Abstract
To examine the electroretinograms (ERGs) of the short-wavelength-sensitive (S-) and the mixed long- and middle-wavelength-sensitive (L,M-) cones, and the ON- and OFF-responses of the cone ERGs in three patients with X-linked juvenile retinoschisis (XLRS). Cone ERGs elicited by different color flashes and those elicited by long duration stimuli under Ganzfeld conditions were recorded from three patients with XLRS. The S-cone b-waves were undetectable to short-wavelength stimuli in all three XLRS patients, while the L,M-cone ERG b-waves were within the normal range. To long-duration white stimuli, the ON-response (b-wave) was reduced and delayed in all patients compared with that of the normal subjects, while the d-wave or OFF-response appeared normal in amplitude and implicit time. These results support the hypothesis that the normal S-cone ERG arises primarily from the ON-pathway of the cone ERGs and the hypothesis that ON-bipolar cells are predominant in the S-cone system.
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