Prosopagnosia is a selective visual agnosia characterized by the inability to recognize the identity of faces. There are both acquired forms secondary to brain damage and developmental forms without obvious structural lesions. In this review, we first discuss the diagnosis of acquired and developmental prosopagnosia, and the challenges present in the latter case. Second, we discuss the evidence regarding the selectivity of the prosopagnosic defect, particularly in relation to the recognition of other objects, written words (another visual object category requiring high expertise), and voices. Third, we summarize recent findings about the structural and functional basis of prosopagnosia from studies using magnetic resonance imaging, functional magnetic resonance imaging, and event-related potentials. Finally, we discuss recent attempts at rehabilitation of face recognition in prosopagnosia.

The “many-to-many” hypothesis proposes that visual object processing is supported by distributed circuits that overlap for different object categories. For faces and words the hypothesis posits that both posterior fusiform regions contribute to both face and visual word perception and predicts that unilateral lesions impairing one will affect the other. However, studies testing this hypothesis have produced mixed results. We evaluated visual word processing in subjects with developmental prosopagnosia, a condition linked to right posterior fusiform abnormalities. Ten developmental prosopagnosic subjects performed a word-length effect task and a task evaluating the recognition of word content across variations in text style, and the recognition of style across variations in word content. All subjects had normal word-length effects. One had prolonged sorting time for word recognition in handwritten stimuli. These results suggest that the deficit in developmental prosopagnosia is unlikely to affect visual word processing, contrary to predictions of the many-to-many hypothesis.

Developmental prosopagnosia (DP) is defined by severe face recognition problems resulting from a failure to develop the necessary visual mechanisms for processing faces. While there is a growing literature on DP in adults, little has been done to study this disorder in children. The profound impact of abnormal face perception on social functioning and the general lack of awareness of childhood DP can result in severe social and psychological consequences for children. This review discusses possible aetiologies of DP and summarizes the few cases of childhood DP that have been reported. It also outlines key objectives for the growth of this emerging research area and special considerations for studying DP in children. With clear goals and concerted efforts, the study of DP in childhood will be an exciting avenue for enhancing our understanding of normal and abnormal face perception for all age groups.