Late-Onset Retinal Degeneration

Stephen H Tsang; Tarun Sharma

doi:10.1007/978-3-319-95046-4_23

Late-Onset Retinal Degeneration

Adv Exp Med Biol. 2018:1085:115-116. doi: 10.1007/978-3-319-95046-4_23.

Authors

Stephen H Tsang^{1

2}, Tarun Sharma³

Affiliations

¹ Jonas Children's Vision Care, Bernard & Shirlee Brown Glaucoma Laboratory, Columbia Stem Cell Initiative-Departments of Ophthalmology, Biomedical Engineering, Pathology & Cell Biology, Institute of Human Nutrition, Vagelos College of Physicians and Surgeons, Columbia University, New York, NY, USA.
² Department of Ophthalmology, Columbia University, Edward S. Harkness Eye Institute, NewYork-Presbyterian Hospital, New York, NY, USA.
³ Department of Ophthalmology, Columbia University, Edward S. Harkness Eye Institute, NewYork-Presbyterian Hospital, New York, NY, USA. ts3118@cumc.columbia.edu.

PMID: 30578496
DOI: 10.1007/978-3-319-95046-4_23

Abstract

Patients with late-onset retinal degeneration (LORD) usually present with nyctalopia in the fifth or sixth decade of life. The fundus shows yellowish-white, punctate deposits, usually progressive and giving rise to scalloped areas of retinal pigment epithelium (RPE) atrophy in the mid-periphery and posterior pole (Fig. 23.1). The anterior segment shows elongated zonules with central lens insertion and transillumination defect in the iris, due to iris atrophy.

Keywords: Autosomal dominant; LORD; Late-onset retinal degeneration.

Publication types

Review

MeSH terms

Age of Onset
Humans
Retinal Degeneration / physiopathology*
Retinal Pigment Epithelium / pathology

Supplementary concepts