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Background:
Case Report

Assessment of Developmental Prosopagnosia in an Individual with Tourette Syndrome and Attention Deficit Hyperactivity Disorder: A Case Report

by
Petter Espeseth Emhjellen
1,
Randi Starrfelt
2,*,
Rune Raudeberg
3 and
Bjørnar Hassel
1
1
Department of Neurohabilitation, Oslo University Hospital, 0424 Oslo, Norway
2
Department of Psychology, University of Copenhagen, 1172 Copenhagen, Denmark
3
Department of Biological and Medical Psychology, Faculty of Psychology, University of Bergen, 5007 Bergen, Norway
*
Author to whom correspondence should be addressed.
Brain Sci. 2025, 15(1), 56; https://doi.org/10.3390/brainsci15010056
Submission received: 10 December 2024 / Revised: 5 January 2025 / Accepted: 8 January 2025 / Published: 10 January 2025
(This article belongs to the Section Neuropsychology)
Review Reports Versions Notes

Abstract

:
Background/Objectives: Prosopagnosia is the inability to recognize people by their faces. Developmental prosopagnosia is the hereditary or congenital variant of the condition. The aim of this study was to demonstrate the assessment of developmental prosopagnosia in a clinical context, using a combination of commercially available clinical assessment tools and experimental tools described in the research literature. Methods: We conducted a comprehensive neuropsychological assessment of a man with Tourette syndrome and attention deficit hyperactivity disorder (ADHD). The patient (ON) had experienced difficulties with face identity recognition throughout his life but believed they were caused by a lack of interest in others. Results: The neuropsychological assessment revealed varying degrees of difficulties primarily related to executive functions, attention, reaction time, and memory processes, as expected in a person with Tourette’s syndrome and ADHD. In addition, ON reported severe problems with face recognition on a prosopagnosia questionnaire and demonstrated severely impaired performance on tests of face memory and face perception commonly used to diagnose prosopagnosia. Interestingly, he reported familial face recognition problems on the maternal side of the family, while tics and ADHD symptoms occurred on the paternal side. This suggests that, in this case, the conditions were likely inherited through different genetic pathways. Conclusions: Proper assessment of face recognition problems, which includes a broad spectrum of clinical assessment tools, could help patients develop awareness and acceptance of themselves and their difficulties, and could serve as a basis for the development of clinical interventions. While ON’s DP, Tourette syndrome, and ADHD may have distinct genetic origins, impairment in face identity recognition has been observed across several neurodevelopmental conditions and is likely more common than currently thought.

1. Introduction

Prosopagnosia, or face blindness, is characterized by severely impaired face identity recognition due to disturbances in face processing mechanisms [1]. Prosopagnosia following brain injury (acquired prosopagnosia) is well documented following focal lesions in occipital and temporal areas in the right hemisphere or bilaterally [2]. More recently, it has become clear that prosopagnosia also exists in a congenital or developmental form (developmental prosopagnosia; DP) [3].
Face perception and recognition are supported by a distributed neural network where portions of the inferior occipital gyrus, the fusiform gyrus, and the medial temporal lobe play crucial roles [4]. The right hemisphere is dominant for face processing in most people, but regions in the left hemisphere also contribute to efficient face processing, and acquired prosopagnosia is typically more severe following bilateral lesions [2]. DP has been estimated to affect around 2–3% of the population [5,6,7], although both diagnostic criteria, cut-off scores, and prevalence estimates are continuously debated within the field (see for example [8,9,10,11]).
Face recognition may be impaired in different neurodevelopmental and psychiatric conditions, e.g., autism spectrum disorder [12,13,14,15], schizophrenia [16,17], dyslexia [18,19,20], attention deficit hyperactivity disorder (ADHD) [21,22], Turner syndrome [23], and Möbius sequence [24]. Many people with face recognition problems report other co-occurring deficits, for example, perceptual or memory problems, and diagnoses [25]. Despite the increasing focus on both specific and associated impairments in face recognition, few clinical tools are available for the assessment of this important cognitive function. The aim of this report is to illustrate how face identity recognition can be assessed, informed by DP assessment guidelines [3,26], in patients with neurodevelopmental disorders with the use of both experimental and commercially available clinical assessment tools.

