Severe developmental prosopagnosia in a child with superior intellect.

Developmental prosopagnosia, a lifelong inability to learn and recognize familiar faces, has rarely been reported, and there are even fewer cases that have been studied during childhood. Of the cases studied during childhood, significant "apperceptive" features to the face recognition defect have been noted. We had an opportunity to conduct extensive standard and experimental neuropsychological, psychophysiological, and neuroanatomical studies in a five-year-old child with severe developmental prosopagnosia. The subject was intellectually gifted (FSIQ = 130), but had a marked discrepancy between verbal and nonverbal abilities (VIQ = 140, PIQ = 110). Although some visual perceptual weaknesses were apparent, the subject's face recognition defect was found to cnform most closely to the "associative" type, and he did not have visual recognition deficits for any categories of nonunique entities. A novel finding was that the child's covert recognition of familiar faces based on an autonomic index was normal, suggesting that as in some adult-onset cases, the brain is capable of acquiring some information about familiar faces, even without conscious recognition. The child also had normal judgments of facial emotional expressions. Our report extends the understanding of the neuropsychological features of developmental prosopagnosia, and may help narrow the search for neuroanatomical correlates of this condition, which have yet to be identified.