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韦伯综合征

Weber Syndrome

作者信息

Munakomi Sunil, Das Joe M.

机构信息

Kathmandu University

Imperial College Healthcare NHS Trust, London

PMID:32644584
Abstract

Weber's syndrome was described first by the German-born English physician Hermann Weber in a 52-year-old male who had developed left-sided third nerve palsy with right-sided hemiplegia caused by an acute bleed in the left cerebral peduncle. Weber syndrome, classically described as a midbrain stroke syndrome and superior alternating hemiplegia, involves oculomotor fascicles in the interpeduncular cisterns and cerebral peduncle, thereby causing ipsilateral third nerve palsy with contralateral hemiparesis. It most commonly results from the occlusion of a branch of the posterior cerebral artery. The oculomotor(third cranial) nerve has two main motor nuclei, the main motor nucleus, and the accessory parasympathetic nucleus. The main motor nucleus is located in the tectum portion of the midbrain at the level of the superior colliculus and supplies all the extraocular muscles except the lateral rectus and the superior oblique muscles, and the levator palpebrae superioris. The accessory parasympathetic nucleus, also known as the Edinger Westphal nucleus, is situated posterior to the main motor nucleus and its postganglionic fibers pass through the short ciliary nerves to supply the constrictor pupillae of the iris and the ciliary muscles. The nerve fascicles then travel forward and lateral through the red nucleus and converge at the interpeduncular fossa before exiting the midbrain. As the nucleus and fascicles are spread over a large area of the midbrain, lesions in the midbrain can present with either partial or complete third nerve palsy. The lesions in lower midbrain affect the extraocular muscles but spare the pupils, while lesions affecting both upper and middle parts of the midbrain are associated with pupillary dilatation.

摘要

韦氏综合征最早由出生于德国的英国医生赫尔曼·韦伯描述,患者为一名52岁男性,因左侧大脑脚急性出血,出现左侧动眼神经麻痹伴右侧偏瘫。韦氏综合征,经典描述为中脑卒中和上交替性偏瘫,累及脚间池和大脑脚内的动眼神经束,从而导致同侧动眼神经麻痹伴对侧偏瘫。其最常见的病因是大脑后动脉分支闭塞。动眼神经(第三脑神经)有两个主要运动核,即主要运动核和副交感神经核。主要运动核位于中脑上丘水平的顶盖部分,支配除外直肌、上斜肌和上睑提肌以外的所有眼外肌。副交感神经核,也称为埃丁格 - 韦斯特法尔核,位于主要运动核后方,其节后纤维通过睫状短神经支配虹膜瞳孔括约肌和睫状肌。神经束随后向前和外侧穿过红核,在脚间窝汇聚,然后从中脑穿出。由于核和神经束分布在中脑的大片区域,中脑病变可表现为部分或完全性动眼神经麻痹。中脑下部的病变影响眼外肌,但不累及瞳孔,而影响中脑上部和中部的病变则伴有瞳孔散大。

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