Miller M T, Strömland K
Department of Ophthalmology, Eye and Ear Infirmary, University of Illinois at Chicago, 60612, USA.
J AAPOS. 1999 Aug;3(4):199-208. doi: 10.1016/s1091-8531(99)70003-0.
The constellations of findings often referred to as Mobius syndrome might be better described as Mobius sequence, because the term sequence defines a cascade of secondary events after an embryonic insult from heterogeneous causes. Classic clinical findings include evidence of sixth and seventh cranial nerve involvement, often with associated malformations of limbs, craniofacial structures, and other cranial nerves.
A prospective study was undertaken in Sweden of 25 patients who showed characteristic findings of Mobius sequence.
Of the patients who did not have strabismus surgery, 10 patients had straight eyes in the primary position, 7 had esotropia, 2 had exotropia, and 1 had hypertropia. All had significant limitation of abduction, except 1 patient with exotropia who showed minimal underaction on abduction but a large limitation of adduction. In the description in these early cases, some patients manifested a clinical pattern resembling a horizontal gaze paresis. Narrowing of the palpebral fissure on adduction similar to that seen in Duane syndrome was observed in a few cases. Two patients had ptosis. Nineteen patients had diminished facial expression bilaterally, often asymmetric, and 6 cases appeared to be unilateral. Seven patients had abnormal tearing. Associated systemic findings included Poland anomaly (2), club feet or other limb anomalies (8), micrognathia (8), tongue malformations (17), cleft palate (5), and speech problems (18). An unusual finding was autism syndrome (6) or autism-like syndrome (1).
The associated findings in Mobius sequence may give further clues to the location and timing of the developmental disturbance. The wide range of ocular motility patterns suggests that the previous concept of a lesion solely in the sixth nerve nucleus is an inadequate explanation for these findings.
通常被称为莫比乌斯综合征的一系列症状可能更宜被描述为莫比乌斯序列，因为“序列”一词定义了由多种异质性病因导致胚胎受损后一系列继发事件。典型的临床症状包括第六和第七颅神经受累的证据，常伴有肢体、颅面结构及其他颅神经的相关畸形。
在瑞典对25例表现出莫比乌斯序列特征性症状的患者进行了一项前瞻性研究。
在未接受斜视手术的患者中，10例患者第一眼位时眼位正，7例有内斜视，2例有外斜视，1例有上斜视。除1例外斜视患者外展时仅有轻微运动不足但内收受限明显外，所有患者外展均有明显受限。在这些早期病例的描述中，一些患者表现出类似水平凝视麻痹的临床模式。少数病例观察到内收时睑裂变窄，类似于杜安综合征所见。2例患者有上睑下垂。19例患者双侧面部表情减弱，常不对称，6例似乎为单侧。7例患者有异常流泪。相关的全身表现包括波兰综合征（2例）、马蹄内翻足或其他肢体畸形（8例）、小颌畸形（8例）、舌畸形（17例）、腭裂（5例）和言语问题（18例）。一个不寻常的发现是自闭症综合征（6例）或自闭症样综合征（1例）。
莫比乌斯序列中的相关表现可能为发育障碍的位置和时间提供进一步线索。眼动模式的广泛差异表明，以往仅将病变定位于第六神经核的概念不足以解释这些表现。
