Anatomical and Functional Characteritic in Atrophic Maculopathy Aociated With pinocerebellar Ataxia Type 7

Purpoe To ae the anatomic and functional characteritic in a Korean family with pinocerebellar ataxia type 7-aociated atrophic maculopathy. Deign Obervational cae report. Method Three patient in a family underwent funducopic examination, fluorecein angiogram, full-field electroretinogram (ERG), electro-oculogram, and optical coherence tomography (OCT). Macular function were aeed uing multifocal ERG and canning laer ophthalmocope (LO) microperimetry. Reult Both eye howed bull’ eye configuration and cone-rod dytrophy phenotype. OCT revealed that retinal thinning extended outide the viibly atrophic leion. Multifocal ERG demontrated that amplitude from the ix ring egment were reduced in a centrifugal pattern. LO microperimetry howed that fixation were untable and hifted uperior to the atrophic leion. The area of functional deficit were greater than thoe of anatomic deficit. Concluion Macular function were everely depreed in atrophic maculopathy aociated with pinocerebellar ataxia type 7 in both the viibly atrophic area and the area without viible atrophy in a centrifugal pattern.