van der Meché F G, van Doorn P A
Department of Neurology, University Hospital Dijkzigt/Sophia, Rotterdam, Netherlands.
Ann Neurol. 1995 May;37 Suppl 1:S14-31. doi: 10.1002/ana.410370704.
The relation between Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy is discussed. Most likely they represent parts of a continuum, arbitrarily separated by their time course. Within the concept of chronic inflammatory demyelinating polyneuropathy the presence of a monoclonal gammopathy of undetermined significance is discussed. The pathogenesis of inflammatory demyelinating polyneuropathies has not been elucidated yet, but involvement of the immune system has been firmly established. Preceding infections, especially with Campylobacter jejuni, and the analysis of antiganglioside antibodies lend new support to the hypothesis of molecular mimicry between epitopes on infectious agents and peripheral nerve constituents as one of the mechanisms in Guillain-Barré syndrome. In the future, a further classification of individual patients based on clinical, epidemiological, electrophysiological, pathological, microbiological, and immunological criteria may give a basis for more individualized treatment strategies. In Guillain-Barré syndrome the efficacy of high-dose intravenous immune globulin treatment was established after earlier positive findings with plasma exchange; immune globulins are easier to administer and may be superior. Even with these treatments it should be anticipated that one fourth of patients after immune globulin treatment and one third of patients after plasma exchange will show further deterioration in the first 2 weeks after onset of treatment. Despite this, just one treatment course usually is indicated in the individual patient, and no valid arguments were found to switch to the other treatment modality. In chronic inflammatory demyelinating polyneuropathy, prednisone, plasma exchange, and immune globulins are effective in a proportion of patients. The last two are equally effective. Patients may respond to one of these if a previous treatment failed, and here switching therapy may be effective due to the chronic course of the disease. Complexity and costs make plasma exchange the last choice. Whether prednisone or immune globulin is the first choice depends on the speed of recovery and the estimation of long-term loss of quality of life due to side effects of prednisone versus the costs of immune globulins. The mechanism of immune globulins in inflammatory polyneuropathies is discussed. There is evidence that idiotypic-antiidiotypic interaction may play a role, but several other mechanisms also may be involved.
本文讨论了吉兰 - 巴雷综合征与慢性炎症性脱髓鞘性多发性神经病之间的关系。它们很可能代表了一个连续统一体的不同部分，只是根据病程被人为地划分开来。在慢性炎症性脱髓鞘性多发性神经病的概念范畴内，还讨论了意义未明的单克隆丙种球蛋白病的存在情况。炎症性脱髓鞘性多发性神经病的发病机制尚未阐明，但免疫系统的参与已得到确凿证实。先前的感染，尤其是空肠弯曲菌感染，以及抗神经节苷脂抗体的分析，为感染因子表位与周围神经成分之间存在分子模拟这一假说提供了新的支持，而这一假说被认为是吉兰 - 巴雷综合征的发病机制之一。未来，基于临床、流行病学、电生理、病理、微生物学和免疫学标准对个体患者进行进一步分类，可能为更具个体化的治疗策略提供依据。在吉兰 - 巴雷综合征中，高剂量静脉注射免疫球蛋白治疗的疗效在血浆置换取得早期阳性结果后得到确立；免疫球蛋白更易于给药，且可能更具优势。即便采用这些治疗方法，仍可预期免疫球蛋白治疗后四分之一的患者以及血浆置换治疗后三分之一的患者在治疗开始后的头两周内病情会进一步恶化。尽管如此，通常每个患者只需一个疗程的治疗，且未发现有改用另一种治疗方式的合理依据。在慢性炎症性脱髓鞘性多发性神经病中，泼尼松、血浆置换和免疫球蛋白对部分患者有效。后两者效果相当。如果先前的治疗失败，患者可能对其中一种治疗有反应，鉴于该病病程较长，在此情况下更换治疗方法可能有效。血浆置换因操作复杂和费用问题成为最后的选择。泼尼松和免疫球蛋白何者作为首选取决于恢复速度以及对因泼尼松副作用导致的长期生活质量下降与免疫球蛋白费用的权衡。文中还讨论了免疫球蛋白在炎症性多发性神经病中的作用机制。有证据表明独特型 - 抗独特型相互作用可能起作用，但也可能涉及其他几种机制。
