Alam T A, Chaudhry V, Cornblath D R
Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA.
Muscle Nerve. 1998 Oct;21(10):1275-9. doi: 10.1002/(sici)1097-4598(199810)21:10<1275::aid-mus5>3.0.co;2-8.
Guillain-Barré syndrome (GBS) is recognized clinically by the presence of acute, rapidly progressive weakness, areflexia, and albuminocytological dissociation in cerebrospinal fluid. Although GBS was initially considered to be primarily an acute inflammatory demyelinating polyneuropathy (AIDP), several other subtypes have been recognized: acute motor axonal neuropathy (AMAN), acute motor-sensory axonal neuropathy (AMSAN), and Fisher syndrome (FS). Because each of these subtypes may have an independent immunopathogenesis and, therefore, may require selective treatments in the future, recognition of these subtypes is important. When using nerve conductions to classify the subtypes, the most easily and confidently identified subtype is AIDP. Therefore, most electrodiagnostic criteria have attempted to identify demyelination in this acute setting, in which physiology is constantly changing. In a single well-defined GBS population, we compared the various published criteria for demyelination in GBS. We reviewed charts of 43 patients with GBS between 1991 and 1996. Applying six available criteria sets, the number of patients categorized as having AIDP ranged from 21% to 72%. Until investigators can agree on a single set of criteria, considerable variability will continue to exist when identifying cases of AIDP.
吉兰-巴雷综合征(GBS)在临床上表现为急性、快速进展的肌无力、腱反射消失以及脑脊液中蛋白细胞分离。尽管GBS最初被认为主要是一种急性炎症性脱髓鞘性多发性神经病(AIDP),但现已认识到其他几种亚型:急性运动轴索性神经病(AMAN)、急性运动感觉轴索性神经病(AMSAN)和费舍尔综合征(FS)。由于这些亚型中的每一种可能都有独立的免疫发病机制,因此未来可能需要进行选择性治疗,识别这些亚型很重要。当使用神经传导来对亚型进行分类时,最容易且最有把握识别的亚型是AIDP。因此,大多数电诊断标准都试图在这种生理状态不断变化的急性情况下识别脱髓鞘。在一个明确界定的GBS群体中,我们比较了已发表的各种GBS脱髓鞘诊断标准。我们回顾了1991年至1996年间43例GBS患者的病历。应用六种可用的标准集,被归类为AIDP的患者数量从21%到72%不等。在研究者就一套单一标准达成共识之前,在识别AIDP病例时将继续存在相当大的变异性。