Stage 1 Extensive Macular Atrophy with Pseudodrusen-Like Appearance Complicated by Multiple Evanescent White Dot Syndrome-Like Reaction

Purpose:
To describe a case of early-stage Extensive Macular Atrophy with Pseudodrusen-like appearance (EMAP) presenting with acute monocular loss and atypical retinal lesions suggestive of posterior uveitis.

Methods:
Case report with longitudinal follow-up including visual field testing and multimodal imaging.

Results:
A 53-year-old woman presented with sudden vision loss in her right eye (RE). Best corrected visual acuity (BCVA) was 20/400 in RE and unaffected in left eye (LE). Fundoscopy revealed bilateral pseudodrusen and peripheral paving-stone degeneration, consistent with stage 1 EMAP. Autofluorescence showed a hyper-autofluorescent reticular pattern in RE, colocalizing with pseudodrusen. Optical coherence tomography (OCT) demonstrated retinal pigment epithelium (RPE) thickening, RPE-Bruch’s membrane separation, and atypical feathery lesions in the RE, along with ellipsoid (EZ) loss. In the LE, only pseudodrusen and RPE-Bruch’s membrane separation were observed. Oral prednisone (30 mg, tapered) was initiated. At 24 weeks, the EZ had recovered, the feathery lesions resolved, and the RPE appeared normal, with a reduced hyper-autofluorescent pattern in the RE. At 30 weeks, the patient returned with recurrent vision loss in her RE. OCT revealed EZ loss without further lesions. After treatment with intravenous methylprednisolone and oral prednisone (tapered) plus methotrexate (15 mg/week), BCVA improved to 20/32, and EZ fully recovered.

Discussion:
We describe a case of stage 1 EMAP presenting with symptoms and lesions suggestive of concomitant Multiple Evanescent White Dot Syndrome (MEWDS)-like reaction. After treatment, resolution of inflammatory features and a partial improvement of the RPE abnormalities were observed.