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Ankara Etlik City Hospital, Ophthalmology Department, Ankara, Türkiye DOI : 10.37845/ret.vit.2024.33.46 Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a rare disease that was first described in 1978. With the development of optical coherence tomography (OCT), ophthalmological findings, especially peripapillary retinal nerve fiber thickening, have been identified. In this article, we will present a patient diagnosed with ARSACS who has low vision and foveal hypoplasia, which is detected by OCT and OCT-Angiography. Based on this case, we aimed to evaluate ophthalmological findings of ARSACS disease, which is one of the rare causes of foveal hypoplasia. Keywords : ARSACS, Autosomal recessive spastic ataxia of Charlevoix-Saguenay, Foveal hypoplasia, OCT, OCT-Angiography