Progressive Cone Dystrophy and Cone-Rod Dystrophy (XL, AD, and AR).

Stephen H Tsang, Tarun Sharma

Journal: Advances in experimental medicine and biology 2019;1085():53-60

PMID: 30578485

Abstract

A heterogenous group of diseases, progressive cone dystrophy usually begins in the mid-teenage years or later in life. The estimated prevalence is 1 in 30,000-40,000 individuals. Patients usually present with decreased central vision and a color vision deficit; the visual loss is progressive and often accompanied by day blindness (hemeralopia) and light intolerance (photophobia). Over time, affected individuals develop night blindness and loss of peripheral field. Visual acuity deteriorates to 20/200 or even counting fingers. There is some association between X-linked cone-rod dystrophy (CORD) and high myopia.

Address: 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.; Department of Ophthalmology, Columbia University, Edward S. Harkness Eye Institute, NewYork-Presbyterian Hospital, New York, NY, USA.; Department of Ophthalmology, Columbia University, Edward S. Harkness Eye Institute, NewYork-Presbyterian Hospital, New York, NY, USA. [email protected].

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