2. Materials and Methods

2.1. Case Description

The patient, referred to as “ON” in this report, was suspected by his general practitioner to have Tourette syndrome (TS). He was referred to the Department of Neurohabilitation, Oslo University Hospital, Oslo, Norway, where he was diagnosed with TS and ADHD, and he underwent neuropsychological assessment of face recognition difficulties. ON was then in his late forties.
In addition to motor and phonic tics, ON had had lifelong problems related to attention, impulsivity, and hyperactivity. He also reported having difficulties recognizing people by their faces. His father had a diagnosis of TS as well as ADHD-like symptoms. Further, his paternal uncle and grandfather had chronic motor and phonic tics. ON had heard anecdotes of several relatives on his mother’s side, including his maternal uncle, having faced identity recognition problems. It was his partner who, when ON was in his early forties, made him aware that he might have “face blindness”. ON did not know that face blindness, or prosopagnosia, was a recognized disorder. However, he remembered thinking about his face identity recognition difficulties from as early as the age of three and he had a lifetime experience of trying to compensate for his difficulties. For instance, he paid attention to cues such as body figures, gait, posture, or voice to identify individuals. His partner also helped him remember people that he could not recognize. He described how he used his appearance (having distinctive clothes and facial hair) and social roles (he had a prominent position in a local voluntary organization) to attract other people’s attention. Thus, by making other people notice and approach him, instead of him having to address them, cues to their identity other than facial features could be revealed. ON reported experiencing embarrassment and shame because of his face identity recognition problems. He recounted several embarrassing episodes involving problems recognizing people. On one occasion, he mistook a mirror image of his face for a stranger staring at him. On another occasion, he failed to recognize his own son when they unexpectedly met at a shopping center. For a long time, he had assumed that his poor face recognition ability was caused by a lack of interest in others.
At the time of assessment, ON had completed 12 years of formal education, equivalent to completing high school in the U.S. He had had several jobs and currently held a managerial position in a small non-governmental organization. ON reported difficulties with short-term memory and trouble remembering instructions or information that did not interest him. However, his inability to recognize people by their faces stood out to him as a specific memory deficit. He did not report reading or spelling difficulties. Nor did he report topographical orientation difficulties, which have a high prevalence in people with prosopagnosia [27,28]. Apart from the tics, a standard neurological examination identified the left hand as dominant. He had normal sensory function, eyesight, visual field, eye movement, and color vision (assessed by Ishihara’s tests for color deficiency [29]). A 1.5 T MRI examination (T1- and T2-weighted images, including fluid-attenuated inversion recovery and diffusion-weighted images) revealed no structural abnormalities of the brain, including the forebrain, cerebellum, and brainstem. A report from an optician indicated only mild hyperopia. An in-depth psychiatric assessment did not reveal any additional psychiatric conditions.

