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格林-巴利综合征亚型的电诊断陷阱。

Pitfalls in electrodiagnosis of Guillain-Barré syndrome subtypes.

机构信息

Department of Neuroscience and Imaging, University G. d'Annunzio Chieti-Pescara, Italy.

出版信息

J Neurol Neurosurg Psychiatry. 2010 Oct;81(10):1157-63. doi: 10.1136/jnnp.2010.208538.

Abstract

OBJECTIVE

To electrophysiologically classify an Italian Guillain-Barré syndrome (GBS) population into demyelinating and axonal subtypes, to investigate how serial recordings changed the classification and to underline the pitfalls in electrodiagnosis of GBS subtypes.

METHODS

The authors applied two current electrodiagnostic criteria sets for demyelinating and axonal GBS subtypes in 55 patients who had at least two serial recordings in three motor and sensory nerves.

RESULTS

At first test, the electrodiagnosis was almost identical with both criteria: 65-67% of patients were classifiable as acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 18% were classifiable as axonal GBS, and 14-16% were equivocal. At follow-up, 24% of patients changed classification: AIDP decreased to 58%, axonal GBS increased to 38%, and equivocal patients decreased to 4%. The majority of shifts were from AIDP and equivocal groups to axonal GBS, and the main reason was the recognition by serial recordings of the reversible conduction failure and of the length-dependent compound muscle action potential amplitude reduction patterns as expression of axonal pathology.

CONCLUSIONS

Axonal GBS is pathophysiologically characterised not only by axonal degeneration but also by reversible conduction failure at the axolemma of the Ranvier node. The lack of distinction among demyelinating conduction block, reversible conduction failure and length-dependent compound muscle action potential amplitude reduction may fallaciously classify patients with axonal GBS as having AIDP. Serial electrophysiological studies are mandatory for proper diagnosis of GBS subtypes and the identification of pathophysiological mechanisms of muscle weakness. More reliable electrodiagnostic criteria taking into consideration the reversible conduction failure pattern should be devised.

摘要

目的

用电生理学方法将意大利吉兰-巴雷综合征(GBS)人群分为脱髓鞘和轴索亚型,研究连续记录如何改变分类,并强调GBS 亚型电诊断的陷阱。

方法

作者应用两种当前的电诊断标准,用于诊断脱髓鞘和轴索 GBS 亚型,对 55 例至少有 2 次连续记录的 3 条运动和感觉神经的患者进行分类。

结果

初次检查时,两种标准的电诊断几乎相同:65-67%的患者可归类为急性炎症性脱髓鞘性多发性神经根神经病(AIDP),18%可归类为轴索 GBS,14-16%为不确定。随访时,24%的患者改变了分类:AIDP 减少到 58%,轴索 GBS 增加到 38%,不确定的患者减少到 4%。大多数转变是从 AIDP 和不确定的组到轴索 GBS,主要原因是连续记录识别出的可逆性传导失败和长度依赖性复合肌肉动作电位幅度降低模式,这是轴索病理学的表现。

结论

轴索 GBS 的病理生理学特征不仅是轴突变性,还有郎飞结轴突的可逆性传导失败。脱髓鞘性传导阻滞、可逆性传导失败和长度依赖性复合肌肉动作电位幅度降低之间的区别不明显,可能错误地将轴索 GBS 患者归类为 AIDP。连续的电生理研究对于正确诊断 GBS 亚型和识别肌肉无力的病理生理学机制是必需的。应该制定更可靠的电诊断标准,考虑到可逆性传导失败模式。

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