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Abnormal pattern electroretinograms with macular cherry-red spots: evidence for selective ganglion cell damage
Current Eye Research
|January 1, 1981
Summary
This study investigated electroretinograms (ERG) in a patient with cherry-red spot-myoclonus syndrome. Findings suggest pattern ERG originates in ganglion cells, while flash ERG is generated in the distal retina.
Area of Science:
- Ophthalmology
- Neuroscience
- Genetics
Background:
- Macular cherry-red spot signifies abnormal metabolic product storage in retinal ganglion cells.
- Cherry-red spot-myoclonus syndrome is a sialidosis caused by neuraminidase deficiency.
Purpose of the Study:
- To investigate the electroretinograms (ERG) and visual evoked potentials (VEP) in a patient with cherry-red spot-myoclonus syndrome.
- To determine the retinal origin of pattern and flash ERGs.
Main Methods:
- Studied flash and pattern electroretinograms (ERG) and visual evoked potentials (VEP).
- Analyzed electrophysiological responses in a patient with sialidosis due to neuraminidase deficiency.
Main Results:
- Flash ERG was normal.
- Pattern ERG showed an abnormal waveform with an attenuated p-q wave component.
- Flash VEPs were normal, but pattern VEPs were delayed.
Conclusions:
- This case provides human evidence that pattern ERG is organized in retinal ganglion cells.
- Confirms flash ERG is generated in the distal retina.
- Highlights the utility of pattern ERG in diagnosing conditions affecting ganglion cells.