Familial Transmission of Developmental Prosopagnosia: New Case Reports from an Extended Family and Identical Twins.

Existing evidence suggests that developmental prosopagnosia (DP) is a surprisingly prevalent condition, with some individuals describing lifelong difficulties with facial identity recognition. Together with case reports of multiple family members with the condition, this evidence suggests that DP is inherited in at least some instances. Here, we offer some novel case series that further support the heritability of the condition. First, we describe five adult siblings who presented to our lab with symptoms of DP. Second, for the first known time in the literature, we describe a pair of adult identical twins who contacted us in the belief that they both experience DP. The condition was confirmed in three of the five siblings (with minor symptoms observed in the remaining two) and in both twins. Supplementary assessments suggested that all individuals also experienced some degree of difficulty with facial identity perception, but that object recognition was preserved. These findings bolster the evidence supporting the heritability of DP and suggest that it can be a specific impairment in some cases.

Face specific inversion effects provide evidence for two subtypes of developmental prosopagnosia.

Many studies have attempted to identify the perceptual underpinnings of developmental prosopagnosia (DP). The majority have focused on whether holistic and configural processing mechanisms are impaired in DP. However, previous work suggests that there is substantial heterogeneity in holistic and configural processing within the DP population; further, there is disagreement as to whether any deficits are face-specific or reflect a broader perceptual deficit. This study used a data-driven approach to examine whether there are systematic patterns of variability in DP that reflect different underpinning perceptual deficits. A group of individuals with DP (N = 37) completed a cognitive battery measuring holistic/configural and featural processing in faces and non-face objects. A two-stage cluster analysis on data from the Cambridge Face Perception Test identified two subgroups of DPs. Across several tasks, the first subgroup (N = 21) showed typical patterns of holistic/configural processing (measured via inversion effects); the second (N = 16) was characterised by reduced or abolished inversion effects compared to age-matched control participants (N = 91). The subgroups did not differ on tasks measuring upright face matching, object matching, non-face holistic processing, or composite effects. These findings indicate two separable pathways to face recognition impairment, one characterised by impaired configural processing and the other potentially by impaired featural processing. Comparisons to control participants provide some preliminary evidence that the deficit in featural processing may extend to some non-face stimuli. Our results demonstrate the utility of examining both the variability between and consistency across individuals with DP as a means of illuminating our understanding of face recognition in typical and atypical populations.

An update of the Benton Facial Recognition Test.

The Benton Facial Recognition Test (BFRT) is a paper-and-pen task that is traditionally used to assess face perception skills in neurological, clinical and psychiatric conditions. Despite criticisms of its stimuli, the task enjoys a simple procedure and is rapid to administer. Further, it has recently been computerised (BFRT-c), allowing reliable measurement of completion times and the need for online testing. Here, in response to calls for repeat screening for the accurate detection of face processing deficits, we present the BFRT-Revised (BFRT-r): a new version of the BFRT-c that maintains the task's basic paradigm, but employs new, higher-quality stimuli that reflect recent theoretical advances in the field. An initial validation study with typical participants indicated that the BFRT-r has good internal reliability and content validity. A second investigation indicated that while younger and older participants had comparable accuracy, completion times were longer in the latter, highlighting the need for age-matched norms. Administration of the BFRT-r and BFRT-c to 32 individuals with developmental prosopagnosia resulted in improved sensitivity in diagnostic screening for the BFRT-r compared to the BFRT-c. These findings are discussed in relation to current diagnostic screening protocols for face perception deficits. The BFRT-r is stored in an open repository and is freely available to other researchers.

Diagnosing developmental prosopagnosia: repeat assessment using the Cambridge Face Memory Test.

Developmental prosopagnosia (DP) is a cognitive condition characterized by a relatively selective impairment in face recognition. Currently, people are screened for DP via a single attempt at objective face-processing tests, usually all presented on the same day. However, several variables probably influence performance on these tests irrespective of actual ability, and the influence of repeat administration is also unknown. Here, we assess, for the first known time, the test-retest reliability of the Cambridge Face Memory Test (CFMT)-the leading task used worldwide to diagnose DP. This value was found to fall just below psychometric standards, and single-case analyses revealed further inconsistencies in performance that were not driven by testing location (online or in-person), nor the time-lapse between attempts. Later administration of an alternative version of the CFMT (the CFMT-Aus) was also found to be valuable in confirming borderline cases. Finally, we found that performance on the first 48 trials of the CFMT was equally as sensitive as the full 72-item score, suggesting that the instrument may be shortened for testing efficiency. We consider the implications of these findings for existing diagnostic protocols, concluding that two independent tasks of unfamiliar face memory should be completed on separate days.

