Allen Brian M, Wert Matthew A, Tatum Sherard A
SUNY Upstate Medical University, 750 East Adams Street Syracuse, NY 13210, USA.
Int J Pediatr Otorhinolaryngol. 2006 May;70(5):931-4. doi: 10.1016/j.ijporl.2005.09.013. Epub 2005 Oct 17.
Mobius syndrome was originally described as bilateral 6th and 7th cranial nerve palsy and has since been expanded to include involvement of nearly all cranial nerves, limb deformities, orofacial anomalies and deficits in intellectual function. The etiology of Mobius syndrome remains elusive. Here we present a case with unilateral 5th, 7th, 9th, 10th and 12th cranial nerve palsy along with MRI evidence of ipsilateral brainstem hypoplasia. We propose the unilateral pathology supports the theory that vascular malformation in utero, which can be due to various etiologies, leads to brainstem ischemia and is the key factor in development of Mobius syndrome.
默比厄斯综合征最初被描述为双侧第六和第七颅神经麻痹，此后其范围已扩大到几乎包括所有颅神经受累、肢体畸形、口面部异常以及智力功能缺陷。默比厄斯综合征的病因仍然不明。在此，我们报告一例单侧第五、第七、第九、第十和第十二颅神经麻痹的病例，同时伴有同侧脑干发育不全的MRI证据。我们提出，这种单侧病变支持如下理论：子宫内血管畸形（其可由多种病因引起）导致脑干缺血，是默比厄斯综合征发病的关键因素。
