Acute motor axonal neuropathy: An antibody-mediated attack on axolemma
Acute motor axonal neuropathy: An antibody-mediated attack on axolemma
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DOI：
10.1002/ana.410400414
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发表时间：
1996-10-01
影响因子：
11.2
通讯作者：
Griffin, JW
中科院分区：
文献类型：
--
作者：
HaferMacko, C;Hsieh, ST;Griffin, JW
格林-巴利综合征的急性运动性轴索神经病(AMAN)是一种突然发病的麻痹性疾病，病理特征是运动神经纤维变性，严重程度不等，感觉纤维稀少。几乎没有脱髓鞘或淋巴细胞性炎症。大多数病例有空肠弯曲菌的先天感染，许多病例有针对GM1神经节苷脂表位的抗体，但神经纤维损伤的机制尚未确定。在7例AMAN死亡病例中，免疫细胞化学显示免疫球蛋白G和补体激活产物C3d结合在运动纤维的轴膜上。最常见的受累部位是结节轴膜，但在更严重的病例中，IgG和C3d出现在有髓节间的轴突周围间隙内，与运动轴突外表面结合。这些结果表明，AMAN是一种新的疾病，由抗体和补体介导的对运动纤维轴膜的攻击引起。
The acute motor axonal neuropathy (AMAN) form of the Guillain-Barre syndrome is a paralytic disorder of abrupt onset characterized pathologically by motor nerve fiber degeneration of variable severity and by sparing of sensory fibers. There is little demyelination or lymphocytic inflammation. Most cases have antecedent infection with Campylobacter jejuni and many have antibodies directed toward GM1 ganglioside-like epitopes, but the mechanism of nerve-fiber injury has not been defined. In 7 fatal cases of AMAN, immunocytochemistry demonstrated the presence of IgG and the complement activation product C3d bound to the axolemma of motor fibers. The most frequently involved site was the nodal axolemma, but in more severe cases IgG and C3d were found within the periaxonal space of the myelinated internodes, bound to the outer surface of the motor axon. These results suggest that AMAN is a novel disorder caused by an antibody- and complement-mediated attack on the axolemma of motor fibers.