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[一例伴有脑动脉多发狭窄及短暂神经症状和体征的干燥综合征]

[A Case of Sjögren's Syndrome with Multiple Stenoses of the Cerebral Arteries and Transient Neurological Symptoms and Signs].

作者信息

Nagata Tomoyuki, Hosoyama Sachiko, Shida Norihiko, Ohori Nobuhira

机构信息

Department of Neurology, Japanese Red Cross Yamaguchi Hospital.

出版信息

Brain Nerve. 2018 Dec;70(12):1389-1396. doi: 10.11477/mf.1416201198.

DOI:10.11477/mf.1416201198
PMID:30523219
Abstract

A 31-year-old woman was admitted to the hospital after several episodes of transient numbness in her fingers and dysarthria. No neurological symptoms and signs were observed in this patient on admission; however, blood tests revealed elevated amylase and immunoglobulin G levels, an increased erythrocyte sedimentation rate, and positive anti-Sjögren's-syndrome (SjS)-related antigen A antibodies. The cerebrospinal fluid examination revealed a slight increase in the cell count and protein content. Diffusion-weighted and fluid-attenuated inversion recovery images on magnetic resonance imaging showed high-signal-intensity lesions in the cerebral white matter and basal ganglia supplied by the left middle cerebral artery. A part of the lesions was enhanced by the administration of gadolinium contrast medium. Magnetic resonance angiography suggested stenosis in the left anterior, middle, and posterior cerebral arteries. The patient was thus diagnosed with acute cerebral infarction and was then treated with argatroban, edaravone, and clopidogrel. Furthermore, Schirmer's test, sialography, and salivary gland biopsy results led to the diagnosis of SjS. A nerve conduction study suggested concomitant mild polyneuritis or mononeuritis multiplex. Collectively, we speculate that cerebral arterial stenosis associated with SjS leads to transient ischemic attack-like episodes, transient neurological symptoms and signs, and cerebral infarction. (Received June 13, 2018; Accepted September 11, 2018; Published December 1, 2018).

摘要

一名31岁女性在经历数次手指短暂麻木和构音障碍后入院。入院时该患者未观察到神经症状和体征;然而,血液检查显示淀粉酶和免疫球蛋白G水平升高、红细胞沉降率增加以及抗干燥综合征(SjS)相关抗原A抗体呈阳性。脑脊液检查显示细胞计数和蛋白质含量略有增加。磁共振成像的扩散加权成像和液体衰减反转恢复图像显示,由左大脑中动脉供血的脑白质和基底节区有高信号强度病变。部分病变在给予钆对比剂后有强化。磁共振血管造影提示左大脑前、中、后动脉狭窄。该患者因此被诊断为急性脑梗死,随后接受了阿加曲班、依达拉奉和氯吡格雷治疗。此外,施密特试验、涎腺造影和唾液腺活检结果确诊为干燥综合征。神经传导研究提示合并轻度多发性神经炎或多灶性单神经炎。总体而言,我们推测与干燥综合征相关的脑动脉狭窄会导致短暂性脑缺血发作样发作、短暂性神经症状和体征以及脑梗死。(2018年6月13日收稿;2018年9月11日接受;2018年12月1日发表)

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