Department of Neuroscience and Imaging, University G. d'Annunzio, Chieti-Pescara, Italy.
Clin Neurophysiol. 2012 Aug;123(8):1487-95. doi: 10.1016/j.clinph.2012.01.025. Epub 2012 Apr 4.
Electrophysiology plays a determinant role in Guillain-Barré syndrome (GBS) diagnosis, classification of the subtypes and in establishing prognosis. In the last three decades, different electrodiagnostic criteria sets have been proposed for acute inflammatory demyelinating neuropathy (AIDP), acute motor axonal neuropathy (AMAN) and acute motor and sensory axonal neuropathy (AMSAN). Criteria sets for AIDP varied for the parameters indicative of demyelination considered, for the cut-off limits and the number of required abnormalities (all a priori established) showing different sensitivities. Criteria sets for AMAN and AMSAN were proposed on the initial assumption that these subtypes were pathologically characterised by simple axonal degeneration. However, some AMAN patients show transient conduction block/slowing in intermediate and distal nerve segments, mimicking demyelination but without the development of abnormal temporal dispersion, named reversible conduction failure (RCF). The lack of distinction between RCF and demyelinating conduction block leads to fallaciously classify AMAN patients with RCF as AIDP or AMAN with axonal degeneration. Serial electrophysiological studies are mandatory for proper diagnosis of GBS subtypes, identification of pathophysiological mechanisms and prognosis. More reliable electrodiagnostic criteria should be devised to distinguish axonal and demyelinating subtypes of GBS, taking into consideration the RCF pattern and focussing on temporal dispersion.
电生理学在吉兰-巴雷综合征(GBS)的诊断、亚型分类和预后评估中起着决定性作用。在过去的三十年中,已经提出了不同的电诊断标准集,用于急性炎症性脱髓鞘性神经病(AIDP)、急性运动轴索性神经病(AMAN)和急性运动感觉轴索性神经病(AMSAN)。AIDP 的标准集因所考虑的脱髓鞘指示参数、截止值和所需异常的数量(均为事先确定)而有所不同,表现出不同的敏感性。AMAN 和 AMSAN 的标准集最初假设这些亚型在病理学上表现为单纯的轴突变性。然而,一些 AMAN 患者在中间和远端神经节段出现短暂的传导阻滞/减慢,模拟脱髓鞘,但没有异常的时间离散发展,称为可逆性传导失败(RCF)。RCF 和脱髓鞘性传导阻滞之间没有区别,导致将具有 RCF 的 AMAN 患者错误地分类为 AIDP 或具有轴突变性的 AMAN。为了正确诊断 GBS 亚型、确定病理生理机制和预后,必须进行连续的电生理研究。应该制定更可靠的电诊断标准来区分 GBS 的轴突和脱髓鞘亚型,考虑到 RCF 模式,并侧重于时间离散。