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皮质类固醇有助于鉴别吉兰-巴雷综合征和慢性炎症性脱髓鞘性多发性神经病首次发作:一例说明性病例报告。

Corticosteroids can help distinguish between Guillain-Barré syndrome and first attack of chronic inflammatory demyelinating neuropathy: an illustrative case report.

作者信息

Alexander K John, Alshubaili Asmahan F, Santhamoorthy P, Sharafuddin K M

机构信息

Department of Neurology, Ibn Sina Hospital, Kuwait.

出版信息

Med Princ Pract. 2008;17(5):422-4. doi: 10.1159/000141510. Epub 2008 Aug 6.

Abstract

OBJECTIVE

To report a case of acquired demyelinating neuropathy that failed to improve upon treatment with intravenous immunoglobulins and plasmapheresis but responded dramatically to corticosteroids, illustrating the occasional difficulty in distinguishing Guillain-Barré syndrome (GBS) from a first attack of chronic inflammatory demyelinating polyneuropathy (CIDP).

CLINICAL PRESENTATION AND INTERVENTION

A 25-year-old previously healthy man was admitted with a 5-day history of ascending areflexic paralysis of all 4 limbs and diagnosed with GBS. On admission, he was administered intravenous immunoglobulins at a dosage of 400 mg/kg/day for 5 days yet continued to worsen. He became quadriparetic by the second week. As there had been no improvement, he was plasmapheresed with 7 sessions of plasma exchange, 50 ml/kg of plasma at each session, including appropriate replacement fluid. After failing to improve within 8 weeks, he was started on intravenous methylprednisolone and a dramatic improvement was observed by the 5th day. He continued to get better on oral prednisolone, was ambulatory with support 4 weeks later and could walk without support on follow-up.

CONCLUSION

This case illustrates that there is a subset of patients initially diagnosed with GBS who do not respond to immunoglobulins or plasmapheresis but do specifically well on steroids. Hence treatment with prednisolone should not be delayed in selective cases of GBS as it may actually be a first episode of CIDP.

摘要

目的

报告一例获得性脱髓鞘性神经病病例,该病例经静脉注射免疫球蛋白和血浆置换治疗后未见改善,但对皮质类固醇有显著反应,说明在将吉兰 - 巴雷综合征(GBS)与慢性炎症性脱髓鞘性多发性神经病(CIDP)的首次发作相鉴别时偶尔会遇到困难。

临床表现及干预措施

一名25岁既往健康的男性因四肢进行性无反射性瘫痪5天入院,被诊断为GBS。入院时,他接受了静脉注射免疫球蛋白治疗,剂量为400mg/kg/天,持续5天,但病情仍继续恶化。到第二周时他出现四肢瘫。由于病情没有改善,他接受了7次血浆置换,每次置换50ml/kg血浆,并给予适当的置换液。在8周内未见改善后,他开始接受静脉注射甲泼尼龙治疗,第5天观察到病情显著改善。他继续口服泼尼松龙后病情持续好转,4周后在辅助下可行走,随访时可独立行走。

结论

该病例表明,有一部分最初被诊断为GBS的患者对免疫球蛋白或血浆置换无反应,但对类固醇治疗反应良好。因此,在GBS的某些选择性病例中,不应延迟使用泼尼松龙治疗,因为其可能实际上是CIDP的首发发作。

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