Cernea P, Grapă C
Clinica Oftalmologică, Craiova.
Oftalmologia. 1991;35(3-4):79-84.
The patient, 9 years old, presents bilateral congenital paralysis of the facial, disability of the ocular globes abduction, facial cutanate atrophy and partial paralysis of hypoglos, syndrome manifestations that are framed in the disease described by Moebius. The patient also presents epicanthus, hiperthlorysm, atresia of the tear points. The disease is caused by some teratogenic factors which act between the fourth and the seventh week of the intrauterine life with the atresia of the sixth and seventh nucleus and partially of the ninth nucleus as well as of the supranuclear way from the proximity of these nucleus. The variety of the clinical manifestations can be explained through the affectation of the different nervous structures during the embryogenesis, but the congenital paralysis of the abduction and the facial diplegia are the most important manifestations of the syndrome.
该患者9岁，表现为双侧先天性面瘫、眼球外展功能障碍、面部皮肤萎缩和舌下神经部分麻痹，这些综合征表现符合默比厄斯描述的疾病。患者还伴有内眦赘皮、眼睑裂狭小、泪点闭锁。该疾病由一些致畸因素引起，这些因素在子宫内生活的第4至7周起作用，导致第六和第七核以及部分第九核闭锁，以及来自这些核附近的核上通路闭锁。临床表现的多样性可通过胚胎发育过程中不同神经结构的受累来解释，但外展先天性麻痹和双侧面瘫是该综合征最重要的表现。
