Stilling-Türk-Duane syndrome (Siegmund Türk)
A congenital syndrome of ocular and systemic abnormalities with fibrosis of the external rectus. On attempted adduction of the affected eye, there is contraction of the globe and narrowing of the palpebral fissure, because musculus rectus lateralis is replaced by an inelastic string.
Prevalent in females, often familial, resembling sixth nerve palsy. The lateral rectus muscle is usually more affected than the medial rectus muscle. Unilateral involvement is most common, the left eye being the affected one in almost 80 % of cases. Radiological findings are similar to those seen in the Holt-Oram syndrome and may include triphalangeal or fingerlike thumbs, extra carpal bones, and thumb hypoplasia. Frequently associated is Klippel-Feil’s syndrome and various malformations of face, ears, and teeth. Electromyographic investigations suggest it may be partly a disorder of innervation. Inheritance is probably autosomal dominant.
There is some disagreement as to whether Stilling's, Duane's and Türk's are the same entity. Some authors define Türk's syndrome as an incomplete Duane's syndrome. A partial manifestation of the condition has been entered as Türk's syndrome.
The disturbance was probably first described by Williams in 1875. Jakob Stilling reported an initial case in 1887, and Türk described several cases in 1896. Duane in 1905 described 14 cases and collected 40 additional ones from the literature. The hereditary nature of the disorder was established in 1910 when Cooper reported the transmission through four generations of a kindred. Since that time there have been numerous reports in which the title «Duane reaction syndrome» has been employed, including a review of 101 affected persons by Isenberg and Urist in 1977.
When associated with cervical spine and radial ray abnormalities and deafness, this condition is known as the Okihiro syndrome. See under M. M. Okihiro, Japanese physician.
- J. Stilling:
Untersuchungen über die Entstehung der Kurzsichtigkeit.
Wiesbaden, Bergmann, 1887: 13.
- S. Türk:
Über Retraktionsbewegungen der Augen.
Deutsche medicinische Wochenschrift, Berlin, 1896, 22: 199.
- A. Duane:
Congenital deficiency of abduction, associated with impairment of adduction, retraction movements, contraction of palpebral fissure and oblique movements of the eye.
Archives of Ophthalmology, Chicago, 1905, 34: 133-159.