Suppr超能文献

1例表现为双侧基底节病变的干燥综合征病例报告

A case report of Sjögren syndrome manifesting bilateral basal ganglia lesions.

作者信息

Niu Bing, Zou Zhenzhen, Shen Yuqin, Cao Bingzhen

机构信息

Department of Neurology, General Hospital of Jinan Military Region, Jinan, Shandong Province, China.

出版信息

Medicine (Baltimore). 2017 Apr;96(17):e6715. doi: 10.1097/MD.0000000000006715.

Abstract

RATIONALE

Peripheral neurological complications in primary Sjögren's syndrome (pSS) seem the most common, however the involvement of central nervous system (CNS) remains unclear. While abnormalities in pSS revealed by brain magnetic resonance imaging (MRI) are usually small discrete hyperintense areas in the white matter on T2-FLAIR weighted MRI, massive brain lesions have been rarely reported, particularly in bilateral basal ganglia.

PATIENT CONCERNS

A 51-year-old woman exhibited dizziness, slurred speech and hemiplegia as a manifestation of pSS. Brain MRI revealed bilateral and symmetrical lesions extending into the basal ganglia, corona radiata and corpus callosum.

DIAGNOSES

Primary Sjögren's syndrome was diagnosed on the basis of clinical features, abnormal Schirmer's test and tear break-up time (BUT) findings, high levels of anti-Sjögren's-syndrome-related antigen A (anti-SSA) (Ro) and anti-Sjögren's-syndrome-related antigen B (anti-SSB) (La) antibodies, and positive labial minor salivary gland biopsy results.

INTERVENTIONS

She was treated with intravenous methylprednisolone and discharged on oral steroid therapy of prednisolone acetate.

OUTCOMES

The patient had an excellent response to steroid therapy.

LESSONS

The present case suggests that symmetry bilateral lesions can occur as a symptom of pSS, which could be induced by an autoimmune mechanism.

摘要

理论依据

原发性干燥综合征(pSS)的周围神经并发症似乎最为常见,然而中枢神经系统(CNS)受累情况仍不明确。虽然脑磁共振成像(MRI)显示的pSS异常通常是T2-FLAIR加权MRI上白质中的小而离散的高信号区,但大量脑病变很少被报道,尤其是双侧基底节病变。

患者情况

一名51岁女性表现出头晕、言语不清和偏瘫,为pSS的表现。脑MRI显示双侧对称病变延伸至基底节、放射冠和胼胝体。

诊断

根据临床特征、Schirmer试验和泪膜破裂时间(BUT)检查结果异常、抗干燥综合征相关抗原A(抗SSA)(Ro)和抗干燥综合征相关抗原B(抗SSB)(La)抗体水平升高以及唇小唾液腺活检结果阳性,诊断为原发性干燥综合征。

干预措施

给予静脉注射甲泼尼龙治疗,出院时接受醋酸泼尼松口服类固醇治疗。

结果

患者对类固醇治疗反应良好。

经验教训

本病例表明,对称性双侧病变可作为pSS的症状出现,可能由自身免疫机制诱发。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fd5d/5413247/368d957cd0bc/medi-96-e6715-g001.jpg

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验