Diagnostic clinical findings of a new syndrome with night blindness, maculopathy, and enchanced S cone sensitivity

M. F. Marmor, S. G. Jacobson, M. H. Foerster, U. Kellner, R. G. Weleber

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117 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)124-134
Number of pages11
JournalAmerican journal of ophthalmology
Issue number2
StatePublished - Jan 1 1990


ASJC Scopus subject areas

  • Ophthalmology

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