PURPOSE: To present electroretinographic (ERG) findings in a patient with retinal dysfunction with supernormal scotopic ERG, and to analyze rod and cone PIII components and rod inner nuclear layer (derived PII) responses. PATIENT: A Japanese 11-year-old girl complained of poor visual acuity. There was no parental consanguinity in her family. The corrected visual acuity was 0.7 in both eyes. No abnormal finding was observed in both fundi. METHODS: The patient underwent full-field ERGs. Rod and cone a-waves were analyzed using photoreceptor models. The derived PII responses were analyzed using a technique described by Hood and Birch. RESULTS: In the photopic ERG, responses to single flash and 30-Hz flicker were attenuated. In the scotopic ERG, b-wave was supernormal in amplitude in response to intense flashes, but smaller than normal and markedly delayed over a lower range of flash intensities. By the PIII analysis, phototransductions (values of S) of both rod and cone were remarkably decreased. The derived PII responses for this patient were larger than the responses for normal subjects, and the onset of the PII responses in this patient are significantly delayed compared to those in normal subjects. CONCLUSIONS: The ophthalmological findings in this patient are consistent with previous publications of this disease. Although it has been reported that the sites of disease action were beyond the outer segment (values of S were within the normal range), our results suggest that photoreceptors could be involved in sites of disease action in at least some patients with this disease.
PURPOSE: To present electroretinographic (ERG) findings in a patient with retinal dysfunction with supernormal scotopic ERG, and to analyze rod and cone PIII components and rod inner nuclear layer (derived PII) responses. PATIENT: A Japanese 11-year-old girl complained of poor visual acuity. There was no parental consanguinity in her family. The corrected visual acuity was 0.7 in both eyes. No abnormal finding was observed in both fundi. METHODS: The patient underwent full-field ERGs. Rod and cone a-waves were analyzed using photoreceptor models. The derived PII responses were analyzed using a technique described by Hood and Birch. RESULTS: In the photopic ERG, responses to single flash and 30-Hz flicker were attenuated. In the scotopic ERG, b-wave was supernormal in amplitude in response to intense flashes, but smaller than normal and markedly delayed over a lower range of flash intensities. By the PIII analysis, phototransductions (values of S) of both rod and cone were remarkably decreased. The derived PII responses for this patient were larger than the responses for normal subjects, and the onset of the PII responses in this patient are significantly delayed compared to those in normal subjects. CONCLUSIONS: The ophthalmological findings in this patient are consistent with previous publications of this disease. Although it has been reported that the sites of disease action were beyond the outer segment (values of S were within the normal range), our results suggest that photoreceptors could be involved in sites of disease action in at least some patients with this disease.
Authors: Frederick T Collison; Jason C Park; Gerald A Fishman; Edwin M Stone; J Jason McAnany Journal: Doc Ophthalmol Date: 2019-03-29 Impact factor: 2.379