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Fourth Cranial Nerve Palsy and Brown Syndrome: Two Interrelated Congenital Cranial Dysinnervation Disorders?

  • Neuro-ophthalmology (A Kawasaki, Section Editor)
  • Published:
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Abstract

Based on neuroimaging data showing absence of the trochlear nerve, congenital superior oblique palsy is now classified as a congenital cranial dysinnervation disorder. A similar absence of the abducens nerve is accompanied by misinnervation to the lateral rectus muscle from a branch of oculomotor nerve in the Duane retraction syndrome. This similarity raises the question of whether some cases of Brown syndrome could arise from a similar synkinesis between the inferior and superior oblique muscles in the setting of congenital superior oblique palsy. This hypothesis has gained support from the confluence of evidence from a number of independent studies. Using Duane syndrome as a model, we critically review the accumulating evidence that some cases of Brown syndrome are ultimately attributable to dysgenesis of the trochlear nerve.

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Acknowledgment

Supported in part by a grant from Research to Prevent Blindness, Inc.

Conflict of Interest

Pierre-François Kaeser declares that he has no conflict of interest.

Michael C. Brodsky declares that he has no conflict of interest.

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Authors and Affiliations

  1. Jules Gonin Eye Hospital, University of Lausanne, Avenue de France 15, 1004, Lausanne, Switzerland

    Pierre-François Kaeser

  2. Departments of Ophthalmology and Neurology, Mayo Clinic, Rochester, MN, USA

    Michael C. Brodsky

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  1. Pierre-François Kaeser
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  2. Michael C. Brodsky
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Correspondence to Pierre-François Kaeser.

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This article is part of the Topical Collection on Neuro-ophthalmology

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Kaeser, PF., Brodsky, M.C. Fourth Cranial Nerve Palsy and Brown Syndrome: Two Interrelated Congenital Cranial Dysinnervation Disorders?. Curr Neurol Neurosci Rep 13, 352 (2013). https://doi.org/10.1007/s11910-013-0352-5

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  • DOI: https://doi.org/10.1007/s11910-013-0352-5

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