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由于包括下行束发育不全在内的多种脑异常导致的类莫比乌斯综合征。病例报告。

Möbius-like syndrome due to multiple cerebral abnormalities including hypoplasia of the descending tracts. A case report.

作者信息

Nardelli E, Vio M, Ghersini L, Rizzuto N

出版信息

J Neurol. 1982;227(1):11-9. doi: 10.1007/BF00313542.

Abstract

The clinicopathological findings are reported in a child with congenital oculofacial diplegia, paresis of pharyngeal and laryngeal muscles, and concomitant tetraparesis. The constellation of signs and symptoms correlates with a complex picture of maldevelopment, including absence of olfactory bulbs, hippocampal abnormalities, hypoplasia of the corpus callosum and of the cerebellum, and severe hypoplysia of the descending pathways. We stress the role played by the hypoplasia of the descending fibres, with consequent denervation of the cranial nerve nuclei, in producing the Möbius-like picture.

摘要

本文报告了一名患有先天性眼面部麻痹、咽和喉部肌肉麻痹并伴有四肢轻瘫的儿童的临床病理结果。体征和症状的组合与发育异常的复杂情况相关,包括嗅球缺失、海马体异常、胼胝体和小脑发育不全,以及下行通路严重发育不全。我们强调下行纤维发育不全所起的作用,其导致脑神经核失神经支配,从而产生类似莫比乌斯综合征的表现。

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