Grayson-wilbrandt syndrome (reis-buecklers syndrome; corneal dystrophy of reis-buecklers)

General: Onset at end of first decade; infrequent episodes of eye redness and pain; autos 131b19b omal dominant trait; electron microscopy reveals peculiar curly material in the subepithelial fibrous tissue that parallels the distribution of attachment proteins.

Ocular: Corneal changes variable from a mottled scarring to gray macular opacities of the anterior limiting membrane of the cornea; strabismus.

Clinical: None.

Buecklers M. Ueber Eine Weltere Familiaere Hornhautdystrophie (Reis). Klin Monatsbl Augenheilkd 1949; 144: 386-397.

Grayson M, et al. Dystrophy of the anterior limiting membrane of the cornea (Reis-Buecklers type). Am J Ophthalmol 1966; 61:345-349.

Lohse E, et al. Reis-Bucklers corneal dystrophy: immunofluorescent and electron microscopic studies. Cornea 1989; 8:200.

McKusick VA. Mendelian Inheritance in Man; A Catalog of Human Genes and Genetic Disorders, 12th ed. Baltimore: The Johns Hopkins University Press, 1998.

Online Mendelian Inheritance in Man, OMIM. McKusick-Nathans Institute for Genetic Medicine, Johns Hopkins University and National Center for Biotechnology Information, National Library of Medicine, February 12, 2007. World Wide Web URL: https://www.ncbi.nlm.nih.gov/omim/.

Pavan-Langston D. Reis-Buecklers dystrophy. In: Pavan-Langston D, ed. Manual of ocular diagnosis and therapy, 4th ed. Boston: Little, Brown and Company, 1995:106.