Differences in patterns of progression in demyelinating and axonal Guillain-Barre syndromes
Differences in patterns of progression in demyelinating and axonal Guillain-Barre syndromes
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DOI：
10.1212/01.wnl.0000081231.08914.a1
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发表时间：
2003-08-26
期刊：
影响因子：
9.9
通讯作者：
Kuwabara, S
中科院分区：
文献类型：
--
作者：
Hiraga, A;Mori, M;Kuwabara, S
背景资料：免疫治疗被推荐用于不能独立行走的格林-巴利综合征（GBS）患者，但相当数量的GBS患者在首次检查时处于进展期。目的：探讨脱髓鞘型和轴突型GBS的进展模式是否不同。方法：回顾了131例GBS患者的临床、实验室和电生理数据。根据电诊断标准将患者分为急性炎性脱髓鞘性多发性神经病（AIDP）或急性运动轴索神经病（AMAN）。结果：AIDP 41例，AMAN 62例，未分型28例。AIDP和AMAN患者在首次体检时的年龄、性别和休斯功能分级量表评分没有差异。AIDP组的神经系统发作与首次检查之间的平均时间（5.3 vs 4.2天; p = 0.01）和神经系统发作与最低点之间的平均时间（18.0 vs 11.5天; p = 0.001）更长。在轻度残疾的亚组中（在第一次神经系统检查时能够独立行走），88%的AMAN患者达到了最低点，而65%的AIDP患者达到了最低点，剩下的35%在接下来的1 - 2周内进展到最低点，并且不能行走。结论：AMAN和AIDP的进展模式和速度不同，AMAN进展迅速，并在早期达到最低点。AIDP患者在第一次检查后通常会有很长的进展;因此，需要仔细监测。
Background: Immune treatments are recommended for patients with Guillain-Barre 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.