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脱髓鞘型和轴索性吉兰-巴雷综合征的病情进展模式差异。

Differences in patterns of progression in demyelinating and axonal Guillain-Barré syndromes.

作者信息

Hiraga A, Mori M, Ogawara K, Hattori T, Kuwabara S

机构信息

Department of Neurology, Chiba University School of Medicine, Japan.

出版信息

Neurology. 2003 Aug 26;61(4):471-4. doi: 10.1212/01.wnl.0000081231.08914.a1.

Abstract

BACKGROUND

Immune treatments are recommended for patients with Guillain-Barré syndrome (GBS) who cannot walk independently, but a considerable number of GBS patients are in the progressive phase at the first examination.

OBJECTIVE

To investigate whether progression patterns differ in demyelinating and axonal subtypes of GBS.

METHODS

Clinical, laboratory, and electrophysiologic data on 131 consecutive patients with GBS were reviewed. Patients were classified as having acute inflammatory demyelinating polyneuropathy (AIDP) or acute motor axonal neuropathy (AMAN) based on electrodiagnostic criteria.

RESULTS

Forty-one patients had AIDP, 62 AMAN, and 28 were unclassified. Age, sex, and Hughes Functional Grading Scale score at the first medical examination did not differ for the AIDP and AMAN patients. Mean periods between neurologic onset and first examination (5.3 vs 4.2 days; p = 0.01) and neurologic onset and nadir (18.0 vs 11.5 days; p = 0.001) were longer for the AIDP group. In the subgroup of those with mild disability (able to walk independently at the first neurologic examination), 88% of the AMAN patients had reached the nadir, whereas 65% of the AIDP patients had reached it. The remaining 35% progressed to it over the next 1 to 2 weeks and were unable to walk at nadir.

CONCLUSIONS

The patterns and speeds of progression differ in AMAN and AIDP, AMAN having a rapid progression and an early nadir. AIDP patients frequently have a significantly long progression after the first examination; therefore, they need to be carefully monitored.

摘要

背景

对于无法独立行走的吉兰-巴雷综合征(GBS)患者,推荐进行免疫治疗,但相当数量的GBS患者在首次检查时处于疾病进展期。

目的

研究GBS脱髓鞘型和轴索性亚型的病情进展模式是否存在差异。

方法

回顾了131例连续性GBS患者的临床、实验室及电生理数据。根据电诊断标准,将患者分为急性炎症性脱髓鞘性多发性神经病(AIDP)或急性运动轴索性神经病(AMAN)。

结果

41例患者为AIDP,62例为AMAN,28例未分类。AIDP组和AMAN组患者在首次医学检查时的年龄、性别及休斯功能分级量表评分无差异。AIDP组从神经症状发作到首次检查的平均时间(5.3天对4.2天;p = 0.01)以及从神经症状发作到最低点的平均时间(18.0天对11.5天;p = 0.001)更长。在轻度残疾亚组(首次神经科检查时能够独立行走)中,88%的AMAN患者已达到最低点,而65%的AIDP患者已达到最低点。其余35%在接下来1至2周内进展至此,且在最低点时无法行走。

结论

AMAN和AIDP的病情进展模式及速度不同,AMAN进展迅速且最低点出现较早。AIDP患者在首次检查后病情进展通常明显较长;因此,需要对他们进行仔细监测。

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