Visual Field Characteristics in East Asian Patients With Occult Macular Dystrophy (Miyake Disease): EAOMD Report No. 3open access
- Authors
- Ahn, Seong Joon; Yang, Lizhu; Tsunoda, Kazushige; Kondo, Mineo; Fujinami-Yokokawa, Yu; Nakamura, Natsuko; Iwata, Takeshi; Kim, Min Seok; Mun, Yongseok; Park, Jun Young; Joo, Kwangsic; Park, Kyu Hyung; Miyake, Yozo; Sui, Ruifang; Fujinami, Kaoru; Woo, Se Joon
- Issue Date
- Jan-2022
- Publisher
- ASSOC RESEARCH VISION OPHTHALMOLOGY INC
- Keywords
- Miyake disease; occult macular dystrophy; RP1L1; visual fields
- Citation
- Investigative Ophthalmology and Visual Science, v.63, no.1, pp.1 - 9
- Indexed
- SCIE
SCOPUS
- Journal Title
- Investigative Ophthalmology and Visual Science
- Volume
- 63
- Number
- 1
- Start Page
- 1
- End Page
- 9
- URI
- https://scholarworks.bwise.kr/hanyang/handle/2021.sw.hanyang/138438
- DOI
- 10.1167/iovs.63.1.12
- ISSN
- 0146-0404
- Abstract
- PURPOSE. The purpose of this study was to investigate the perimetric features and their associations with structural and functional features in patients with RP1L1-associated occult macular dystrophy (OMD; i.e. Miyake disease).
METHODS. In this international, multicenter, retrospective cohort study, 76 eyes of 38 patients from an East Asian cohort of patients with RP1L1-associated OMD were recruited. Visual field tests were performed using standard automated perimetry, and the patients were classified into three perimetric groups based on the visual field findings: central scotoma, other scotoma (e.g. paracentral scotoma), and no scotoma. The association of the structural and functional findings with the perimetric findings was evaluated.
RESULTS. Fifty-four eyes (71.1%) showed central scotoma, 14 (18.4%) had other scotomata, and 8 (10.5%) had no scotoma. Central scotoma was mostly noted in both eyes (96.3%) and within the central 10 degrees (90.7%). Among the three perimetric groups, there were significant differences in visual symptoms, best-corrected visual acuity (BCVA), and structural phenotypes (i.e. severity of photoreceptor changes). The central scotoma group showed worse BCVA often with severe structural abnormalities (96.3%) and a pathogenic variant of p.R45W (72.2%). The multifocal electroretinogram (mfERG) groups largely corresponded with the perimetric groups; however, 8 (10.5%) of 76 eyes showed mfERG abnormalities preceding typical central scotoma.
CONCLUSIONS. The patterns of scotoma with different clinical severity were first identified in occult macular dystrophy, and central scotoma, a severe pattern, was most frequently observed. These perimetric patterns were associated with the severity of BCVA, structural phenotypes, genotype, and objective functional characteristics which may precede in some cases.
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