Frontiers in Bioscience-Elite (FBE) is published by IMR Press from Volume 13 Issue 2 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on imrpress.com as a courtesy and upon agreement with Frontiers in Bioscience.
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The goal of the current paper is to review recent findings concerning the neural basis of congenital prosopagnosia (CP), a lifelong impairment in face processing that occurs in the absence of explicit brain damage. As such, CP offers a unique model for exploring the psychological and neural bases of normal face processing. We start by providing background about face perception and representation, and then review behavioral evidence gleaned from individuals with CP. We then review recent functional and structural neural investigations which offer a comprehensive account of the mechanisms underlying CP and support a characterization of this impairment as a disconnection syndrome rather than as a syndrome related to focal brain malfunction. We end the paper by offering a general framework for CP which, we believe, best integrates the behavioral and neural findings, and offers a platform for generating hypotheses for future studies. There remain many open issues in our understanding of CP and, to address these unanswered questions, we lay out several future research directions and testable hypotheses for further investigation.