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纯感觉性吉兰-巴雷综合征:一例报告及文献复习

Pure sensory Guillain-Barré syndrome: A case report and review of the literature.

作者信息

Yang Jingjing, Huan Mingming, Jiang Huajun, Song Chunli, Zhong Lin, Liang Zhanhua

机构信息

Department of Neurology, First Affiliated Hospital of Dalian Medical University, Dalian, Liaoning 116011, P.R. China.

Department of Orthopedics, First Affiliated Hospital of Dalian Medical University, Dalian, Liaoning 116011, P.R. China.

出版信息

Exp Ther Med. 2014 Nov;8(5):1397-1401. doi: 10.3892/etm.2014.1955. Epub 2014 Sep 11.

Abstract

Sensory Guillain-Barré syndrome (GBS) is an acute demyelinating neuropathy that presents clinically with involvement of the sensory peripheral nerve only. To date, <10 cases of pure sensory GBS have been reported; thus, the clinical and pathological features of sensory variant GBS are yet to be well characterized. The current study reports the case of a 43-year-old female that presented with acute, symmetric and monophasic sensory neuropathy, without motor weakness. Patient history, clinical examination, routine nerve conduction studies and sural nerve biopsy were reviewed. All the observations were consistent with a diagnosis of pure sensory GBS. In particular, the pathological features of the sural nerve biopsy revealed that the form of regenerated nerve fibers have complete structure of myelinated nerve fascicles, and these myelinated nerve fibers are thicker than other parts of the biopsy. The patient received small-dose (20 mg/day) prednisone initially, but without any benefit. Satisfactory improvements were observed with one course of intravenous immunoglobulin.

摘要

感觉性吉兰-巴雷综合征(GBS)是一种急性脱髓鞘性神经病,临床上仅表现为感觉性周围神经受累。迄今为止,纯感觉性GBS的报道病例<10例;因此,感觉变异型GBS的临床和病理特征尚未得到充分描述。本研究报告了一例43岁女性患者,表现为急性、对称性和单相性感觉神经病,无肌无力。回顾了患者病史、临床检查、常规神经传导研究和腓肠神经活检。所有观察结果均符合纯感觉性GBS的诊断。特别是,腓肠神经活检的病理特征显示,再生神经纤维的形态具有完整的有髓神经束结构,且这些有髓神经纤维比活检的其他部位更粗。患者最初接受小剂量(20mg/天)泼尼松治疗,但无任何益处。静脉注射免疫球蛋白一个疗程后观察到满意的改善。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/136f/4186393/a8e5fc50f889/ETM-08-05-1397-g00.jpg

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