Enhanced Matching of Children's Faces in "Super-Recognisers" But Not High-Contact Controls.

Face matching is notoriously error-prone, and some work suggests additional difficulty when matching the faces of children. It is possible that individuals with natural proficiencies in adult face matching ("super-recognisers" [SRs]) will also excel at the matching of children's faces, although other work implicates facilitations in typical perceivers who have high levels of contact with young children (e.g., nursery teachers). This study compared the performance of both of these groups on adult and child face matching to a group of low-contact controls. High- and low-contact control groups performed at a remarkably similar level in both tasks, whereas facilitations for adult and child face matching were observed in some (but not all) SRs. As a group, the SRs performed better in the adult compared with the child task, demonstrating an extended own-age bias compared with controls. These findings suggest that additional exposure to children's faces does not assist the performance in a face matching task, and the mechanisms underpinning superior recognition of adult faces can also facilitate the child face recognition. Real-world security organisations should therefore seek individuals with general facilitations in face matching for both adult and child face matching tasks.

The domain-specificity of face matching impairments in 40 cases of developmental prosopagnosia.

A prevailing debate in the psychological literature concerns the domain-specificity of the face recognition system, where evidence from typical and neurological participants has been interpreted as evidence that faces are "special". Although several studies have investigated the same question in cases of developmental prosopagnosia, the vast majority of this evidence has recently been discounted due to methodological concerns. This leaves an uncomfortable void in the literature, restricting our understanding of the typical and atypical development of the face recognition system. The current study addressed this issue in 40 individuals with developmental prosopagnosia, completing a sequential same/different face and biological (hands) and non-biological (houses) object matching task, with upright and inverted conditions. Findings support domain-specific accounts of face-processing for both hands and houses: while significant correlations emerged between all the object categories, no condition correlated with performance in the upright faces condition. Further, a categorical analysis demonstrated that, when face matching was impaired, object matching skills were classically dissociated in six out of 15 individuals (four for both categories). These findings provide evidence about domain-specificity in developmental disorders of face recognition, and present a theoretically-driven means of partitioning developmental prosopagnosia.

Objective Patterns of Face Recognition Deficits in 165 Adults with Self-Reported Developmental Prosopagnosia.

In the last 15 years, increasing numbers of individuals have self-referred to research laboratories in the belief that they experience severe everyday difficulties with face recognition. The condition "developmental prosopagnosia" (DP) is typically diagnosed when impairment is identified on at least two objective face-processing tests, usually involving assessments of face perception, unfamiliar face memory, and famous face recognition. While existing evidence suggests that some individuals may have a mnemonic form of prosopagnosia, it is also possible that other subtypes exist. The current study assessed 165 adults who believe they experience DP, and 38% of the sample were impaired on at least two of the tests outlined above. While statistical dissociations between face perception and face memory were only observed in four cases, a further 25% of the sample displayed dissociations between impaired famous face recognition and intact short-term unfamiliar face memory and face perception. We discuss whether this pattern of findings reflects (a) limitations within dominant diagnostic tests and protocols, (b) a less severe form of DP, or (c) a currently unrecognized but prevalent form of the condition that affects long-term face memory, familiar face recognition or semantic processing.

Self-ratings of face recognition ability are influenced by gender but not prosopagnosia severity.

Growing awareness of developmental prosopagnosia has resulted in large numbers of people self-referring for prosopagnosia screening. Objective assessment depends heavily on available resources; thus, some researchers use self-ratings of face recognition ability to reduce candidate lists. However, our own metacognitive awareness of our face recognition skills has been much debated, and there is mixed evidence on the reliability of self-report measures. Nevertheless, some behavioral trait questionnaires have proved more useful, although it remains unclear whether these instruments can tap prosopagnosia severity or whether responses are influenced by participant gender (as in other developmental disorders). We investigated these issues in 47 adults with developmental prosopagnosia. No relationship was observed between questionnaire scores and prosopagnosia severity, but males were found to underreport prosopagnosia symptoms relative to females. Thus, we recommend caution in the interpretation of low scores on self-report questionnaires and suggest that separate norms are developed for male and female participants. (PsycINFO Database Record (c) 2019 APA, all rights reserved).

The limits of super recognition: An other-ethnicity effect in individuals with extraordinary face recognition skills.

