ArticleGraefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie2026
Clinical characteristics and long-term follow-up of extensive macular atrophy with pseudodrusen-like appearance in Japanese patients.
Article in Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
purposeTo describe the clinical characteristics and long-term follow-up findings of extensive macular atrophy with pseudodrusen-like appearance (EMAP) in a Japanese cohort.
methodsThis retrospective observational study analyzed Japanese patients diagnosed with EMAP who underwent genetic tests, which did not detect known pathogenic variants of inherited retinal dystrophies or age-related macular degeneration. Patients underwent comprehensive ophthalmic evaluations, including multimodal imaging (fundus autofluorescence and optical coherence tomography) and full-field electroretinography.
resultsThis study included eight Japanese patients with EMAP, encompassing five males and three females. The mean follow-up period was 102.5 (range, 25-180) months. The mean best-corrected visual acuity declined from 0.18 to 1.08 logarithm of the minimum angle of resolution (right eye) over the follow-up period. Macular atrophy and peripheral paving-stone-like degeneration were present in 75% and 87.5% of patients, respectively. Rod responses were reduced in 62.5% of patients, and delayed flicker full-field electroretinography implicit times were observed in 62.5%. Longitudinal imaging suggested phenotypic changes in a limited number of cases including progression from a predominantly central pattern to confluent involvement in one patient.
conclusionJapanese patients with EMAP exhibited structural and functional abnormalities during long-term follow-up that were consistent with broader retinal involvement extending beyond the macula.
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