Abnormal pattern electroretinograms with macular cherry-red spots: evidence for selective ganglion cell damage
- 1 January 1981
- journal article
- research article
- Published by Taylor & Francis in Current Eye Research
- Vol. 1 (6), 367-372
- https://doi.org/10.3109/02713688108998364
Abstract
A macular cherry red spot is a strikingly visible abnormality indicating storage of an abnormal metabolic product by the retinal ganglion cells. The flash and pattern electroretinograms (ERG) and visual evoked potentials (VEP) of a patient with the cherry-red spot-myoclonus syndrome, a sialidosis due to neuraminidase deficiency, were studied. The flash ERG was normal but the pattern ERG had an abnormal waveform with a significantly attenuated p-q wave component. Corresponding flash VEPs were normal, but pattern VEPs were delayed. This case documents human evidence that the pattern ERG is organised in the ganglion cells whereas the flash ERG, as is well known, is generated in the distal retina.This publication has 9 references indexed in Scilit:
- Electroretinographic Responses to Alternating Gratings Before and After Section of the Optic NerveScience, 1981
- SIALIDOSIS - CHERRY-RED SPOT-MYOCLONUS SYNDROME1980
- Sialidosis type 1: cherry red spot-myoclonus syndrome with sialidase deficiency and altered electrophoretic mobilities of some enzymes known to be glycoproteins. 1. Clinical findings.Journal of Neurology, Neurosurgery & Psychiatry, 1979
- Cherry-Red Spot-Myoclonus SyndromeOphthalmology, 1979
- SIALIDOSIS - REVIEW OF HUMAN NEURAMINIDASE DEFICIENCY1979
- SIMULTANEOUS ELECTRORETINOGRAMS AND VISUALLY EVOKED-POTENTIALS FROM ADULT AMBLYOPES IN RESPONSE TO A PATTERN STIMULUS1979
- GOLD FOIL ELECTRODE - EXTENDING THE HORIZONS FOR CLINICAL ELECTRORETINOGRAPHY1979
- Sialidase (α‐N‐acetyl neuraminidase) deficiency: the enzyme defect in an adult with macular cherry‐red spots and myoclonus without dementiaClinical Genetics, 1978
- The cherry‐red spot‐myoclonus syndromeAnnals of Neurology, 1978