Other Tests

On testing of brainstem auditory evoked responses, findings of Duane syndrome (DS) occasionally are 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 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 Duane syndrome.

Histologic Findings

Early histopathologic and surgical studies led to the conclusion that Duane syndrome 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.

Miller and coauthors from the John Hopkins University performed complete intracranial and orbital pathologic examination of 2 cases of Duane syndrome in which the clinical findings were well documented.^[20]The first case was bilateral Duane syndrome 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.

Treatment & Management

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Media Gallery

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.

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Sections Duane Syndrome
Overview
Presentation
- History
- Physical
- Causes
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DDx
Workup
Treatment
Media Gallery
Tables
References

Category	Description
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
4	Not usual Duane syndrome or Duane syndrome-type appearance Large-angle exotropia Face turn to uninvolved side Limited adduction Simultaneous abduction when looking toward uninvolved side Usually suppresses 1 eye
Subtype
A	Affected eye turned inward toward the nose (esotropia)
B	Affected eye turned outward toward the ear (exotropia)
C	Eyes in a straight primary position

Duane Syndrome Workup

Other Tests

Histologic Findings

References

Tables

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