Dooley Joseph M, Stewart Wendy A, Hayden James D, Therrien Arawn
Department of Pediatrics, Division of Pediatric Neurology, Dalhousie University and IWK Health Centre, Halifax, Nova Scotia, Canada.
Pediatr Neurol. 2004 Jan;30(1):39-41. doi: 10.1016/s0887-8994(03)00408-9.
Möbius syndrome is characterized by congenital facial diplegia, and may be associated with limb or orofacial malformations. A number of mechanisms have been proposed to explain the pathogenesis, including prenatal ischemia. We identified seven children with Möbius syndrome over the 10-year interval 1992-2001, all of whom manifested incomplete bilateral facial palsy. Associated limb and orofacial anomalies were observed in six cases. Computed tomographic scans were available in six children, and five of them manifested brainstem calcification which was most prominent in the floor of the fourth ventricle. The calcification was detected as early as 7 days of age and did not change with time, suggesting a static condition of prenatal onset. These observations support the hypothesis that the pathology in Möbius syndrome is secondary to prenatal brain ischemia.
莫比乌斯综合征的特征为先天性面瘫，可能伴有肢体或口面部畸形。已经提出了多种机制来解释其发病机制，包括产前缺血。我们在1992年至2001年的10年间确定了7例莫比乌斯综合征患儿，他们均表现为不完全性双侧面瘫。6例观察到相关的肢体和口面部异常。6名儿童进行了计算机断层扫描，其中5名表现出脑干钙化，在第四脑室底部最为明显。钙化最早在7日龄时被检测到，且不随时间变化，提示为产前发病的静止状态。这些观察结果支持了莫比乌斯综合征的病理改变继发于产前脑缺血这一假说。
