Department of Neurology, College of Medicine, Hallym University, Seoul, Korea Neuroscience Research Australia, Sydney, Australia.
Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore.
J Neurol Neurosurg Psychiatry. 2014 Aug;85(8):907-13. doi: 10.1136/jnnp-2013-306212. Epub 2013 Dec 19.
Over the past 20 years, the most notable advance in understanding Guillain-Barré syndrome (GBS) has been the identification of an axonal variant. This advance arose chiefly through studies undertaken in East Asian countries and comprised two major aspects: first, the immunopathogenesis of axonal GBS related to anti-ganglioside antibodies and molecular mimicry of Campylobacter jejuni; and second, the observation that distinct electrophysiological patterns of axonal GBS existed, reflecting reversible conduction failure (RCF). As a consequence, the pathophysiology of acute motor axonal neuropathy (AMAN) has perhaps become better understood than acute inflammatory demyelinating polyneuropathy. Despite these more recent advances, a critical issue remains largely unresolved: whether axonal GBS is more common in Asia than in Europe or North America. If it is more common in Asia, then causative factors must be more critically considered, including geographical differences, issues of genetic susceptibility, the role of antecedent infections and other potential triggering factors. It has become apparent that the optimal diagnosis of AMAN requires serial electrophysiological testing, to better delineate RCF, combined with assessment for the presence of anti-ganglioside antibodies. Recent collaborative approaches between Europe and Asia have suggested that both the electrophysiological pattern of AMAN and the seropositivity for anti-ganglioside antibodies develop similarly. Separately, however, current electrodiagnostic criteria for AMAN limited to a single assessment appear inadequate to identify the majority of cases. As such, diagnostic criteria will need to be revised to improve the diagnostic sensitivity for AMAN.
在过去的 20 年中,对吉兰-巴雷综合征(GBS)的理解最显著的进展是发现了轴索性变异型。这一进展主要源于东亚国家开展的研究,包括两个主要方面:首先,与抗神经节苷脂抗体和空肠弯曲菌分子模拟相关的轴索性 GBS 的免疫发病机制;其次,观察到存在明显不同的轴索性 GBS 电生理模式,反映出可逆性传导失败(RCF)。因此,急性运动轴索性神经病(AMAN)的病理生理学可能比急性炎性脱髓鞘性多发性神经病理解得更透彻。尽管有了这些最近的进展,但一个关键问题仍然在很大程度上没有得到解决:轴索性 GBS 在亚洲是否比在欧洲或北美更常见。如果在亚洲更常见,那么必须更仔细地考虑致病因素,包括地理差异、遗传易感性问题、前驱感染的作用以及其他潜在的触发因素。显然,对 AMAN 的最佳诊断需要进行连续的电生理测试,以更好地描绘 RCF,并结合对神经节苷脂抗体存在的评估。最近欧洲和亚洲之间的合作研究表明,AMAN 的电生理模式和抗神经节苷脂抗体的血清阳性率发展相似。然而,目前 AMAN 的电诊断标准仅限于单次评估,似乎不足以识别大多数病例。因此,有必要修订诊断标准以提高 AMAN 的诊断敏感性。