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Electrophysiological study on cone rod dystrophy of bull's eye macular lesion type

, : Electrophysiological study on cone rod dystrophy of bull's eye macular lesion type. Nihon University Journal of Medicine 28(3): 175-188

Nine cases of cone-rod dystrophy of bull's eye macular lesion type were examined electrophysiologically. 7 eyes of 7 normal cases were studied as a control by the same electrophysiological techniques. 1) Widespread cone-dysfunction could be detected from abnormalities of the photopic b wave, rapid off decay and 30 Hz flicker ERG. 2) The degree of cone-dysfunction which was evaluated from the abnormalities of the photopic b wave and 30 Hz flicker ERG was found not to correlate with the extent of the pathologic lesion as demonstrated by fluorescein angiography and ophthalmoscopy. 3) The results of color vision tests in 5 cases were compared with their spectral response curves, which were obtained from the amplitude of the rapid off decay of ERG stimulated by monochromatic light of various wavelengths. The total error scores of the color vision tests were found to correlate with the amplitude of the monochromatic ERG. 4) Even in cases which showed ophthalmoscopic abnormalities only in the macular area, an abnormal scotopic b wave was observed. These results indicate that rod dysfunction precedes discernible ophthalmoscopic abnormalities.

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