Abstract
The study investigates long-term recognition memory in congenital prosopagnosia (CP), a lifelong impairment in face identification that is present from birth. Previous investigations of processing deficits in CP have mostly relied on short-term recognition tests to estimate the scope and severity of individual deficits. We firstly report on a controlled test of long-term (one year) recognition memory for faces and objects conducted with a large group of participants with CP. Long-term recognition memory is significantly impaired in eight CP participants (CPs). In all but one case, this deficit was selective to faces and didn't extend to intra-class recognition of object stimuli. In a test of famous face recognition, long-term recognition deficits were less pronounced, even after accounting for differences in media consumption between controls and CPs. Secondly, we combined test results on long-term and short-term recognition of faces and objects, and found a large heterogeneity in severity and scope of individual deficits. Analysis of the observed heterogeneity revealed a dissociation of CP into subtypes with a homogeneous phenotypical profile. Thirdly, we found that among CPs self-assessment of real-life difficulties, based on a standardized questionnaire, and experimentally assessed face recognition deficits are strongly correlated. Our results demonstrate that controlled tests of long-term recognition memory are needed to fully assess face recognition deficits in CP. Based on controlled and comprehensive experimental testing, CP can be dissociated into subtypes with a homogeneous phenotypical profile. The CP subtypes identified align with those found in prosopagnosia caused by cortical lesions; they can be interpreted with respect to a hierarchical neural system for face perception.
Highlights
Prosopagnosia refers to a selective deficit in the processing of facial identity [1]
Categorization of processing deficits The second aim of this study is to investigate a symptomatic categorization of congenital prosopagnosia along the lines of an apperceptive, associative, or amnestic subtype [5,49]
Testing for group differences in face recognition performance, we found that Congenital prosopagnosia (CP), with a median error rate of 14.7%, perform significantly worse than controls, with a median of 6.3% (Wilcoxon rank sum test, W = 267, n0 = 25, nCP = 13, p,0.001 one-sided)
Summary
Prosopagnosia refers to a selective deficit in the processing of facial identity [1]. Initial reports of prosopagnosia only covered cases where the deficit was acquired due to cortical lesions [1,2,3,4,5,6,7,8,9], see [10] for a review of 74 cases. Acquired prosopagnosia (AP) is a heterogeneous disorder, where the nature, extent, and selectivity of the deficit depend on the exact location of the lesion. Variations include a lack of overt recognition of familiar faces with intact covert recognition [8,11,12,13,14], a deficit in configural encoding of faces (and objects) [15,16,17], an impaired imagery of faces [18], and difficulties in processing facial expressions [19]. Congenital prosopagnosia (CP) is among the most common anomalies in humans with a prevalence of 2.5% [28], and is almost always hereditary [29,30,31,32], see Discussion
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