Description : Duane retraction syndrome (DURS) is a congenital disorder characterized by restricted
horizontal eye movement with globe retraction and palpebral fissure narrowing on attempted
adduction. DURS is observed in approximately 0.1% of the general population, accounts
for 1 to 5% of all strabismus, and if untreated in childhood can result in loss of
binocular vision and amblyopia. Postmortem examinations of individuals with sporadic
DURS have shown absence of the abducens motor neurons and abducens cranial nerve on
the affected side(s), and aberrant innervation of the lateral rectus by axons of the
oculomotor nerve that normally innervate the medial rectus muscle. Most patients are
affected unilaterally and have no family history of the disorder (summary by Miyake
et al., 2010). For a discussion of genetic heterogeneity of Duane retraction syndrome,
see DURS1 (126800).;
Inheritance : Autosomal dominant;
Molecular basis : Caused by mutation in chimerin 1 gene (CHN1, 118423.0001);