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Neurological Sciences

, Volume 40, Issue 12, pp 2645–2647 | Cite as

Combined superior rectus hypoplasia and superior oblique palsy without a trochlear nerve

  • Hee Kyung Yang
  • Jae Hyoung Kim
  • Jeong-Min HwangEmail author
Letter to the Editor
  • 45 Downloads

Dear Editor,

Congenital cranial dysinnervation disorders (CCDDs) represent a group of neurodevelopmental diseases of the brainstem and cranial nerves [1]. Congenital superior oblique palsy (SOP) is one of the most representative CCDD because 73% of patients show an absent trochlear nerve and superior oblique hypoplasia [2]. CCDD is not limited to a single nerve and sometimes affects two or more nerves [3, 4, 5, 6, 7]. Coexistence of SOP with other CCDDs such as Duane’s retraction syndrome has been reported [2]. However, combined superior rectus hypoplasia and superior oblique palsy without a trochlear nerve has never been reported.

A 35-year-old man presented with persistent diplopia for the past 5 years and an abnormal head posture consisting of a face turn to the right, head tilt to the left, and chin up position. He had noticeable asymmetry of the face. He had previously undergone strabismus surgery twice of a right superior rectus resection followed by left superior rectus...

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

References

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    Gutowski NJ, Bosley TM, Engle EC (2003) 110th ENMC International Workshop: the congenital cranial dysinnervation disorders (CCDDs). Naarden, The Netherlands, 25-27 October, 2002. Neuromuscul Disord 13:573–578CrossRefGoogle Scholar
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    Yang HK, Kim JH, Hwang JM (2012) Congenital superior oblique palsy and trochlear nerve absence: a clinical and radiological study. Ophthalmology 119:170–178CrossRefGoogle Scholar
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    Hwang JM, Park SH (2001) A case of Marcus Gunn jaw winking and pseudo inferior oblique overaction. Am J Ophthalmol 131:148–150CrossRefGoogle Scholar

Copyright information

© Fondazione Società Italiana di Neurologia 2019

Authors and Affiliations

  1. 1.Department of OphthalmologySeoul National University College of Medicine, Seoul National University Bundang HospitalSeongnamSouth Korea
  2. 2.Department of RadiologySeoul National University College of Medicine, Seoul National University Bundang HospitalSeongnamSouth Korea

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