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

PURPOSE: 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.

METHODS: 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.

RESULTS: 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.

CONCLUSION: Electroretinography may be valuable in determining optimal timing for therapeutic intervention and response before loss of recordable retinal function in CAPN5 vitreoretinopathy.