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Most patients are asymptomatic, and evidence suggest that PPCRA is slowly progressing. Unless macular involvement is present, the majority of patients usually retain a normal visual function. Our ability to diagnose PPCRA has recently improved thanks to multimodal imaging, especially with the advent of ultra-widefield (UWF) imaging. Blood tests and functional and genetic testing can help with the correct differential diagnosis of pseudo-PPCRA or other disorders with similar characteristics. Although the cause of PPCRA is unknown, it is possible that it has a genetic basis. In this review we provide a summary of the multimodal imaging characteristics of PPCRA, and discuss its possible pathogenesis, based on the genes that have been associated with this disease.<\/jats:p>","DOI":"10.1177\/11206721231199118","type":"journal-article","created":{"date-parts":[[2023,9,6]],"date-time":"2023-09-06T02:15:56Z","timestamp":1693966556000},"page":"941-951","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":13,"title":["Pigmented paravenous chorioretinal atrophy: Updated scenario"],"prefix":"10.1177","volume":"34","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9630-0830","authenticated-orcid":false,"given":"Alessio","family":"Antropoli","sequence":"first","affiliation":[{"name":"Department of Ophthalmology, IRCCS San Raffaele Scientific Institute, Milan, 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