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PURPOSE: To assess the anatomic and functional characteristics in a Korean family with spinocerebellar ataxia type 7-associated atrophic maculopathy. DESIGN: Observational case report. METHODS: Three patients in a family underwent funduscopic examination, fluorescein angiogram, full-field electroretinogram (ERG), electro-oculogram, and optical coherence tomography (OCT). Macular functions were assessed using multifocal ERG and scanning laser ophthalmoscope (SLO) microperimetry. RESULTS: Both eyes showed bull’s eye configuration and cone-rod dystrophy phenotype. OCT revealed that retinal thinning extended outside the visibly atrophic lesions. Multifocal ERGdemonstrated that amplitudes from the six ring segments were reduced in a centrifugal pattern. SLO microperimetry showed that fixations were unstable and shifted superior to the atrophic lesions. The areas of functional deficits were greater than those of anatomic deficits. CONCLUSIONS: Macular functions were severely depressed in atrophic maculopathy associated with spinocerebellar ataxia type 7 in both the visibly atrophic areas and the areas without visible atrophy in a centrifugal pattern.
METHODS: Three patients in a family underwent funduscopic examination, fluorescein angiogram, full-field electroretinogram (ERG) OCT revealed that retinal thinning extended, OCT revealed that retinal thinning extended outside the visibly atrophic lesions. Multifocal ERGdemonstrated that amplitudes from the six ring segments were reduced in a centrifugal pattern. SLO microperimetry showed that fixations were unstable and shifted superior to the atrophic lesions. The areas of functional deficits were greater than those of anatomic deficits. CONCLUSIONS: Macular functions were severely depressed in atr ophic maculopathy associated with spinocerebellar ataxia type 7 in both the visibly atrophic areas and the areas without visible atrophy in a centrifugal pattern.