Hyper-vision in a patient with central and paracentral vision loss reflects cortical reorganisation

SM, a 21-year-old female, presents an extensive central scotoma (30 deg) with dense absolute scotoma (visual acuity = 10/100) in the macular area (10 deg) due to Stargardt's disease. We provide behavioral evidence of cortical plastic reorganization since the patient could perform several visual tasks with her poor-vision eyes better than controls, although high spatial frequency sensitivity and visual acuity are severely impaired. Between 2.5-deg and 12-deg eccentricities, SM presented (1) normal acuity for crowded letters, provided stimulus size is above acuity thresholds for single letters; (2) a two-fold sensitivity increase (d-prime) with respect to controls in a simple search task; and (3) largely above-threshold performance in a lexical decision task carried out randomly by controls. SM's hyper-vision may reflect a long-term sensory gain specific for unimpaired low spatial-frequency mechanisms, which may result from modifications in response properties due to practice-dependent changes in excitatory/inhibitory intracortical connections.