Abstract
Purpose
To investigate the center-periphery distribution of ON and OFF retinal responses in complete congenital stationary night blindness (cCSNB).
Methods
Photopic full-field flash ERGs (photopic ffERGs) and OPs (photopic ffOPs) and slow m-sequence (to enhance OP prominence) mfERGs (and filtered mfOPs) evoked by a 37 hexagon stimulus array were recorded from normal subjects and cCSNB patients. Discrete wavelet transform (DWT) analysis of photopic ffERGs and mfERGs was also performed in order to assess the contribution of the ON and OFF retinal pathways (i.e., OFF-to-ON ratio) in both cohorts.
Results
As expected, the photopic ffERG (and ffOPs) responses in cCSNB were devoid of the first two of the three OPs (i.e., OP2 and OP3 and OP4) normally seen on the ascending limb of the b-wave. A similar finding was also noted in the mfERGs (and mfOPs) of ring 4. In contrast, the mfERGs (and mfOPs) of ring 1 included all three OPs. DWT analysis revealed that while in normal subjects, the OFF-to-ON ratio of mfERGs slightly increased from rings 1 to 4 (from 0.61 ± 0.03 to 0.78 ± 0.04; p < 0.05; median: from 0.62 to 0.79; p < 0.05), in cCSNB this ratio increased significantly more [from 0.73 ± 0.13 (ring 1) to 1.18 ± 0.17 (ring 4); p < 0.05; median: 0.78 to 1.22; p < 0.05], hence from a normal ON-dominated ratio (central ring) to an OFF-dominated ratio (peripheral ring).
Conclusions
Our results show a clear discrepancy of ON and OFF mfOP components in cCSNB. Responses originating from the most central ring (i.e., ring 1) disclosed a near-normal electrophysiological contribution (as revealed with the presence of OP2, OP3 and OP4 as well as with the DWT OFF-to-ON ratio) of the retinal ON and OFF pathways in mfERG (and mfOPs) responses compared to responses from the more peripheral ring (and ffOP) which are devoid of the ON OPs (i.e., OP2 and OP3).
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Canadian Institutes of Health Research Grant # MOP126082.
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Authors Allison L. Dorfman, Mathieu Gauvin, Dylan Vatcher, John M. Little, Robert C. Polomeno and Pierre Lachapelle declare that they have no conflict of interest to disclose.
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This study was approved by the Institutional Ethics Review Board of the Montreal Children’s Hospital.
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All procedures performed fulfilled the principles of the Declaration of Helsinki, and all participants signed an informed consent.
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John M. Little died on June 10, 2018.
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Supplementary Fig. 1
Photopic ffERG (panel A) and mfERG (rings 4, 3, 2 and 1; panels B, C, D and E, respectively) waveforms obtained from all control subjects (blue tracings) and all cCSNB patients (red tracings). Major wave components are identified with corresponding letters (a, b, i and n-waves) or numbers (OP2, 3, 4). Response starts at time 0. Note in column A that the typical photopic ffERG waveform (i.e., square-wave-like a-wave, truncated b-wave, no oscillation on the ascending limb of the b-wave), which is pathognomonic for this condition, was seen in all our cCSNB patients (tracings 1 to 5). (JPEG 10811 kb)
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Dorfman, A.L., Gauvin, M., Vatcher, D. et al. Ring analysis of multifocal oscillatory potentials (mfOPs) in cCSNB suggests near-normal ON–OFF pathways at the fovea only. Doc Ophthalmol 141, 99–109 (2020). https://doi.org/10.1007/s10633-020-09755-2
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DOI: https://doi.org/10.1007/s10633-020-09755-2