Pathology of the motor-sensory axonal Guillain-Barre syndrome
Pathology of the motor-sensory axonal Guillain-Barre syndrome
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DOI：
10.1002/ana.410390105
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发表时间：
1996-01-01
影响因子：
11.2
通讯作者：
Asbury, AK
中科院分区：
文献类型：
--
作者：
Griffin, JW;Li, CY;Asbury, AK
由免疫攻击轴突引起的急性发作的严重运动感觉神经病（“轴突性”格林-巴利综合征）的概念主要是基于电诊断和有限的病理学数据提出的，但仍存在争议。在尸检中，一些病例表现出异常严重的炎性脱髓鞘神经病。关于先前的空肠弯曲菌感染是否与“轴索型”格林-巴利综合征相关，目前存在相互矛盾的数据。我们报告了4例来自中国河北省的患者，他们在临床诊断为格林-巴利综合征的综合征发病后7、7、18和60天死亡。高滴度的抗体识别C。空肠符合近期感染，发现在2名患者测试。尸检时，3例早期疾病患者的腹根和背根以及周围神经中的纤维出现持续的沃勒样变性，仅有极轻微的脱髓鞘或淋巴细胞浸润。所有3例患者的有髓节间轴突周围间隙中均存在大量巨噬细胞，以及罕见的轴突内巨噬细胞。对患有该综合征的患者进行了60天的检查，证实了脊神经根和神经中大纤维的广泛丢失，以及脱髓鞘和髓鞘再生的缺乏。这些观察结果证实了这样的预测，即临床上定义的一些严重运动-感觉格林-巴利综合征患者，即使在疾病的早期阶段，也主要有运动和感觉纤维的轴突损伤，并且轴突格林-巴利综合征可以在C.空肠感染病理学支持这样的可能性，即运动感觉轴突格林-巴利综合征的病例代表了针对轴突上表位的免疫攻击谱的最严重的一端。
The concept of a severe motor-sensory neuropathy of acute onset caused by an immune attack on the axon (''axonal'' Guillain-Barre syndrome) has been advanced primarily based on electrodiagnostic and limited pathological data, but remains controversial. At autopsy some cases demonstrate unusually severe inflammatory demyelinating neuropathy. There are conflicting data about whether antecedent Campylobacter jejuni infection is associated with ''axonal'' Guillain-Barre syndrome. We report 4 individuals from Hebei Province, China, who died 7, 7, 18, and 60 days after onset of a syndrome diagnosed clinically as Guillain-Barre syndrome. High titers of antibodies recognizing C. jejuni consistent with recent infection, were found in the 2 patients tested. At autopsy the 3 with early disease had ongoing wallerian-like degeneration of fibers in the ventral and dorsal roots and in the peripheral nerves, with only minimal demyelination or lymphocytic infiltration. All 3 had numerous macrophages in the periaxonal space of myelinated internodes, and rare intraaxonal macrophages as well. Examination of the patient having the syndrome for 60 days confirmed the extensive loss of large fibers in the spinal roots and nerves, and the paucity of demyelination and remyelination. These observations confirm predictions that some patients with severe motor-sensory Guillain-Barre syndrome, as defined clinically, have predominantly axonal lesions of both motor and sensory fibers, even in the early stages of the disease, and that axonal Guillain-Barre syndrome can follow C. jejuni infection. The pathology supports the possibility that such cases of motor-sensory axonal Guillain-Barre syndrome represent the most severe end of a spectrum of immune attack directed toward epitopes on the axon.