Megnetic resonance imaging findings in sporadic Möbius syndrome
Megnetic resonance imaging findings in sporadic Möbius syndrome
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散发性莫比乌斯综合征的磁共振成像发现
DOI：
10.3760/cma.j.issn.0366-6999.20130334
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发表时间：
2013
影响因子：
6.1
通讯作者：
Zhen
中科院分区：
文献类型：
--
作者：
Shaoqin Wu;F. Man;Y. Jiao;Jun‐fang Xian;Yi;Zhen
背景 尽管莫比乌斯综合征的神经放射学检查结果已被报道为大脑和脑干异常的结果，但磁共振成像 (MRI) 现在允许对脑神经 (CN) 和眼眶分支进行直接成像。本研究介绍了散发性莫比乌斯综合征患者的 MRI 结果。方法 前瞻性地，使用头部线圈和采用稳态采集 (3D-FIESTA) 的三维快速成像 (3D-FIESTA) 对脑池中的 CN 进行成像，在 7 名散发性莫比乌斯综合征患者的 0.8 毫米厚度的平面中产生 0.5 毫米2 的分辨率。所有患者的 CN 海绵体段和眶内段以及眼外肌 (EOM) 均采用 T1 加权进行成像。海绵状段在冠状平面中成像，而准冠状平面中的轨道内使用表面线圈成像。在 2.0 毫米厚的平面内，眶内分辨率为 0.16 毫米2。结果 7 例患者的脑池、海绵窦和眼眶中 CN 缺失或发育不全。受影响一侧没有外展神经 (CN VI) 和面神经 (CN VII)。两例单侧 CN IX（舌咽神经）出现发育不良。观察到 CN III 下支的分支在 3 例双侧和 1 例单侧支配外直肌 (LR)，并且在 2 例双侧和 2 例单侧与 LR 肌有密切的连续性。 5 例显示 EOM 发育不全。一名患者发现左侧髓质发育不良。结论 MRI 对 CN 和 EOM 的直接成像有助于莫比乌斯综合征的诊断。它可以直接显示CN和眼眶结构的异常。 CN VI 和 CN VII 缺失或发育不全可能是散发性莫比乌斯综合征最常见的放射学特征，CN IX 发育不全可能是一个相关特征。应观察 EOM 的异常和眼眶神经支配的异常，这对于病因学的研究可能很重要。
Background Although neuroradiological findings of Möbius syndrome have been reported as a result of brain and brainstem abnormalities, magnetic resonance imaging (MRI) now permits the direct imaging of the cranial nerve (CN) and branches in the orbits. This study presents the MRI findings in patients with sporadic Möbius syndrome. Methods Prospectively, CNs were imaged in the cistern using head coils and three dimensional fast imaging employing steady‐state acquisition (3D‐FIESTA), yielding a 0.5 mm2 resolution in planes of 0.8 mm thickness in seven patients with sporadic Möbius syndrome. The cavernous and intraorbital segment of the CN and the extraocular muscles (EOMs) were imaged with T1 weighting in all patients. The cavernous segment was imaged in coronal planes, while the intraorbit in quasicoronal planes were imaged using surface coils. Intraorbital resolution was 0.16 mm2 within 2.0 mm thick planes. Results In the seven patients, the CN were absent or showed hypoplasia in the cistern, cavernous sinus, and orbit. Abducens (CN VI) and facial (CN VII) nerves were absent on the affected sides. Unilateral CN IX (glossopharyngeal nerve) in two cases displayed dysplasia. Branches from the inferior division of CN III were observed to innervate the lateral rectus (LR) bilaterally in three cases and unilaterally in one case, and had intimate continuity with the LR muscle in two cases bilaterally and two cases unilaterally. Hypoplasia of EOMs was shown in five cases. Dysplasia of the medulla on the left side was found in one patient. Conclusions Direct imaging of CNs and EOMs by MRI is useful in diagnosis of Möbius syndrome. It can directly demonstrate the abnormalities of the CN and orbital structures. The absence or hypoplasia of CN VI and CN VII may be the most common radiologic features in sporadic Möbius syndrome, and hypoplasia of CN IX may be an associated feature. The abnormality of EOMs and aberrant innervations in the orbit should be observed, and may be important for the study of the etiology.
影响因子：
1.6
作者：
Dumars, Sean;Andrews, Caroline;Demer, Joseph L.
通讯作者：
Demer, Joseph L.
影响因子：
1.6
作者：
Demer, Joseph L.;Ortube, Maria Carolina;Thacker, Neepa
通讯作者：
Thacker, Neepa