以脊髓病变为突出表现的干燥综合征:1例报告及文献复习

白书维,蒋仙国,管阳太

神经病学与神经康复学杂志 ›› 2018, Vol. 14 ›› Issue (3) : 178-184.

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神经病学与神经康复学杂志 ›› 2018, Vol. 14 ›› Issue (3) : 178-184. DOI: 10.12022/jnnr.2018-0078
病例报告

以脊髓病变为突出表现的干燥综合征:1例报告及文献复习

  • 白书维,蒋仙国,管阳太
作者信息 +

Sjögren’s syndrome manifesting myelopathy: one case eport and literature review

  • BAI Shuwei, JIANG Xianguo, GUAN Yangtai
Author information +
文章历史 +

摘要


目的:提高对干燥综合征并脊髓病变(Sjögren's syndrome myelopathy,SSM)的临床表现及影像学特征的认识。

方法:报道1 例SSM 患者的临床诊治经过,结合文献复习,重点讨论SSM 的临床表现、病因、影像学特征和治疗原则。

结果:1 例老年男性患者以肢体无力、二便困难为主诉;磁共振成像检查提示脊髓炎表现,血清抗核抗体谱筛查、眼科干燥性角结膜炎检查以及唇腺活检病理证实为SSM;转入风湿科接受糖皮质激素及利妥昔单抗治疗后,症状好转。

结论:许多干燥综合征患者以神经系统症状为首发表现,可累及中枢及周围神经系统,临床表现多样,但不存在典型的口干、眼干症状。对于临床诊断为干燥综合征的患者,也应注意可能伴发的神经系统病变。SSM 发病率不高,易被误诊或漏诊,因此应对SSM与脊髓病变、视神经脊髓炎谱系疾病和多发性硬化等进行鉴别诊断,以便尽早作出正确的诊断,并给予相应的个体化治疗。

Abstract


Objective: To improve the understanding of clinical manifestations and imaging features of Sjögren’s syndrome myelopathy (SSM).

Methods: A case of SSM was reported. The clinical manifestations, etiology, imaging features and treatment principles of SSM were discussed with literature review.

Results: One elderly male patient complained mainly of limb weakness and diuresis difficulty; magnetic resonance imaging showed myelitis, and serum anti-nuclear antibody screening, ophthalmic dry keratoconjunctivitis and lip biopsy pathology confirmed SSM. The patient was transferred to department of rheumatology for treatment with glucocorticoid and rituximab, and the symptoms were relieved. 

Conclusion: Instead of the typical symptoms of dry mouth and dry eyes, the neuropathy included peripheral and central nervous system is often observed as the early manifestation in many Sjögren’s syndrome patients. Therefore, for the clinical diagnosis of Sjögren’s syndrome, attention should also be paid to the possible concomitant neuropathy. The incidence of SSM is not high, and it is easy to be misdiagnosed or missed. Therefore, differential diagnosis should be made between SSM and myelopathy, optic neuromyelitis spectrum disease and multiple sclerosis, so as to make a correct diagnosis as soon as possible, and give appropriate individual treatment.

关键词

干燥综合征 / 脊髓病变 / 神经影像

Key words

Sjögren’s syndrome /  Myelopathy /  Neuroimage

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白书维,蒋仙国,管阳太. 以脊髓病变为突出表现的干燥综合征:1例报告及文献复习[J]. 神经病学与神经康复学杂志. 2018, 14(3): 178-184 https://doi.org/10.12022/jnnr.2018-0078
BAI Shuwei, JIANG Xianguo, GUAN Yangtai. Sjögren’s syndrome manifesting myelopathy: one case eport and literature review[J]. Journal of Neurology and Neurorehabilitation. 2018, 14(3): 178-184 https://doi.org/10.12022/jnnr.2018-0078

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