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Duane syndrome

Abstract

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NORD is very grateful to N.J. Gutowski, MD, Consultant Neurologist and Senior Lecturer, Royal Devon and Exeter Hospital and Peninsula Medical School, UK, for assistance in the preparation of this report.

Synonyms of Duane syndrome

  • DR syndrome
  • Duane radial ray syndrome (DRRS)
  • Duane's retraction syndrome
  • eye retraction syndrome
  • retraction syndrome
  • Stilling-Turk-Duane syndrome

Disorder Subdivisions

  • Duane syndrome Type 2A, 2B, 2C
  • Duane syndrome Type 3A, 3B, 3C
  • Duane syndrome Type IA, 1B, 1C

General Discussion

Duane syndrome (DS) is an eye movement disorder present at birth (congenital) characterized by horizontal eye movement limitation [a limited ability to move the eye inward toward the nose (adduction), outward toward the ear (abduction), or in both directions]. In addition, when the affected eye(s) moves inward toward the nose, the eyeball retracts (pulls in) and the eye opening (palpebral fissure) narrows. In some cases, when the eye attempts to look inward, it moves upward (upshoot) or downward (downshoot).

Duane syndrome falls under the larger heading of strabismus (misalignment of the eyes) under the subclassification of incomitant strabismus (misalignment of the eyes that varies with gaze directions) and subheading of what was previously termed extraocular fibrosis syndromes (conditions associated with fibrosis of the muscles that move the eyes), now termed Congenital Cranial Dysinnervation Disorders (CCDDs). The CCDDs are a group of congenital neuromuscular diseases resulting from developmental errors in innervation, the abnormalities involve one or more cranial nerves/nuclei with absence of normal innervation and/or secondary aberrant innervation. The group includes Duane syndrome, congenital fibrosis of the extraocular muscles (CFEOM), congenital ptosis, Marcus Gunn Jaw winking, Möbius syndrome, Crocodile tears, horizontal gaze palsy and congenital facial palsy, but this is not an exhaustive list.

Duane syndrome has been subdivided clinically into three types: Type 1, Type 2, and Type 3.

Organizations related to Duane syndrome

Further information can be found at the reviewer's web page: http://www.rdehospital.nhs.uk/prof/molecular_genetics/default.html

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