de Souza-Dias Carlos Ramos, Goldchmit Mauro
Departamento de Oftalmologia, Faculdade de Ciências Médicas, Santa Casa de São Paulo, São Paulo, SP, Brasil.
Arq Bras Oftalmol. 2007 May-Jun;70(3):451-7. doi: 10.1590/s0004-27492007000300012.
There is no uniformity in the literature about the core features required to make the diagnosis of Möbius sequence. Originally, the minimum requirements were the bilateral paralysis of the VI and the VII cranial nerves. The bilateral facial nerve paralysis or paresis, often asymmetric, is common to all patients but some facts show that the isolated VI nerve palsy in the Möbius sequence is not the rule. 1) When there is an esotropia in Möbius sequence, it is often too small to be caused by a bilateral isolated VI nerve palsy. There are many cases in which there is no esotropia in the primary position and even some cases, though rare, with exotropia. 2) In most cases of Möbius sequence, the esotropia can be eliminated with a mere recession of the medial rectus muscles. 3) In most patients with Möbius sequence there is, besides the lateral rectus palsy, a variable degree of adduction limitation, which defines a horizontal gaze palsy. The authors present some arguments to show that the isolated lateral rectus muscle palsy cannot be considered as a sine qua non factor for the diagnosis of Möbius sequence.
The binocular alignment in primary position and the incidence of abduction and adduction limitations among 28 of the authors' consecutive patients with Möbius sequence and in patients of 5 other randomly selected publications are presented for comments.
The eyes' position in primary position among 135 of those 6 authors' patients (28 belonging to the authors of this study and 107 to the other 5) were recorded; 55 of them (40.74%) had orthotropia and 9 (6.66%) had exotropia. Among 80 patients of 4 authors (22 belonging to the authors of this study and 52 to the other 3), in whom the horizontal versions were analyzed, 79 (98.75%) had limitation of abduction and 53 (66.25%) had limitation of adduction.
The authors emphasize that the recent studies have shown that inside the VI nerve nucleus there are two types of cells: those which axons form the ipsolateral abducens nerve and those (interneurons) whose axons reach the medial longitudinal fasciculus and ascend for innervating the subnucleus of the contralateral III nerve subserving the contralateral medial rectus. Because of this arrangement, a lesion at the region of the VI nerve nucleus generally causes a paralysis of the ipsolateral lateral rectus and the contralateral medial rectus muscles, which characterizes the ipsolateral horizontal gaze palsy.
The definition of the Möbius sequence is the paralysis of the facial nerve and the horizontal gaze palsy, instead of a VI nerve palsy, as seen in most published papers.
关于做出莫比乌斯序列诊断所需的核心特征，文献中尚无统一标准。最初，最低要求是双侧第六和第七颅神经麻痹。双侧面神经麻痹或轻瘫，通常不对称，在所有患者中都很常见，但一些事实表明，莫比乌斯序列中孤立的第六神经麻痹并非普遍规律。1）当莫比乌斯序列存在内斜视时，其程度往往过小，不可能由双侧孤立的第六神经麻痹引起。有许多病例在第一眼位时并无内斜视，甚至有些病例（虽罕见）存在外斜视。2）在大多数莫比乌斯序列病例中，仅通过内直肌后徙就能消除内斜视。3）在大多数莫比乌斯序列患者中，除了外直肌麻痹外，还存在不同程度的内收受限，这构成了水平凝视麻痹。作者提出一些论据以表明，孤立的外直肌麻痹不能被视为莫比乌斯序列诊断的必要因素。
呈现了作者连续收治的28例莫比乌斯序列患者以及其他5篇随机选取的出版物中患者的第一眼位双眼视轴对齐情况，以及外展和内收受限的发生率以供评论。
记录了这6位作者的135例患者（28例为本研究作者的患者，107例为其他5篇出版物的患者）的第一眼位眼位；其中55例（40.74%）为正视，9例（6.66%）为外斜视。在4位作者的80例患者（22例为本研究作者的患者，52例为其他3篇出版物的患者）中，对其水平向运动进行了分析，79例（98.75%）存在外展受限，53例（66.25%）存在内收受限。
作者强调，最近的研究表明，在第六神经核内部有两种类型的细胞：其轴突形成同侧展神经的细胞，以及其轴突到达内侧纵束并上行以支配对侧第三神经亚核（该亚核为对侧内直肌提供神经支配）的细胞（中间神经元）。由于这种结构，第六神经核区域的病变通常会导致同侧外直肌和对侧内直肌麻痹，这是同侧水平凝视麻痹的特征。
莫比乌斯序列的定义是面神经麻痹和水平凝视麻痹，而非如大多数已发表论文中所述的第六神经麻痹。
