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Cerebral Cortex, Vol. 10, No. 1, 2-22, January 2000
© 2000 Oxford University Press

Visual Function and Brain Organization in Non-decussating Retinal–Fugal Fibre Syndrome

Jonathan D. Victor1, Patricia Apkarian2, Joy Hirsch1,3, Mary M. Conte1, Maurine Packard1, Norman R. Relkin1, Karl H.S. Kim1,3 and Robert M. Shapley4

1 Department of Neurology and Neuroscience, The New York Presbyterian Hospital, New York, NY 10021, USA, , 2 Department of Physiology, Medical Faculty, Erasmus University Rotterdam, 3000 DR Rotterdam, The Netherlands, , 3 Department of Neurology, Memorial Sloan–Kettering Cancer Center, New York, NY 10021 and , 4 Center for Neural Science, New York University, New York, NY 10003, USA

Functional neuroimaging, psychophysical and electrophysiological investigations were performed in a patient with non-decussating retinal–fugal fibre syndrome, an inborn achiasmatic state in which the retinal projections of each eye map entirely to the ipsilateral primary visual cortex. Functional magnetic resonance imaging (fMRI) studies showed that for monocularly presented simple visual stimuli, only the ipsilateral striate cortex was activated. Within each hemisphere's striate cortex, the representation of the two hemifields overlapped extensively. Despite this gross miswiring, visual functions that require precise geometrical information (such as vernier acuity) were normal, and there was no evidence for the confounding of visual information between the overlapping ipsi-lateral and contralateral representations. Contrast sensitivity and velocity judgments were abnormal, but their dependence on the orientation and velocity of the targets suggests that this deficit was due to ocular instabilities, rather than the miswiring per se. There were no asymmetries in performance observed in visual search, visual naming or illusory contour perception. fMRI analysis of the latter two tasks under monocular viewing conditions indicated extensive bilateral activation of striate and prestriate areas. Thus, the remarkably normal visual behavior achieved by this patient is a result of both the plasticity of visual pathways, and efficient transfer of information between the hemispheres.


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