Abstract
The flash and pattern electroretinogram were investigated in a group of families with rare forms of inherited macular dystrophy, which included Sorsby's fundus dystrophy, X-linked retinoschisis and macular dystrophy of uncertain classification and variable expression. Flash electroretinograms, under both photopic and scotopic conditions, were attenuated in both Sorsby's fundus dystrophy and X-linked retinoschisis—with some effect on implicit time being noted in the latter condition—but in the unknown group the effect was less demonstrable, only 50% having attenuated flash electroretinograms. Pattern electroretinograms were reduced in all three conditions and in almost all cases. The study demonstrates that some so-called macular dystrophies also have widespread abnormalities affecting the peripheral retina. These findings may contribute to a better understanding of the underlying pathophysiologic mechanisms in these rare forms of retinal dysfunction.
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Abbreviations
- SFD:
-
Sorsby's fundus dystrophy
- XLJR:
-
X-linked juvenile retinoschisis
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Clarke, M.P., Mitchell, K.W. & McDonnell, S. Electroretinographic findings in macular dystrophy. Doc Ophthalmol 92, 325–339 (1996). https://doi.org/10.1007/BF02584086
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DOI: https://doi.org/10.1007/BF02584086