Axonal Guillain-Barre syndrome: carbohydrate mimicry and pathophysiology
Axonal Guillain-Barre syndrome: carbohydrate mimicry and pathophysiology
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DOI：
10.1111/j.1529-8027.2007.00153.x
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发表时间：
2007-12-01
影响因子：
3.8
通讯作者：
Kuwabara, Satoshi
中科院分区：
文献类型：
--
作者：
Yuki, Nobuhiro;Kuwabara, Satoshi
急性运动性轴索神经病（Acute motor axonal neuropathy，AMAN）是格林-巴利综合征（Guillain-Barre syndrome，GBS）的一种轴索亚型，其特征为单纯运动受累、频繁的空肠弯曲菌前期感染、与抗GM 1或抗GD 1a免疫球蛋白G（IgG）抗体相关以及轴索变性和可逆性传导阻滞的电生理特征。GM 1和GD 1a神经节苷脂与C.空肠分离物。用GM 1或C.空肠LOS诱导抗GM 1 IgG抗体和随后的弛缓性麻痹。在兔模型周围神经中观察到的病理变化与人AMAN中的那些相同。AMAN兔的免疫组织化学显示结钠通道簇的破坏和结旁髓鞘末端环的脱离，类似于结旁脱髓鞘，这将显著降低脉冲传输的安全系数，并且可能是导致人类AMAN中经常存在的快速可逆传导阻滞的原因。C.空肠唾液酸转移酶（Cst-II）在神经节苷脂样LOS的生物合成中起作用，其决定转移酶活性。具有cst-II（Thr 51）的菌株表达GM 1和GD 1a表位，而感染cst-II（Thr 51）菌株的GBS患者具有抗GM 1或抗GD 1a IgG抗体。cst-II基因负责GBS的发展。免疫学、病理学、电生理学和细菌学研究提供了有力的证据，证明碳水化合物拟态是AMAN的一个原因，并阐明了AMAN中神经传导失败的机制。
Acute motor axonal neuropathy (AMAN), an axonal subtype of Guillain-Barre syndrome (GBS), is characterized by pure motor involvement, frequent antecedent infection by Campylobacter jejuni, association with anti-GM1 or anti-GD1a immunoglobulin G (IgG) antibodies, and the electrophysiological features of axonal degeneration and reversible conduction block. Molecular mimicry exists between GM1 and GD1a gangliosides and lipooligosaccharides (LOSs) of C. jejuni isolates from AMAN. Sensitization of rabbits with GM1 or C. jejuni LOS induces anti-GM1 IgG antibodies and subsequent flaccid paralysis. Pathological changes seen in rabbit model peripheral nerves are identical to those in human AMAN. Immunohistochemistry of AMAN rabbits shows disruption of nodal sodium channel clusters and detachment of paranodal myelin terminal loops, similar to paranodal demyelination, which would significantly reduce the safety factor for impulse transmission and might be responsible for the rapidly reversible conduction block frequently present in human AMAN. C. jejuni sialyltransferase (Cst-II), which functions in the biosynthesis of ganglioside-like LOSs, determines the transferase activity. Strains with cst-II (Thr51) express GM1 and GD1a epitopes, whereas GBS patients infected with cst-II (Thr51) strains have anti-GM1 or anti-GD1a IgG antibodies. The cst-II gene is responsible for the development of GBS. Immunological, pathological, electrophysiological, and bacteriological studies have provided strong evidence of carbohydrate mimicry being a cause of AMAN and clarified the mechanisms of nerve conduction failure in AMAN.