In the last decade there has been increasing interest in super-recognizers, who have an extraordinary ability to recognize faces. However, it has not yet been investigated whether these individuals are subject to the same biases in face recognition as typical perceivers. The most renowned constraint reported to date is the other-ethnicity effect, whereby people are better at recognizing faces from their own, compared with other, ethnicities. If super-recognizers also show this bias, it is possible that they are no better at other-ethnicity face recognition than typical native perceivers-a finding that would have important theoretical and practical implications. In the current study, eight Caucasian super-recognizers performed other-ethnicity tests of face memory and face matching. In Experiment 1, super-recognizers outperformed Caucasian but not Asian controls in their memory for Asian faces. In Experiment 2, a similar pattern emerged in some super-recognizers on a test of face matching. Finally, Experiment 3 examined the consistency of superior other-ethnicity face matching in relation to Caucasian controls, using Arab and Black faces. Only four super-recognizers consistently outperformed controls, and other-ethnicity matching performance was not related to Caucasian face-matching or own- or other-ethnicity face memory. These findings suggest that super-recognizers are subject to the same biases as typical perceivers, and are simply those at the top end of a common face recognition spectrum as opposed to a qualitatively different group of individuals. (PsycINFO Database Record (c) 2019 APA, all rights reserved).

Applied screening tests for the detection of superior face recognition.

In recent years there has been growing interest in the identification of people with superior face recognition skills, for both theoretical and applied investigations. These individuals have mostly been identified via their performance on a single attempt at a tightly controlled test of face memory-the long form of the Cambridge Face Memory Test (CFMT+). The consistency of their skills over a range of tests, particularly those replicating more applied policing scenarios, has yet to be examined systematically. The current investigation screened 200 people who believed they have superior face recognition skills, using the CFMT+ and three new, more applied tests (measuring face memory, face matching and composite-face identification in a crowd). Of the sample, 59.5% showed at least some consistency in superior face recognition performance, although only five individuals outperformed controls on overall indices of target-present and target-absent trials. Only one participant outperformed controls on the Crowds test, suggesting that some applied face recognition tasks require very specific skills. In conclusion, future screening protocols need to be suitably thorough to test for consistency in performance, and to allow different types of superior performer to be detected from the outset. Screening for optimal performers may sometimes need to directly replicate the task in question, taking into account target-present and target-absent performance. Self-selection alone is not a reliable means of identifying those at the top end of the face recognition spectrum.

Identifying Hallmark Symptoms of Developmental Prosopagnosia for Non-Experts.

Developmental prosopagnosia (DP) is characterised by a severe and relatively selective deficit in face recognition, in the absence of neurological injury. Because public and professional awareness of DP is low, many adults and children are not identified for formal testing. This may partly result from the lack of appropriate screening tools that can be used by non-experts in either professional or personal settings. To address this issue, the current study sought to (a) explore when DP can first be detected in oneself and another, and (b) identify a list of the condition's everyday behavioural manifestations. Questionnaires and interviews were administered to large samples of adult DPs, their unaffected significant others, and parents of children with the condition; and data were analysed using inductive content analysis. It was found that DPs have limited insight into their difficulties, with most only achieving realisation in adulthood. Nevertheless, the DPs' reflections on their childhood experiences, together with the parental responses, revealed specific indicators that can potentially be used to spot the condition in early childhood. These everyday hallmark symptoms may aid the detection of individuals who would benefit from objective testing, in oneself (in adults) or another person (for both adults and children).

Prevalence of face recognition deficits in middle childhood.

Approximately 2-2.5% of the adult population is believed to show severe difficulties with face recognition, in the absence of any neurological injury-a condition known as developmental prosopagnosia (DP). However, to date no research has attempted to estimate the prevalence of face recognition deficits in children, possibly because there are very few child-friendly, well-validated tests of face recognition. In the current study, we examined face and object recognition in a group of primary school children (aged 5-11 years), to establish whether our tests were suitable for children and to provide an estimate of face recognition difficulties in children. In Experiment 1 (n = 184), children completed a pre-existing test of child face memory, the Cambridge Face Memory Test-Kids (CFMT-K), and a bicycle test with the same format. In Experiment 2 (n = 413), children completed three-alternative forced-choice matching tasks with faces and bicycles. All tests showed good psychometric properties. The face and bicycle tests were well matched for difficulty and showed a similar developmental trajectory. Neither the memory nor the matching tests were suitable to detect impairments in the youngest groups of children, but both tests appear suitable to screen for face recognition problems in middle childhood. In the current sample, 1.2-5.2% of children showed difficulties with face recognition; 1.2-4% showed face-specific difficulties-that is, poor face recognition with typical object recognition abilities. This is somewhat higher than previous adult estimates: It is possible that face matching tests overestimate the prevalence of face recognition difficulties in children; alternatively, some children may "outgrow" face recognition difficulties.