Duane retraction syndrome

Definition:

Duane retraction syndrome (DRS) is a congenital disorder of eye movement that occurs in approximately 1 in 50 patients with strabismus. This condition prevents horizontal eye movement, with absence of outward movement and deficiency of inward movement. Normal ocular motility depends on precise relations between cranial motor neurons and their target, extraocular muscles. In DRS parients, absence of the abducens motor neurons and aberrant innervations of extraocular muscles by third cranial nerve have been reported.

References:

[1]. Demet Yüksel, et al. Review of the major findings about Duane retraction syndrome (DRS) leading to an updated form of classification. Vision Res. 2010 Nov 23;50(23):2334-47. [Content Brief]

[2]. Jong G Park, et al. Loss of MAFB Function in Humans and Mice Causes Duane Syndrome, Aberrant Extraocular Muscle Innervation, and Inner-Ear Defects. Am J Hum Genet. 2016 Jun 2;98(6):1220-1227. [Content Brief]

[3]. Noriko Miyake, et al. Human CHN1 mutations hyperactivate alpha2-chimaerin and cause Duane's retraction syndrome. Science. 2008 Aug 8;321(5890):839-43. [Content Brief]

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