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Möbius syndrome: clinico-radiologic correlation

  • Namju Kim
  • Jae Hyoung Kim
  • Ji-Soo Kim
  • Jeong-Min HwangEmail author
Neurophthalmology
  • 155 Downloads

Abstract

Purpose

Möbius syndrome is characterized by abducens and facial nerve palsy. However, the presence/absence of corresponding cranial nerves on MRI was not fully evaluated. The purpose of this study was to investigate the mechanism of Möbius syndrome by associating the presence of abducens and facial nerves on MR imagings with clinical features.

Methods

We retrospectively reviewed the medical records and MR imagings of nine patients with Möbius syndrome between January 2004 and October 2015. The presence/absence of abducens and facial nerves on MR imaging, as well as corresponding neuro-ophthalmologic clinical features, was investigated.

Results

Facial palsy was bilateral in six and unilateral in three patients. Abduction was limited bilaterally in five and unilaterally in four patients. The degrees of facial palsy and abduction limitation were variable, and asymmetric even in the bilateral cases. MR imaging documented bilateral absence of the abducens and facial nerves in six, absence of unilateral facial nerve and bilateral abducens nerves in one, and absence of facial and abducens nerves unilaterally on the same side in another. Both abducens and facial nerves were visualized bilaterally only in the one remaining patient.

Conclusions

The absence of abducens and facial nerves on MR imaging was mostly correlated with the findings of facial palsy and abduction limitation in patients with Möbius syndrome. MR imaging aids in diagnosis of Möbius syndrome by documenting the absence or presence of abducens and facial nerves.

Keywords

Möbius syndrome Abducens nerve Facial nerve MR imaging 

Notes

Authors’ contributions

The following contributions were made by the coauthors. Design of the study (NK, JMH); conduct of the study (NK, JHK); manuscript preparation (NK, JSK, JHK); and review or approval of manuscript (JMH).

Compliance with ethical standards

Conflict of interest

All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge, or beliefs) in the subject matter or materials discussed in this manuscript.

Ethics approval

This retrospective study was approved by the Institutional Review Board of the Seoul National University Bundang Hospital.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Namju Kim
    • 1
  • Jae Hyoung Kim
    • 2
  • Ji-Soo Kim
    • 3
  • Jeong-Min Hwang
    • 1
    Email author
  1. 1.Department of Ophthalmology, Seoul National University College of MedicineSeoul National University Bundang HospitalSeongnamSouth Korea
  2. 2.Department of Radiology, Seoul National University College of MedicineSeoul National University Bundang HospitalSeongnamSouth Korea
  3. 3.Department of Neurology, Seoul National University College of MedicineSeoul National University Bundang HospitalSeongnamSouth Korea

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