2.2. Neuropsychological Assessment

A thorough neuropsychological assessment, informed by DP assessment guidelines [3,26] and previous studies of DP (e.g., [28,30,31]), was conducted by a neuropsychologist in training (the first author) under the supervision of an experienced board-certified neuropsychologist. Scores on commercially available clinical assessment tools were compared to norms from official manuals. For non-commercial assessment tools normative samples from the literature, described in more detail below, were used. Official Norwegian translations of assessment measures were used where available.
General cognitive ability, including verbal comprehension, perceptual reasoning, working memory, and processing speed, were assessed using the Wechsler Adult Intelligence Scale—Fourth Edition (WAIS-IV) [32]. Low-level visual perception was assessed using the Stars Cancellation Test [33], six subtests from the Birmingham Object Recognition Battery (BORB) [34] and all subtests of the Visual Object and Space Perception Battery (VOSP) [35]. Visuospatial memory was assessed using the Brief Visuospatial Memory Test—Revised (BVMT-R) [36], the Continuous Visual Memory Test (CVMT) [37], and the Spatial Span subtest from the Wechsler Memory Scale—Third Edition (WMS-III) [38]. Verbal memory was assessed using the California Verbal Learning Test—Second Edition (CVLT-II) [39] and the Logical Memory subtests from the WMS-III [38]. Executive and attentional control functions were assessed using the Conners Continuous Performance Test—Third Edition (CCPT-3) [40], the Tower Test from the Delis–Kaplan Executive Function System (D-KEFS) [41], and the Behavior Rating Inventory of Executive Function—Adult Version (BRIEF-A) [42]. Social cognitive functioning was assessed using the Social Responsiveness Scale—Second Edition (SRS-2) [43].
Face recognition memory was assessed using the Cambridge Face Memory Test (CFMT) [44] and the Australian CFMT (CFMT-Aus) [45]. CFMT requires learning and recognition of six cropped faces (excluding hairlines) among distractor faces and under different conditions, such as varying viewpoints, levels of lightening, and levels of visual noise [44]. The CFMT-Aus is equivalent to the CFMT in design, but the latter uses Australian faces that are more similar to Northern European faces [45]. For the CFMT, we used data available from Gerlach et al. [46]. These data are from a Scandinavian (Danish) control sample of 61 individuals (40 female), and match ON fairly well in terms of age (mean age = 36.7 years; SD = 9.8; range 16–56) and education (mean education = 16 years; SD = 1.3; range 10–17). Norms for the CFMT-Aus were taken from an Australian sample of 34 young males (whole sex-mixed sample aged 18–32 years) [45].
Because prosopagnosia may affect face memory as well as perception, we used the Cambridge Face Perception Test (CFPT) [47] to assess face perception abilities. It requires subjects to rank a series of six faces for the degree of similarity to a target face in each trial. The six face images have been morphed with other faces to obtain varying degrees of similarity to the target face. The CFPT is thus a test of face perception without demands on memory. Scores are computed by summarizing each face’s deviations from the correct position (e.g., two positions away = two errors) [47]. For the CFPT we applied reference data from Gerlach et al. [46].
We used the self-report questionnaire 20-Item Prosopagnosia Index (PI-20) to assess DP severity [48]. For the PI-20, we used norms from 319 healthy adults aged 18–74 years [48].
The two CFMTs, the CFPT, and the PI-20 are considered leading diagnostic tests with evidence of good psychometric properties [3,7,26,27,49,50].
Within-class [47] object recognition memory was assessed using the Cambridge Car Memory Test (CCMT) [51]. This test is matched in format to the CFMT but uses cars as stimuli instead of faces. The CCMT and the CFMT thus provide a direct comparison between recognition memory for faces versus cars (cars are considered a proxy for object recognition) [52]. For the CCMT we used reference data from Gerlach et al. [46].
Qualitative descriptive labeling of performance test scores follows the American Academy of Clinical Neuropsychology consensus conference statement, which prescribes different labeling depending on whether normative scores are near-normally or non-normally distributed [53]. For questionnaires, scores are labeled exceptionally high (>2 SD above the normative mean), high (>1 SD above the normative mean), or within normal levels (within 1 SD above the normative mean).

2.3. Single Case Analysis

In order to further explore the severity and specificity of ON’s face recognition deficits in relation to the recognition of other visual objects, his scores were compared to the controls’ mean scores on the CFMT, CCMT, and CFPT using single case statistics developed by Crawford and colleagues [54,55]. We tested whether ON’s performance in each individual test was significantly impaired compared to controls using the program Singlims_ES (available here https://homepages.abdn.ac.uk/j.crawford/pages/dept/Single_Case_Effect_Sizes.htm, accessed on 16 November 2024). To determine if his face recognition was selectively impaired, i.e., if there was a dissociation between performance with faces and other objects, we compared performance on the CFMT and CCMT to controls, using the BTSD test for dissociation as implemented in the program DissocBayes_ES (available at the website linked to above). This analysis tests whether the difference in ON’s performance on the two tasks exceeds the difference in performance observed in the control group while taking the correlation between tasks in the control group into account. SPSS Version 29.0.1.0 was used to calculate means and SDs used in the single case analyses.

