PROGRESSION OF SCOTOPIC SINGLE-FLASH ELECTRORETINOGRAPHY IN THE STAGES OF CAPN5 VITREORETINOPATHY.

Abstract

To characterize the changes found in the electroretinography (ERG) recordings of patients with autosomal dominant neovascular inflammatory vitreoretinopathy and correlate with clinical stages of the disease. Retrospective chart review. Bright- and dim-flash full-field scotopic, photopic, and 30-Hz flicker ERGs were obtained according to international standards. The scotopic ERGs were further processed to analyze the oscillatory potential. The patient described in the case report underwent full ERG testing; five patients composed the archival case series data and included scotopic ERG recordings. Stage I autosomal dominant neovascular inflammatory vitreoretinopathy is characterized by a decrease in the b-wave amplitude on scotopic flash ERG and the disappearance of late OPs; however, the a-wave amplitude is normal. In Stage II, attenuation of early OPs and the c-wave are observed in scotopic ERG recordings, but both a- and b-wave amplitudes are unchanged. For patients in Stage III, there is a continued decline of both a- and b-wave amplitudes in scotopic ERG recordings. There was a loss of recordable scotopic ERG response in patients with Stage IV disease. Electroretinography may be valuable in determining optimal timing for therapeutic intervention and response before loss of recordable retinal function in CAPN5 vitreoretinopathy.