TY - JOUR
T1 - Diagnostic clinical findings of a new syndrome with night blindness, maculopathy, and enchanced S cone sensitivity
AU - Marmor, M. F.
AU - Jacobson, S. G.
AU - Foerster, M. H.
AU - Kellner, U.
AU - Weleber, R. G.
N1 - Funding Information:
From the Department of Ophthalmology, Stanford University School of Medicine, Stanford, California (Dr. Marmor); Bascom Palmer Eye Institute, University of Miami School of Medicine, Miami, Florida (Dr. Jacobson); Universitatsklinikum Essen, West Germany (Drs. Foerster and Kellner); and Departments of Ophthalmology and Medical Genetics, Oregon Health Sciences University, Portland, Oregon (Dr. Weleber). This study was supported in part by National Eye Institute research grants EY01678 (Dr. Marmor) and EY05627 (Dr. Jacobson), an unrestricted grant from Research to Prevent Blindness, Inc. (Dr. Marmor), and the National Retinitis Pigmentosa Foundation, Inc., Baltimore, Maryland (Drs. Jacobson and Weleber).
PY - 1990
Y1 - 1990
N2 - We studied eight patients who had night blindness, maculopathy (often cystoid), degenerative changes in the region of the vascular arcades, relatively mild visual field loss, and an unusual but characteristic electroretinogram. The dark-adapted electroretinogram showed no response to low-intensity stimuli that normally activate the rods, but large, slow responses to high-intensity stimuli. These large, slow waveforms persisted without change under light adaptation, and showed a striking mismatch to photopically balanced short and long wavelength stimuli (with sensitivity much greater to short than long wave-lenghts). Since there is evidence from other studies that the electroretinogram and psychophysical responses represent hypersensitivity of short wavelength-sensitive (S or blue) cones, we propose that this disorder be called the enhanced S cone syndrome. There can be different degrees of severity in this syndrome, and progression appears to be slow.
AB - We studied eight patients who had night blindness, maculopathy (often cystoid), degenerative changes in the region of the vascular arcades, relatively mild visual field loss, and an unusual but characteristic electroretinogram. The dark-adapted electroretinogram showed no response to low-intensity stimuli that normally activate the rods, but large, slow responses to high-intensity stimuli. These large, slow waveforms persisted without change under light adaptation, and showed a striking mismatch to photopically balanced short and long wavelength stimuli (with sensitivity much greater to short than long wave-lenghts). Since there is evidence from other studies that the electroretinogram and psychophysical responses represent hypersensitivity of short wavelength-sensitive (S or blue) cones, we propose that this disorder be called the enhanced S cone syndrome. There can be different degrees of severity in this syndrome, and progression appears to be slow.
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U2 - 10.1016/S0002-9394(14)76980-6
DO - 10.1016/S0002-9394(14)76980-6
M3 - Article
C2 - 2378376
AN - SCOPUS:0025333641
SN - 0002-9394
VL - 110
SP - 124
EP - 134
JO - American journal of ophthalmology
JF - American journal of ophthalmology
IS - 2
ER -