To investigate psychophysical thresholds in Stargardt disease with the full-field stimulus test (FST).
Visual acuity, spectral domain optical coherence tomography, full-field electroretinogram, and FST measurements were made in 1 eye of 24 patients with Stargardt disease. Dark-adapted rod FST thresholds were measured with short-wavelength stimuli, and cone FST thresholds were obtained from the cone plateau phase of dark adaptation using long-wavelength stimuli. Correlation coefficients were calculated for FST thresholds versus macular thickness, visual acuity, and electroretinogram amplitudes.
The Stargardt disease patients' FST cone thresholds correlated significantly with visual acuity, macular thickness, and electroretinogram cone response amplitudes (all P < 0.01). The patients' FST rod thresholds correlated with electroretinogram rod response amplitudes (P < 0.01) but not macular thickness (P = 0.05). All patients with Stargardt disease with flecks confined to the macula, and most of the patients with flecks extending outside of the macula had normal FST thresholds. All patients with extramacular atrophic changes had elevated FST cone thresholds and most had elevated FST rod thresholds.
Full-field stimulus test rod and cone threshold elevation in patients with Stargardt disease correlated well with measures of structure and function, as well as ophthalmoscopic retinal appearance. The Full-field stimulus test appears to be a useful tool for assessing rod and cone function in Stargardt disease.
Rod- and cone-mediated full-field stimulus test thresholds were found to correlate with visual acuity, full-field electroretinogram amplitudes, and macular thickness in 24 patients with Stargardt disease. Full-field stimulus test seems to be a useful tool for assessing rod and cone function in Stargardt disease.
*Pangere Center for Hereditary Retinal Diseases, The Chicago Lighthouse for People Who Are Blind or Visually Impaired, Chicago, Illinois;
†Department of Ophthalmology, University of Illinois at Chicago, Chicago, Illinois; and
Departments of ‡Ophthalmology, and
§Pathology and Cell Biology, Columbia University, New York, New York.
Reprint requests: Gerald A. Fishman, MD, The Pangere Center for Hereditary Retinal Diseases, The Chicago Lighthouse for People Who Are Blind or Visually Impaired, 1850 W. Roosevelt Road, Chicago, IL 60608; e-mail: firstname.lastname@example.org
None of the authors have any conflicting interests to disclose.
Supported by Cless Family Foundation (Northbrook, IL), Foundation Fighting Blindness (Owings Mills, MD), Pangere Corporation, and Grousbeck Family Foundation (Stanford, CA) (G.A.F.); NIH R01EY021163 and R24EY019861 (R.A.); NIH R00EY0195510 (J.J.M.); NIH core grant P30EY001792; and an unrestricted departmental grant from Research to Prevent Blindness.