3. Results

3.1. Neuropsychological Profile

ON’s scores on neuropsychological performance-based tests and self-report questionnaires are shown in Table 1.
Most scores were within normal expectations (for tests with non-normal distributions) or average or higher (for tests with near-normal distributions). ON obtained low average and below average scores on specific tests of verbal working memory under high attentional demands (first trials of list A and B from the CVLT-II), visual perception (VOSP Object decision), visual recognition memory (CVMT Total Score, CCMT), and reaction time and attention (CCPT-3 Hit Reaction Time, Hit Reaction Time SD, and Hit Reaction Time Interstimulus Interval Change). ON reported exceptionally high levels of executive dysfunction on the BRIEF-A. He reported normal social cognitive responses on the SRS-2, which was consistent with the clinical impression that he did not have an autism spectrum disorder. He obtained exceptionally low scores on a test of face perception (CFPT) and on two tests of face memory (CFMT; CFMT-Aus). His responses on the PI-20 qualified for severe DP [48].

3.2. Single-Case Analysis

Comparison of ON’s scores on CFMT, CCMT, and CFPT compared to controls from Gerlach et al. [46] are presented in Table 2.
In sum, the analyses revealed that ON’s performance on the CFMT was significantly impaired (z score = −3.49) compared to controls, while his performance on the CCMT was within the normal range (z score = −0.99). The difference between the two scores was significant (RSDT t value = 1.807, df = 60, p [one-tailed] = 0.038; the correlation between CFMT and CCMT in the control group = 0.074). This indicates a classical dissociation between ON’s performance on the two tests, meaning that his performance on the CFMT was selectively impaired compared to his within-range performance with cars. ONs performance on the CFPT was significantly impaired compared to the control group, indicating that ON’s trouble with faces was not merely a memory problem but also included face perception.

3.3. DP Diagnosis

ON fulfilled the proposed diagnostic criteria for DP [3,26]. He had exceptionally low scores on three tests of face memory and/or perception, and he reported lifelong difficulties with remembering people’s faces. He did not show signs of visual agnosia. He showed only milder and less consistent difficulties with processing non-facial stimuli, relative to his exceptionally low scores on face processing tests. Furthermore, the assessment excluded any conditions known to be associated with face recognition impairments (e.g., autism spectrum disorder or broader, general cognitive impairment). There was no evidence of brain lesions that could explain his face identity recognition difficulties.

4. Discussion

We have demonstrated how face recognition difficulties can be assessed in a clinical context by a combination of commercial and non-commercial assessment tools. The findings are relevant to several ongoing debates in the field.

4.1. Face Recognition and Other Cognitive Functions

There is a longstanding discussion of whether recognition difficulties related to prosopagnosia are specific to faces, or whether they extend to other objects as well (see [52] and accompanying commentaries for an extensive discussion). Of note, while showing good visual perceptual and memory functioning on many measures, ON obtained low average (CVMT, CCMT) and below average (VOSP Object Decision) scores on three tests of visual processing. These low average and below average scores could be expressions of co-occurring and relatively milder visual perceptual or visual memory difficulties. Indeed, varying degrees of general visual processing deficits have been found in other people with DP [27,56]. Furthermore, if difficulties with face recognition are disproportionally larger than object recognition problems or other perceptual problems, a diagnosis of DP still holds [10]. Moreover, there is some evidence of DP sub-groups, where some show a clear dissociation between face and object recognition and others also have object recognition deficits [57]. The single-case analysis with Danish norms revealed a classical dissociation between recognition memory for faces (CFMT score) versus objects (cars, measured by the CCMT), which provides strong evidence that ON’s memory deficit was specifically related to face recognition.
In addition to face processing deficits and possibly mild visual perceptual or visual memory problems, ON showed varying degrees of difficulties in other cognitive domains, including executive functions, attention, reaction time, and verbal working memory under high attentional demands.
Some of ON’s deficits are most likely related to his TS and/or ADHD as impairment in executive functions, attention, working memory, and memory acquisition, which are common in people with TS and ADHD and might relate to frontal and frontostriatal dysfunctions [58,59,60].

