Frederick T Collison1, Gerald A Fishman, J Jason McAnany, Jana Zernant, Rando Allikmets. 1. *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.
Abstract
PURPOSE: To investigate psychophysical thresholds in Stargardt disease with the full-field stimulus test (FST). METHODS: 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. RESULTS: 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. CONCLUSION: 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.
PURPOSE: To investigate psychophysical thresholds in Stargardt disease with the full-field stimulus test (FST). METHODS: 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. RESULTS: The Stargardt diseasepatients' 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. CONCLUSION: 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.
Authors: Sarah C Westeneng-van Haaften; Camiel J F Boon; Frans P M Cremers; Lies H Hoefsloot; Anneke I den Hollander; Carel B Hoyng Journal: Ophthalmology Date: 2012-03-24 Impact factor: 12.079
Authors: Alberta A H J Thiadens; T My Lan Phan; Renate C Zekveld-Vroon; Bart P Leroy; L Ingeborgh van den Born; Carel B Hoyng; Caroline C W Klaver; Susanne Roosing; Jan-Willem R Pott; Mary J van Schooneveld; Norka van Moll-Ramirez; Maria M van Genderen; Camiel J F Boon; Anneke I den Hollander; Arthur A B Bergen; Elfride De Baere; Frans P M Cremers; Andrew J Lotery Journal: Ophthalmology Date: 2012-01-20 Impact factor: 12.079
Authors: Robert F Mullins; Markus H Kuehn; Roxana A Radu; G Stephanie Enriquez; Jade S East; Emily I Schindler; Gabriel H Travis; Edwin M Stone Journal: Invest Ophthalmol Vis Sci Date: 2012-04-18 Impact factor: 4.799
Authors: Samuel G Jacobson; Artur V Cideciyan; Igor V Peshenko; Alexander Sumaroka; Elena V Olshevskaya; Lihui Cao; Sharon B Schwartz; Alejandro J Roman; Melani B Olivares; Sam Sadigh; King-Wai Yau; Elise Heon; Edwin M Stone; Alexander M Dizhoor Journal: Hum Mol Genet Date: 2012-10-03 Impact factor: 6.150
Authors: Kaoru Fujinami; Noemi Lois; Alice E Davidson; Donna S Mackay; Chris R Hogg; Edwin M Stone; Kazushige Tsunoda; Kazuo Tsubota; Catey Bunce; Anthony G Robson; Anthony T Moore; Andrew R Webster; Graham E Holder; Michel Michaelides Journal: Am J Ophthalmol Date: 2013-03-15 Impact factor: 5.258
Authors: Sarwar Zahid; Thiran Jayasundera; William Rhoades; Kari Branham; Naheed Khan; Leslie M Niziol; David C Musch; John R Heckenlively Journal: Am J Ophthalmol Date: 2012-12-05 Impact factor: 5.258
Authors: R Riveiro-Alvarez; J Aguirre-Lamban; M Angel Lopez-Martinez; M Jose Trujillo-Tiebas; D Cantalapiedra; E Vallespin; A Avila-Fernandez; C Ramos; C Ayuso Journal: Br J Ophthalmol Date: 2008-10-31 Impact factor: 4.638
Authors: Olga Zabek; Giacomo Calzetti; Dominique Prétot; Hendrik P N Scholl; Maria Della Volpe Waizel Journal: Graefes Arch Clin Exp Ophthalmol Date: 2022-03-31 Impact factor: 3.535
Authors: Frederick T Collison; Jason C Park; Gerald A Fishman; J Jason McAnany; Edwin M Stone Journal: Invest Ophthalmol Vis Sci Date: 2015-11 Impact factor: 4.799
Authors: Mays Talib; Mary J van Schooneveld; Jan Wijnholds; Maria M van Genderen; Nicoline E Schalij-Delfos; Herman E Talsma; Ralph J Florijn; Jacoline B Ten Brink; Frans P M Cremers; Alberta A H J Thiadens; L Ingeborgh van den Born; Carel B Hoyng; Magda A Meester-Smoor; Arthur A Bergen; Camiel J F Boon Journal: Acta Ophthalmol Date: 2021-02-02 Impact factor: 3.761
Authors: Stephen R Russell; Arlene V Drack; Artur V Cideciyan; Samuel G Jacobson; Bart P Leroy; Caroline Van Cauwenbergh; Allen C Ho; Alina V Dumitrescu; Ian C Han; Mitchell Martin; Wanda L Pfeifer; Elliott H Sohn; Jean Walshire; Alexandra V Garafalo; Arun K Krishnan; Christian A Powers; Alexander Sumaroka; Alejandro J Roman; Eva Vanhonsebrouck; Eltanara Jones; Fanny Nerinckx; Julie De Zaeytijd; Rob W J Collin; Carel Hoyng; Peter Adamson; Michael E Cheetham; Michael R Schwartz; Wilhelmina den Hollander; Friedrich Asmus; Gerard Platenburg; David Rodman; Aniz Girach Journal: Nat Med Date: 2022-04-04 Impact factor: 87.241
Authors: Artur V Cideciyan; Samuel G Jacobson; Allen C Ho; Arun K Krishnan; Alejandro J Roman; Alexandra V Garafalo; Vivian Wu; Malgorzata Swider; Alexander Sumaroka; Caroline Van Cauwenbergh; Stephen R Russell; Arlene V Drack; Bart P Leroy; Michael R Schwartz; Aniz Girach Journal: Ophthalmol Sci Date: 2022-03-02
Authors: Thomas L Edwards; Charles L Cottriall; Kanmin Xue; Matthew P Simunovic; James D Ramsden; Eberhart Zrenner; Robert E MacLaren Journal: Ophthalmology Date: 2017-10-27 Impact factor: 12.079