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POEMS综合征:腓肠神经的超微结构观察

POEMS syndrome: ultrastructural observations on the sural nerve.

作者信息

Teixeira F, Aranda F, Cabañas A, Estañol B

机构信息

Department of Experimental Neuropathology, National Institute of Neurology, Mexico City, Mexico D.F.

出版信息

Ultrastruct Pathol. 1990 Jan-Feb;14(1):31-40. doi: 10.3109/01913129009050872.

DOI:10.3109/01913129009050872
PMID:2153323
Abstract

POEMS is an acronym that stands for a multisystem disorder that includes progressive sensorimotor Polyneuropathy, Organomegaly, Endocrinopathy, an M component in blood and/or concentrated urine, and Skin changes. The morphology of the peripheral nerves was mentioned in some of these cases and ranged from normal to those with segmental demyelination and/or degeneration of myelinated axons. We analyzed quantitatively the sural nerves of two cases of POEMS syndrome. Both nerves showed a severe loss of myelinated fibers, with a minor degree of regeneration. Degeneration of unmyelinated axons was observed, but to a lesser degree. There was marked collagen deposition in the endoneurium. The pathogenesis of the changes in the peripheral nerves and other organs in POEMS syndrome is still to be elucidated.

摘要

POEMS是一个首字母缩写词,代表一种多系统疾病,包括进行性感觉运动性多发性神经病、器官肿大、内分泌病、血液和/或浓缩尿液中的M成分以及皮肤改变。其中一些病例提到了周围神经的形态,范围从正常到节段性脱髓鞘和/或有髓轴突变性。我们对两例POEMS综合征患者的腓肠神经进行了定量分析。两根神经均显示有髓纤维严重缺失,并伴有轻度再生。观察到无髓轴突有变性,但程度较轻。神经内膜有明显的胶原沉积。POEMS综合征中周围神经和其他器官变化的发病机制仍有待阐明。

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POEMS syndrome: ultrastructural observations on the sural nerve.POEMS综合征:腓肠神经的超微结构观察
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引用本文的文献

1
Uncompacted myelin lamellae in polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome. Ultrastructural study of peripheral nerve biopsy from 22 patients.多神经病、器官肿大、内分泌病、M蛋白血症和皮肤改变综合征中的疏松髓鞘板层。对22例患者的周围神经活检进行超微结构研究。
Acta Neuropathol. 1994;87(3):302-7. doi: 10.1007/BF00296746.