12-21T17:16:23ZengSAGE PublishingJournal of International Medical Research1473-23002020-11-014810.1177/0300060520974465Sjögren’s syndrome with rapidly progressive motor neuron disease: a case reportHuijia YangXiaozhong JingJinhua YanDihui MaSjögren’s syndrome is an autoimmune disease that can affect multiple systems. Sjögren’s syndrome with motor neuron disease is rarely reported. Herein, we describe a patient with rapidly progressive motor neuron disease secondary to Sjögren’s syndrome. A 42-year-old woman was admitted to our hospital with a 2-month history of progressive limb weakness. Neurological assessment revealed fasciculation in the lower limbs and amyotrophy in the bilateral supraspinatus, interosseous, and thenar muscles. Serological examination and labial gland biopsy revealed Sjögren’s syndrome. In addition, electromyography demonstrated neurogenic damage to the upper and lower limbs. The patient received a short course of high-dose corticosteroids, intravenous immunoglobulins, and immunosuppressant treatment, including a weekly dose of 0.4 g cyclophosphamide and a daily dose of 0.2 g hydroxychloroquine. However, the patient’s limb weakness was further aggravated and her respiratory function was compromised. Electromyography re-examination demonstrated extensive neurogenic damage, and she was diagnosed with Sjögren’s syndrome with motor neuron disease. The patient died of respiratory failure after 2 months. We suggest that more effective maintenance treatments should be sought. Further investigation is required to elucidate the association between autoimmune motor neuron disease and Sjögren’s syndrome.https://doi.org/10.1177/0300060520974465
spellingShingle Huijia Yang
Xiaozhong Jing
Jinhua Yan
Dihui Ma
Sjögren’s syndrome with rapidly progressive motor neuron disease: a case report
Journal of International Medical Research
title Sjögren’s syndrome with rapidly progressive motor neuron disease: a case report
title_full Sjögren’s syndrome with rapidly progressive motor neuron disease: a case report
title_fullStr Sjögren’s syndrome with rapidly progressive motor neuron disease: a case report
title_full_unstemmed Sjögren’s syndrome with rapidly progressive motor neuron disease: a case report
title_short Sjögren’s syndrome with rapidly progressive motor neuron disease: a case report
title_sort sjogren s syndrome with rapidly progressive motor neuron disease a case report
url https://doi.org/10.1177/0300060520974465
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AT jinhuayan sjogrenssyndromewithrapidlyprogressivemotorneurondiseaseacasereport
AT dihuima sjogrenssyndromewithrapidlyprogressivemotorneurondiseaseacasereport