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Cone dystrophies with negative photopic electroretinogram
1Freie Universität Berlin, Klinikum Steglitz, Berlin, Germany.
The British Journal of Ophthalmology
|July 1, 1993
Summary
Negative electroretinograms, typically indicating inner retinal issues, were observed in some progressive cone dystrophy patients. This finding suggests cone dystrophy may involve inner retinal defects, potentially inherited separately.
Area of Science:
- Ophthalmology
- Genetics
Background:
- Scotopic electroretinograms (ERG) with larger a-wave than b-wave amplitudes are termed 'negative'.
- Negative ERGs are unusual in progressive cone dystrophy.
Observation:
- Six male patients with negative photopic electroretinograms were studied.
- Three had progressive cone dystrophy, one was a sibling of a cone dystrophy patient.
- Comparison was made with patients having congenital stationary night blindness and X-linked retinoschisis.
Findings:
- Differential diagnosis of cone dystrophies from other conditions was possible using funduscopy, adaptometry, and progression evaluation, but not solely ERG.
- Negative ERGs in cone dystrophy suggest potential inner retinal defects.
- Waveform variations may indicate diverse inner retinal abnormalities.
Implications:
- Inner retinal defects may be a feature of progressive cone dystrophies.
- Cone dystrophy and inner retinal defects might be inherited independently, as suggested by findings in two brothers.