BACKGROUND: In human albinism the plasticity of the visual system is challenged, as part of the temporal retina projects abnormally to the contralateral hemisphere. AIM: To investigate whether the sensitivity of the abnormally projecting temporal retina is preserved. METHODS: Light spot detection sensitivities were assessed in the central 20 degrees of the visual field in 15 patients with albinism and electrophysiologically determined extent of the retinal projection abnormality, and in 6 controls. The sensitivities were determined monocularly with static white-on-white perimetry using Octopus 101. RESULTS: In the patients with albinism, the sensitivity of the abnormally projecting part of the temporal retina was not selectively reduced. Apart from the vicinity of the papilla, there was no significant sensitivity difference between nasal and temporal retina in both patients with albinism and controls. CONCLUSIONS: While in the present study the papilla-induced scotoma was detected in those with albinism tested, there was no indication of a selective visual field defect induced by the projection abnormality. This contrasts with the selective visual field defects observed in some animal models of albinism, and indicates that, in humans, mechanisms of cortical self-organisation make the abnormal representation available for visual perception.
BACKGROUND: In human albinism the plasticity of the visual system is challenged, as part of the temporal retina projects abnormally to the contralateral hemisphere. AIM: To investigate whether the sensitivity of the abnormally projecting temporal retina is preserved. METHODS: Light spot detection sensitivities were assessed in the central 20 degrees of the visual field in 15 patients with albinism and electrophysiologically determined extent of the retinal projection abnormality, and in 6 controls. The sensitivities were determined monocularly with static white-on-white perimetry using Octopus 101. RESULTS: In the patients with albinism, the sensitivity of the abnormally projecting part of the temporal retina was not selectively reduced. Apart from the vicinity of the papilla, there was no significant sensitivity difference between nasal and temporal retina in both patients with albinism and controls. CONCLUSIONS: While in the present study the papilla-induced scotoma was detected in those with albinism tested, there was no indication of a selective visual field defect induced by the projection abnormality. This contrasts with the selective visual field defects observed in some animal models of albinism, and indicates that, in humans, mechanisms of cortical self-organisation make the abnormal representation available for visual perception.
Authors: Bernd Schmitz; Barbara Käsmann-Kellner; Torsten Schäfer; Christoph M Krick; Georg Grön; Martin Backens; Wolfgang Reith Journal: Hum Brain Mapp Date: 2004-09 Impact factor: 5.038
Authors: Falko R Kaule; Barbara Wolynski; Irene Gottlob; Joerg Stadler; Oliver Speck; Martin Kanowski; Synke Meltendorf; Wolfgang Behrens-Baumann; Michael B Hoffmann Journal: Hum Brain Mapp Date: 2014-04-25 Impact factor: 5.038
Authors: Michael B Hoffmann; Falko R Kaule; Netta Levin; Yoichiro Masuda; Anil Kumar; Irene Gottlob; Hiroshi Horiguchi; Robert F Dougherty; Joerg Stadler; Barbara Wolynski; Oliver Speck; Martin Kanowski; Yaping J Liao; Brian A Wandell; Serge O Dumoulin Journal: Neuron Date: 2012-08-09 Impact factor: 17.173
Authors: G Di Rosa; P Malaspina; P Blasi; C Dionisi-Vici; C Rizzo; G Tortorella; S R Crutchfield; K M Gibson Journal: J Inherit Metab Dis Date: 2009-05-30 Impact factor: 4.982