Ricoy J R, Cabello A, Rodriguez J, Téllez I
Brain. 1983 Dec;106 ( Pt 4):817-35. doi: 10.1093/brain/106.4.817.
Biopsies of muscle and sural nerves, and autopsies of patients affected by the toxic and neuromuscular syndrome produced by ingestion of adulterated rapeseed oil were studied using morphological, histochemical and ultrastructural methods. In muscle, two pathological pictures were distinguished according to their temporal sequence. In the early phase, the neuromuscular syndrome was characterized by myalgia and an inflammatory infiltration of the perimysium, the capsules of muscle spindles and intramuscular nerves. The muscle fibres exhibited small subsarcolemmal zones of fibril disintegration and accumulation of electron-dense material similar to Z bands. Areas of peroxidase activity were found in relation to the surface of many muscle fibres. In late stages there was severe neurogenic atrophy of muscle with intense endomysial fibrosis. Minimal perivascular inflammation by round cells, with no interstitial infiltrates, was finally present. The inflammatory myopathy that initially affected these patients differs from other forms of polymyositis and seems to be related to the inflammation present in other systems. The onset of denervation atrophy is secondary to the involvement of peripheral nerves which is the most salient and distinctive pathological feature of the syndrome. The involvement of peripheral nerves was the most severe pathological feature. Perineuritis and, later, fibrosis of the perineurium were conspicuous and peculiar to this toxic syndrome. Degeneration of myelinated axons was constant in late stages. Distal nerves were more affected than proximal nerves. In the CNS, chromatolysis of anterior horn cells and occasionally of cranial nerve nuclei, pontine nuclei and reticular neurons was found. In the brainstem, astrocytes were hypertrophic with abnormal nuclei and there was microglial proliferation in zones where chromatolysis was found. The possibility that free radicals derived from the adulterated oil and cytotoxic complexes formed by mast-cell granules and eosinophil peroxidases might have been involved in the pathogenesis is discussed. The toxic oil was rich in linoleic acid which, by forming an excess of arachidonic acid, might have played an additional role in the pathogenesis of the lesions.
采用形态学、组织化学和超微结构方法，对摄入掺假菜籽油所致中毒性神经肌肉综合征患者的肌肉和腓肠神经活检标本以及尸体解剖样本进行了研究。在肌肉中，根据病理变化的时间顺序可区分出两种病理表现。在早期，神经肌肉综合征的特征为肌痛以及肌束膜、肌梭囊和肌内神经的炎性浸润。肌纤维在肌膜下呈现小区域的原纤维崩解以及类似于Z带的电子致密物质积聚。在许多肌纤维表面发现了过氧化物酶活性区域。在后期，出现严重的神经源性肌肉萎缩，并伴有强烈的肌内膜纤维化。最终出现轻微的血管周围淋巴细胞炎症，无间质浸润。最初影响这些患者的炎性肌病不同于其他形式的多发性肌炎，似乎与其他系统中存在的炎症有关。去神经萎缩的发生继发于周围神经受累，这是该综合征最显著和独特的病理特征。周围神经受累是最严重的病理特征。神经束膜炎以及随后的神经束膜纤维化在这种中毒综合征中明显且具有特异性。在后期，有髓轴突的变性是持续存在的。远端神经比近端神经受影响更严重。在中枢神经系统中，发现前角细胞以及偶尔的脑神经核、脑桥核和网状神经元出现染色质溶解。讨论了掺假油产生的自由基以及肥大细胞颗粒和嗜酸性粒细胞过氧化物酶形成的细胞毒性复合物可能参与发病机制的可能性。有毒油富含亚油酸，通过形成过量的花生四烯酸，可能在病变的发病机制中起到了额外作用。
