Electrophysiological findings in extensive macular atrophy with pseudodrusen

Doc Ophthalmol. 2023 Oct;147(2):121-130. doi: 10.1007/s10633-023-09941-y. Epub 2023 Jul 1.


Purpose: To present electroretinogram findings in extensive macular atrophy with pseudodrusen (EMAP) and describe associated systemic factors.

Design: Retrospective case series.

Methods: Data on medical history, visual symptoms, multimodal imaging findings, and visual field were collected from the medical records of patients with extensive macular atrophy with pseudodrusen who attended a visual electrophysiology laboratory. Electrophysiological tests, including full-field electroretinogram, multifocal electroretinogram and photopic negative response, were performed.

Results: Eighteen patients (10 [56%] females, age 49-66 years) were included. Of these, 17 (94%) had a history of rheumatic fever in childhood and/or adolescence, 7 (39%) had cardiovascular disease, 4 (22%) had autoimmune disease, and 10 (56%) had inflammatory conditions. The primary visual complaints were nyctalopia (95%), followed by visual field loss (67%) and dyschromatopsia (67%). The key retinal findings included retinal pigmented epithelium atrophy in the macular region and subretinal drusenoid deposits. Regarding electrophysiological results, 100% of patients had abnormalities on multifocal electroretinogram, 94% displayed alterations in photopic negative response, and 78% showed changes in the full-field electroretinogram.

Conclusions: In this cohort, electrophysiologic evaluation demonstrated diffuse retinal dysfunction affecting all layers of the retina in patients with EMAP. The disease is associated with immune-mediated systemic conditions, chiefly rheumatic fever.

Keywords: Autoimmune retinopathy; Electroretinogram; Extensive macular atrophy with pseudodrusen; Photopic negative response; Rheumatic fever.

MeSH terms

  • Aged
  • Atrophy / complications
  • Electroretinography
  • Female
  • Humans
  • Macular Degeneration* / complications
  • Macular Degeneration* / diagnosis
  • Male
  • Middle Aged
  • Retinal Drusen* / complications
  • Retinal Drusen* / diagnosis
  • Retrospective Studies
  • Rheumatic Fever* / complications
  • Tomography, Optical Coherence / methods