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Duane syndrome (DS) is a rare, congenital eye movement disorder most commonly characterized by the inability of the eye to turn out. The syndrome was first described by Jakob Stilling (1887) and Siegmund Türk (1896), and subsequently named for Alexander Duane who discussed the disorder in more detail in 1905[1]
Other names for this condition include: Duane’s Retraction Syndrome (or DR syndrome), Eye Retraction Syndrome, Retraction Syndrome, Congenital retraction syndrome and Stilling-Turk-Duane Syndrome.[2]
As described by Duane, the characteristic features of the syndrome are:
In addition to the above, other characteristics may or may not be present, and these include:
In about 80 per cent of cases, only one eye is affected, most often the left. However, in some cases, both eyes are affected, with one eye usually more affected than the other.
DS is a miswiring of the eye muscles, causing some eye muscles to contract when they shouldn’t and other eye muscles not to contract when they should.[2]
Alexandrakis G & Saunders RA [4] state that:
This view is supported by the earlier work of Hotchkiss et al[5] who reported on the autopsy findings of two patients with Duanes syndrome. In both cases the sixth cranial nerve nucleus was absent, as was the sixth nerve, and the lateral rectus muscle was innervated by the inferior division of the third or oculomotor cranial nerve. This misdirection of nerve fibres results in opposing muscles being innervated by the same nerve. Thus, on attempted abduction, stimulation of the lateral rectus via the oculomotor nerve will be accompanied by stimulation of the opposing medial rectus via the same nerve; a muscle which works to adduct the eye. Thus, co-contraction of the muscles takes place, limiting the amount of movement achievable and also resulting in retraction of the eye into the socket.
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