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脊髓先天性畸形病例中周围神经系统的形态学变化

Morphological changes in the peripheral nervous system in the case of congenital malformations of the spinal cord.

作者信息

Drac H, Pniewski J, Rafałowska J

机构信息

Department of Neurology, School of Medicine, Warsaw.

出版信息

Neuropatol Pol. 1993;31(1-2):55-61.

PMID:8208441
Abstract

A 21-year-old oligophrenic man developed after upper respiratory tract infection, quadriplegia with sphincter and respiratory disturbances. Lumbar punction revealed subarachnoid bleeding and elevated cerebrospinal protein level. Guillain-Barré syndrome and subarachnoid hemorrhage were diagnosed. At autopsy intraspinal angioma (C2-D6) and diastematomyelia (D11-lumbar segments) were found. Beside, intraspinal hemorrhage was present. Morphological examination of posterior and anterior spinal roots as well as peripheral nerves was done. Spheroids, axonal degeneration and prominent loss of myelinated fibers were observed in the proximal parts of the spinal roots. Axonal degeneration of myelinated fibers and regenerated fibers were noted in the distal parts of spinal roots and in peripheral nerves. Abnormal, fetal-like vessels were present in the spinal roots. Two mechanisms of acute and chronic changes (transneuronal and Wallerian degeneration) are discussed.

摘要

一名21岁的智力发育迟缓男子在上呼吸道感染后出现四肢瘫痪,并伴有括约肌和呼吸功能障碍。腰椎穿刺显示蛛网膜下腔出血和脑脊液蛋白水平升高。诊断为吉兰-巴雷综合征和蛛网膜下腔出血。尸检发现脊髓内血管瘤(C2-D6)和脊髓纵裂(D11-腰段)。此外,存在脊髓内出血。对脊髓前后根以及周围神经进行了形态学检查。在脊髓根近端观察到球状小体、轴突变性和有髓纤维显著减少。在脊髓根远端和周围神经中发现有髓纤维和再生纤维的轴突变性。脊髓根中存在异常的、类似胎儿的血管。讨论了急性和慢性变化的两种机制(跨神经元变性和沃勒变性)。

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