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一名表现为吉兰-巴雷综合征的变应性肉芽肿性血管炎患者

[A patient with Churg-Strauss syndrome presenting as Guillain-Barré syndrome].

作者信息

Djukic M, Schmidt H, Mazurek C, König F, Schweyer S, Nau R

机构信息

Klinik für Neurologie, Georg-August-Universität, Robert-Koch-Strasse 40, 37075, Göttingen, Deutschland.

出版信息

Nervenarzt. 2008 Apr;79(4):457-61. doi: 10.1007/s00115-007-2377-y.

Abstract

A 74-year-old man presented in a pulmonary clinic with symmetrically ascending tetraparesis. Physical and neurophysiological examinations suggested Guillain-Barré syndrome. The patient was treated with an initial course of 7s immunoglobulins without success. His state worsened until he was unable to walk. Severe eosinophilia (41%) was later noted in the differential white blood cell count. Combined with the onset of asthma-like symptoms, this prompted us to suspect Churg-Strauss syndrome. Despite treatment with high-dose corticoids, the palsy did not improve. It was only after immunosuppression with cyclophosphamide that the patient began to recover. The subgroup of necrotising vasculitides must be considered as differential diagnosis of rapidly progressive, symmetrical neuropathy with ascending course. Early identification and treatment are essential, since early immunosuppressive therapy is often successful, whereas delayed initiation of treatment may lead to a fatal outcome.

摘要

一名74岁男性因对称性上行性四肢轻瘫就诊于肺部诊所。体格检查和神经生理学检查提示吉兰 - 巴雷综合征。患者接受了首个疗程的静脉注射免疫球蛋白治疗,但未成功。他的病情恶化,直至无法行走。后来在白细胞分类计数中发现严重嗜酸性粒细胞增多(41%)。结合哮喘样症状的出现,这促使我们怀疑为变应性肉芽肿性血管炎。尽管使用了大剂量皮质类固醇治疗,麻痹仍未改善。直到使用环磷酰胺进行免疫抑制治疗后,患者才开始康复。坏死性血管炎亚组必须被视为快速进展性、对称性、上行性病程的神经病的鉴别诊断。早期识别和治疗至关重要,因为早期免疫抑制治疗通常会成功,而延迟开始治疗可能导致致命后果。

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