Duane Syndrome Workup

  • Author: Arun Verma, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Nov 17, 2011
 

Other Tests

On testing of brainstem auditory evoked responses, findings are occasionally abnormal, suggesting widespread neurologic abnormalities.

EMG findings show absent lateral rectus firing on attempted abduction and firing of both horizontal recti on attempted adduction.

The affected lateral rectus muscle behaves like a muscle double innervated by a weak abducens nerve and a stronger oculomotor nerve branch or like a muscle only innervated by an oculomotor nerve branch. This finding demonstrates that, though a muscle is present, it has a functioning yet an abnormal nerve supply.

Likewise, simultaneous EMG recording of the lateral and medial recti muscles revealed a paradoxical innervation of the lateral rectus muscle to be the pathogenetic principle of all forms of DS.

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Histologic Findings

Early histopathologic and surgical studies led to the conclusion that DS was a local, purely myogenic phenomenon. Therefore, the generally accepted concept was that the cause of abduction deficiency was fibrosis of the lateral rectus muscle and that the limitation of adduction was caused by a false posterior insertion of the medial rectus muscle or by adhesions between the medial rectus muscle and the orbital wall.

N. R. Miller and coauthors from the John Hopkins University performed complete intracranial and orbital pathologic examination of 2 cases of DS in which the clinical findings were well documented.[20] The first case was bilateral DS type 3. Postmortem examination of the brainstem and the posterior parts of the orbits revealed bilateral absence of the abducens nuclei and the abducens nerves. No large motor neurons were identified at levels the abducens nuclei normally occupy. No intra-axial fibers referable to the sixth cranial nerve could be identified with the brainstem. Both oculomotor nuclei and nerves were normal at the level of the ciliary ganglion, the inferior division of the oculomotor nerve divided into several branches penetrating the inferior medial aspect of the lateral rectus muscle.

The other case showed only 2 thin atrophic fibers of 0.1-mm diameter, both of which had separate exits (1 mm distance between them) from the dura. The further course of these fibers could not be examined.

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Contributor Information and Disclosures
Author

Arun Verma, MD  Senior Consultant, Department of Ophthalmology, Dr Daljit Singh Eye Hospital, India

Disclosure: Nothing to disclose.

Specialty Editor Board

Gerhard W Cibis, MD  Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas School of Medicine

Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Ophthalmological Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

J James Rowsey, MD  Former Director of Corneal Services, St Luke's Cataract and Laser Institute

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association

Disclosure: Nothing to disclose.

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

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Duane syndrome.
Duane syndrome.
An individual diagnosed with Duane syndrome in the left eye. In this image, the affected person is attempting to look far right. Notice the afflicted left eye faces straight and up (an "upshoot"), rather than following the right eye to the right.
Table. Types of DS
CategoryDescription
Type
1 (70%-80%)Inability to abduct



Normal or minimal defect in adduction



Esotropia with head straight



A or V pattern



Usually updrift or a downdrift of affected eye on adduction or attempted abduction



Globe retraction and palpebral-fissure narrowing on adduction



Usual face turn to affected side



Normal stereo possible



2 (about 7%)Inability to adduct



Normal or minimal defect in abduction



Exotropia of the affected eye



Marked upshoot



Globe retraction and palpebral-fissure narrowing on adduction



Stereo normal or suppressed



Face turn to normal side



3 (about 15%)Inability ability to abduct and adduct



Globe retraction and palpebral-fissure narrowing on attempted adduction



Possible upshoot and downshoot on adduction



Straight or nearly straight head position



Usually, normal stereo



4Not usual DS or DS-type appearance



Large-angle exotropia



Face turn to uninvolved side



Limited adduction



Simultaneous abduction when looking toward uninvolved side



Usually suppresses 1 eye



Subtype
AAffected eye turned inward toward the nose (esotropia)
BAffected eye turned outward toward the ear (exotropia)
CEyes in a straight primary position
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