Gibbels E, Berger M, Herbolsheimer M, Korn A, Stammler A
Acta Neuropathol. 1981;55(4):307-18. doi: 10.1007/BF00690995.
Muscle biopsies from the lower extremities of four patients with severe tetraplegic form of diphtheritic polyneuropathy were examined by modern techniques including histochemistry, electron microscopy and morphometric procedures. Until now comparable studies have not been published. The biopsies were removed during the acute stage of the polyneuropathy. We found scattered small angulated muscle fibers beside a more generalized slight atrophy predominantly of type 2 B fibers and targetoid-phenomenons or cores in type 1 fibers. Beside this neurogenic pattern there also were, corresponding with the results of electromyography, primary myogenic alterations with different degenerative phenomenons, suspicious of toxic origin as in cardiac muscle. The intramuscular vessels showed no abnormalities except some perivascular predominantly mononuclear cellular reactions with a remarkable number of cerebriform lymphoid cells, probably T-lymphocytes. No specific pathological alterations could be detected in 11 intramuscular nerves and two motor endplates. This may reflect the more proximal demyelination of human peripheral neurons by the diphtheria toxin as found in experimental diphtheria of the rabbit in contrast to the more distal type of the guinea pig.
对4例患有严重四肢麻痹型白喉性多发性神经病患者的下肢肌肉活检组织采用现代技术进行检查,这些技术包括组织化学、电子显微镜检查和形态测量程序。到目前为止,尚未发表过类似的研究。活检组织是在多发性神经病的急性期取出的。我们发现,除了更普遍的轻度萎缩(主要是2B型纤维萎缩)以及1型纤维中的靶样现象或核心外,还有散在的小角形肌纤维。除了这种神经源性模式外,与肌电图结果一致的是,还存在伴有不同退行性现象的原发性肌源性改变,怀疑其起源与心肌一样具有毒性。肌内血管未见异常,仅见一些血管周围主要为单核细胞反应,伴有大量脑回状淋巴细胞,可能为T淋巴细胞。在11条肌内神经和两个运动终板中未检测到特异性病理改变。这可能反映了与豚鼠更远端类型不同,人类周围神经元被白喉毒素脱髓鞘的部位更靠近近端,这与在兔实验性白喉中发现的情况一致。