GUILLAIN-BARRE-SYNDROME IN NORTHERN CHINA - THE SPECTRUM OF NEUROPATHOLOGICAL CHANGES IN CLINICALLY DEFINED CASES
GUILLAIN-BARRE-SYNDROME IN NORTHERN CHINA - THE SPECTRUM OF NEUROPATHOLOGICAL CHANGES IN CLINICALLY DEFINED CASES
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DOI：
10.1093/brain/118.3.577
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发表时间：
1995-06-01
期刊：
影响因子：
14.5
通讯作者：
ASBURY, AK
中科院分区：
文献类型：
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作者：
GRIFFIN, JW;LI, CY;ASBURY, AK
吉兰-巴雷综合征的病理学仍有争议，可用当代技术研究的尸检病例并不多见。临床诊断为格林-巴利综合征的病例大量发生在中国北方。本文对河北省12例尸检病例的神经病理学改变进行了研究。11人在患病初期死亡。在所有情况下，组织进行了特殊处理和固定，用于电子显微镜检查和免疫细胞化学。这12例病例中有3例患有典型的急性炎症性脱髓鞘性多发性神经病（AIDP），伴有淋巴细胞浸润和巨噬细胞介导的脱髓鞘，重现了北美、欧洲和澳大利亚格林-巴利综合征中最常报告的病理表现。6例主要累及轴突，特征为新纤维的沃勒样变性，5例仅伴有轻微脱髓鞘和轻微炎症。3例患者死亡时虽已瘫痪，但脊神经根和坐骨神经仅有非常轻微的变化。在六个主要轴突病例组中，异常的严重程度和涉及的纤维类别都有重要差异。3例患者的感觉和运动纤维出现广泛的沃勒样变性[急性运动感觉轴突神经病（AMSAN）]，而在其他3例患者中，纤维变性几乎完全影响运动神经纤维。这些后者的情况下建立了一个结构基础的临床和电生理图片称为急性运动轴索神经病（AMAN）模式。在AMAN和AMSAN模式中，一个突出的特征是轴突周围空间内存在巨噬细胞，包围或取代轴突，并被完整的髓鞘包围。这些研究表明，临床诊断为格林-巴利综合征的病例的早期病理变化是多种多样的，并不局限于众所周知的AIDP模式，世界不同地区的主要病理模式可能不同。急性炎性脱髓鞘性多发性神经病与轴索型神经病的病理表现差异可能反映了发病机制的不同。轴索模式中的轴索周巨噬细胞表明，一个重要的表位可能定位于轴膜或轴索周间隙。轻度病例表明，严重瘫痪可发生在格林-巴利综合征早期，但沿神经沿着无明显结构改变，提示可能与生理阻滞或神经末梢改变有关。
The pathology of the Guillain-Barre syndrome remains controversial, and autopsied cases available for study by contemporary techniques are uncommon. Large numbers of cases clinically diagnosed as Guillain-Barre syndrome occur in northern China. In this study we examined the neuropathological changes in 12 autopsied cases from Hebei Province, China. Eleven died early in the course of their disease. In all cases tissue was specially handled and fixed for electron microscopy and for immunocytochemistry. Three of these 12 cases had typical acute inflammatory demyelinating polyneuropathy (AIDP) with lymphocytic infiltration and macrophage-mediated demyelination, reproducing the pathological picture most often reported in Guillain-Barre syndrome in North America, Europe, and Australia. Six cases had predominantly axonal involvement, characterized by Wallerian-like degeneration of new fibres, with only minimal demyelination and with minimal inflammation in five. Three cases, even though paralysed at the time of death, had only very mild changes in the spinal roots and sciatic nerves. Within the group of six predominantly axonal cases, there were important differences both in the severity of the abnormalities and in the classes of fibres involved. Three cases had extensive Wallerian-like degeneration of sensory as well as motor fibres [acute motor-sensory axonal neuropathy (AMSAN)], while in the other three cases the fibre degeneration affected the motor nerve fibres almost exclusively. These latter cases establish a structural basis for the clinical and electrophysiological picture termed the acute motor axonal neuropathy (AMAN) pattern. In both the AMAN and the AMSAN patterns, a prominent feature was the presence of macrophages within the periaxonal space, surrounding or displacing the axon, and surrounded by an intact myelin sheath. These studies show that the early pathological changes in cases clinically diagnosed as the Guillain-Barre syndrome are diverse and not restricted to the well-known pattern of AIDP and that the predominant pathological patterns may differ in different parts of the world. The differences in pathological findings gs between acute inflammatory demyelinating polyneuropathy and the axonal patterns are likely to reflect differences in the pathogenetic mechanisms. The periaxonal macrophages in the axonal patterns suggest that an important epitope may be localized to the axolemma or periaxonal space. The mild cases indicate that severe paralysis can occur early in Guillain-Barre syndrome without prominent structural changes along the nerve, suggesting that physiological block or nerve terminal changes may be implicated.