Moebius Syndrome: Difference between revisions
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'''Original Editor '''- | '''Original Editor '''- [http://www.physio-pedia.com/User:Wendy_Walker Wendy Walker] | ||
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Please see the [[Facial nerve|Facial Nerve]] and [[Facial Palsy|Facial Palsy]] pages for information on the anatomy of the seventh cranial nerve, AKA facial nerve. | Please see the [[Facial nerve|Facial Nerve]] and [[Facial Palsy|Facial Palsy]] pages for information on the anatomy of the seventh cranial nerve, AKA facial nerve. | ||
Cranial nerve six, AKA the abducens nerve, innervates the ipsilateral lateral rectus muscle which abducts the ipsilateral eye. The sixth nerve nucleus is located in the pons, just ventral to the floor of the fourth ventricle. Bilateral palsy of the abducens nerve (as seen in Moebius Syndrome) results in lack of eye movement in the horizontal plane, ie. inability to move the eyes laterally. This can be observed by testing lateral tracking of eye movement (keeping the head still) and also when the individual reads text, it is apparent that they have to move their head laterally. | Cranial nerve six, AKA the abducens nerve, innervates the ipsilateral lateral rectus muscle which abducts the ipsilateral eye. The sixth nerve nucleus is located in the pons, just ventral to the floor of the fourth ventricle. Bilateral palsy of the abducens nerve (as seen in Moebius Syndrome) results in lack of eye movement in the horizontal plane, ie. inability to move the eyes laterally. This can be observed by testing lateral tracking of eye movement (keeping the head still) and also when the individual reads text, it is apparent that they have to move their head laterally. | ||
== Mechanism of Injury / Pathological Process<br> == | == Mechanism of Injury / Pathological Process<br> == | ||
Revision as of 22:50, 13 May 2014
Original Editor - Wendy Walker
Lead Editors
Definition/Description
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Moebius, or Möbius, syndrome is defined as congenital facial palsy combined with abnormal ocular abduction, caused by abnormal development of the 6th and 7th cranial nerves. It is a rare congenital condition that was first described by German neurologist Paul Julius Möbius in 1888[1].
Moebius syndrome is due to the loss of function of motor cranial nerves. Most cases are diagnosed during infancy. The disease is not progressive.
Clinically Relevant Anatomy
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Please see the Facial Nerve and Facial Palsy pages for information on the anatomy of the seventh cranial nerve, AKA facial nerve.
Cranial nerve six, AKA the abducens nerve, innervates the ipsilateral lateral rectus muscle which abducts the ipsilateral eye. The sixth nerve nucleus is located in the pons, just ventral to the floor of the fourth ventricle. Bilateral palsy of the abducens nerve (as seen in Moebius Syndrome) results in lack of eye movement in the horizontal plane, ie. inability to move the eyes laterally. This can be observed by testing lateral tracking of eye movement (keeping the head still) and also when the individual reads text, it is apparent that they have to move their head laterally.
Mechanism of Injury / Pathological Process
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The etiology of Moebius syndrome is not known, but it appears to be multifactorial. There has been evidence of a genetic component related to various loci on chromosomes 3, 10, and 13[2].
The close relationship embryologically between the motor nuclei of the sixth and seventh cranial nerves explains the pathology of this rare condition[3].
Clinical Presentation[edit | edit source]
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Diagnostic Procedures[edit | edit source]
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Outcome Measures[edit | edit source]
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Management / Interventions
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Differential Diagnosis
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Key Evidence[edit | edit source]
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Resources
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Case Studies[edit | edit source]
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Recent Related Research (from Pubmed)[edit | edit source]
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References[edit | edit source]
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- ↑ Mobius PJ. Uber angeboren doppelseitige abducens-facialis-lahmung. Munchen Medizinische Wochenschrift. 1888;35:91-4.
- ↑ Uzumcu A, Karaman B, Toksoy G, Uyguner ZO, Candan S, Eris H. Molecular genetic screening of MBS1 locus on chromosome 13 for microdeletions and exclusion of FGF9, GSH1 and CDX2 as causative genes in patients with Moebius syndrome. Eur J Med Genet. Sep-Oct 2009;52(5):315-20
- ↑ May M, Schaitkin BM. Facial Nerve Disorders in Newborns and Children. In: The Facial Nerve. 2nd ed. New York, NY: Thieme New York; 2000:339-65
