Investig Magn Reson Imaging.  2019 Jun;23(2):167-171. 10.13104/imri.2019.23.2.167.

Möbius Syndrome Demonstrated by the High-Resolution MR Imaging: a Case Report and Review of Literature

• Hwang M
• Baek HJ
• Ryu KH
• Choi BH
• Ha JY
• Do HJ

• KMID: 2452532
• DOI: http://doi.org/10.13104/imri.2019.23.2.167

Abstract

Möbius syndrome is a rare congenital condition, characterized by abducens and facial nerve palsy, resulting in limitation of lateral gaze movement and facial diplegia. However, to our knowledge, there have been few studies on evaluation of cranial nerves, on MR imaging in Möbius syndrome. Herein, we describe a rare case of Möbius syndrome representing limitation of lateral gaze, and weakness of facial expression, since the neonatal period. In this case, high-resolution MR imaging played a key role in diagnosing Möbius syndrome, by direct visualization of corresponding cranial nerves abnormalities.

Keyword

Möbius syndrome; Magnetic resonance imaging; Facial nerve; Palsy

MeSH Terms

Cranial Nerves
Facial Expression
Facial Nerve
Magnetic Resonance Imaging*
Paralysis

Figure

• 

  Fig. 1 A 19-year-old male patient with Möbius syndrome, with facial palsy and bilateral abduction limitation. (a) It shows limitation of facial expressions, due to bilateral facial palsy. (b) Ocular versions demonstrating marked limitation of lateral gaze of both eyes, with no definite limitation of adduction, supraduction, and infraduction of eye movement.

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  Fig. 2 Axial T2 VISTA (a, c, e) and axial FLAIR VISTA (b, d, f) images presenting characteristic imaging features of Möbius syndrome. (a–d) The left facial nerve is absent in the cerebellopontine cistern, and internal auditory canal. The right facial nerve shows markedly thin linear structures, suggesting severe hypoplasia (black arrows in c). Bilateral vestibulocochlear nerves are clearly depicted, with normal configuration. Bilateral abducens nerves are not visualized, in the prepontine cistern (white arrows in b). (e–f) At the level of middle cerebellar peduncles, bilateral abducens nerves show severe hypoplasia, and nerves are more conspicuously delineated on 3D FLAIR VISTA image (white arrows in f) than 3D T2 VISTA image (black arrows in e).

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  Fig. 3 Axial T2 VISTA (a) and sagittal FLAIR VISTA (b) images, show flattened posterior pons (black arrows in a), and straightening with mild inward deformity of the fourth ventricular floor (white arrows in b), due to absence of bilateral medial facial colliculi at the mid pons level.

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