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Updated: Jan 31, 2026

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Late-Onset Retinal Degeneration.

Stephen H Tsang1,2, Tarun Sharma3

  • 1Jonas 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.

Advances in Experimental Medicine and Biology
|December 23, 2018
PubMed
Summary
This summary is machine-generated.

Late-onset retinal degeneration (LORD) typically causes night blindness in adults. Characteristic findings include RPE atrophy and iris abnormalities, impacting vision later in life.

Keywords:
Autosomal dominantLORDLate-onset retinal degeneration

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Area of Science:

  • Ophthalmology
  • Medical Genetics
  • Retinal Diseases

Background:

  • Late-onset retinal degeneration (LORD) is a group of inherited retinal diseases.
  • Patients often experience nyctalopia (night blindness) in their fifth or sixth decade.

Purpose of the Study:

  • To describe the clinical presentation and fundus findings in patients with LORD.
  • To highlight key ophthalmic features associated with this condition.

Main Methods:

  • Clinical examination of patients with LORD.
  • Ophthalmoscopy to assess fundus and anterior segment changes.

Main Results:

  • Fundus examination reveals yellowish-white, punctate deposits.
  • Progressive retinal pigment epithelium (RPE) atrophy in the mid-periphery and posterior pole.
  • Anterior segment findings include elongated zonules, central lens insertion, and iris atrophy with transillumination defects.

Conclusions:

  • LORD presents with characteristic fundus and anterior segment abnormalities.
  • The progressive nature of RPE atrophy and iris changes are key diagnostic features.