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儿童吉兰-巴雷综合征的临床表现及预后

Clinical presentation and prognosis of childhood Guillain-Barré syndrome.

作者信息

Lee Jung Hwan, Sung In Young, Rew Il Sun

机构信息

Department of Physical Medicine and Rehabilitation, Wooridul Spine Hospital, Seoul, Korea.

出版信息

J Paediatr Child Health. 2008 Jul-Aug;44(7-8):449-54. doi: 10.1111/j.1440-1754.2008.01325.x.

DOI:10.1111/j.1440-1754.2008.01325.x
PMID:18557809
Abstract

AIM

Guillain-Barré syndrome (GBS) is an acute inflammatory polyneuropathy commonly characterised by rapidly progressive, symmetric weakness and areflexia. This study is to assess the clinical characteristics of paediatric GBS, as well as its long-term functional prognosis.

METHODS

We retrospectively assessed the clinical manifestations, results of electrodiagnostic tests, functional status and prognosis of 56 children diagnosed with GBS. Based on clinical and electrophysiological findings, the patients were classified as having acute inflammatory demyelinating polyradiculoneuropathy ([AIDP]n = 34), acute motor axonal neuropathy ([AMAN]n = 14), acute motor and sensory axonal neuropathy (n = 1) and Miller Fisher syndrome ([MFS]n = 7).

RESULTS

Upper respiratory infection was the most frequent preceding event, and limb weakness was the most frequent symptom at GBS onset. There was no significant difference in the mean time from the onset of illness to nadir between any of these groups. Both the AIDP and AMAN groups showed significantly poorer functional status, measured by the Hughes scale, than the MFS group. Two years after nadir, however, the three groups did not differ significantly. Functional status at nadir, as estimated by the Hughes scale, is a more important factor than electrophysiological types in predicting long-term outcome.

CONCLUSION

The most common symptom at onset in paediatric GBS was limb weakness. Functional status at nadir in AMAN was not significantly different from that of AIDP, and both types achieved good functional outcome for ambulation after 2 years. Functional status at nadir was more important than the electrophysiological type in predicting long-term outcomes.

摘要

目的

吉兰-巴雷综合征(GBS)是一种急性炎性多发性神经病,通常表现为快速进展的对称性肌无力和腱反射消失。本研究旨在评估儿童GBS的临床特征及其长期功能预后。

方法

我们回顾性评估了56例诊断为GBS的儿童的临床表现、电诊断检查结果、功能状态和预后。根据临床和电生理检查结果,将患者分为急性炎性脱髓鞘性多发性神经根神经病([AIDP],n = 34)、急性运动轴索性神经病([AMAN],n = 14)、急性运动和感觉轴索性神经病(n = 1)和米勒-费雪综合征([MFS],n = 7)。

结果

上呼吸道感染是最常见的前驱事件,肢体无力是GBS发病时最常见的症状。这些组中任何一组从发病到病情最低点的平均时间均无显著差异。用休斯量表测量,AIDP组和AMAN组的功能状态均显著差于MFS组。然而,在病情最低点出现两年后,三组之间没有显著差异。在预测长期预后方面,用休斯量表评估的病情最低点时的功能状态比电生理类型更重要。

结论

儿童GBS发病时最常见的症状是肢体无力。AMAN病情最低点时的功能状态与AIDP无显著差异,且两种类型在两年后行走功能均取得良好预后。在预测长期预后方面,病情最低点时的功能状态比电生理类型更重要。

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