4.2. The Importance of Proper Assessment

Prosopagnosia can lead to anxiety, embarrassment, guilt, a restricted social circle, and limited employment opportunities [61]. Indeed, ON confirmed these experiences. ON reported that learning that DP is a recognized condition made it easier for him to communicate his challenges in social interaction to others. He was also relieved to learn that his cognitive processing deficits were relatively specific to faces and expressed that he could now be more accepting of his shortcomings in facial recognition abilities. Furthermore, he reported not having realized that he had face recognition difficulties before late into adulthood. This has also been reported by other people with DP [62]. Even though many report a positive effect of disclosing their DP, most adults are reluctant to do so, fearing misunderstanding and stigma [63].
One goal of clinical neuropsychology should be testing predictions of different hypotheses (e.g., DP versus other perceptual or memory problems) that may be clinically useful to patients. For years, ON believed that his inability to remember faces was a problem of low interest in other people, that is, a problem of motivation rather than ability. Proper identification, including valid measures of face recognition abilities, could be a first step towards helping people with DP develop awareness and acceptance of their difficulties. Furthermore, proper assessment of face recognition abilities along with general neuropsychological functioning is a prerequisite for designing clinical interventions.

5. Conclusions

We have documented severe impairment in face identity recognition in an individual with TS and ADHD, based on thorough neuropsychological assessment using both clinical and experimental tests. While face recognition problems have been described in people with various other neurodevelopmental impairments, this is to our knowledge, the first description of a co-occurring DP, TS, and ADHD.
Case studies do of course have limited generalizability as to the specific findings, but the clinical approach used here can be generalized and applied to similar cases. When face processing deficits are suspected, we encourage clinicians to employ a sufficiently broad spectrum of tools to assess face recognition difficulties as well as other functions relevant to understanding patients’ difficulties in everyday life. While case studies are well suited to describe specific symptomatology in depth, broader population studies are needed to increase our understanding of the co-occurrence of face recognition impairment and other neurodevelopmental and psychiatric conditions more generally. Such studies may also contribute to the further development of assessment procedures.
Interestingly, ON’s account of several individuals with tics and ADHD traits on his father’s side and prosopagnosic traits on his mother’s side, point to distinct genetic origins in his case. Systematic questions about family history might thus provide clues to whether neurodevelopmental co-morbidity is caused by one or more genetic vulnerability factors.

Supplementary Materials

The following supporting information can be downloaded at: https://www.mdpi.com/article/10.3390/brainsci15010056/s1, Table S1: Primary scores on tests and questionnaires that were not included in the main article. References [64,65,66,67,68] are cited in the Supplementary Materials.

Author Contributions

All authors made substantial contributions to the work reported. Conceptualization, P.E.E., R.S., R.R. and B.H.; methodology, P.E.E., R.S., R.R. and B.H.; formal analysis, P.E.E. and R.S.; investigation, P.E.E., B.H. and R.S.; writing—original draft preparation, P.E.E. and B.H.; writing—review and editing, P.E.E., R.S., R.R. and B.H.; visualization, P.E.E. and R.S. All authors have read and agreed to the published version of the manuscript.

Funding

This research received no external funding.

Institutional Review Board Statement

The control data from Gerlach et al. [46] are freely available. These controls were tested under the approval of The Regional Committee for Health Research Ethics of Southern Denmark (Project-ID: S-20150134, approval date: 4 November 2015) as part of a larger study of DP.

Informed Consent Statement

Written informed consent has been obtained from the patient to publish this paper. The patient has read and approved the final manuscript.

Data Availability Statement

The original contributions presented in this study are included in the article/Supplementary Material. Data availability is restricted due to patient confidentiality.

Acknowledgments

We thank Brad Duchaine (Dartmoth College, Hanover, NH, 03755) for providing the Cambridge Face Perception Test, the Cambridge Face Memory Test—Original and Cambridge Car Memory Test. We further thank researchers at the Australian National University, Canberra, ACT, 2601, for sharing the Cambridge Face Memory Test—Australian and the objective test of pre-experimental car expertise (Supplementary Materials).

Conflicts of Interest

The authors declare no conflicts of interest.

References

  1. Starrfelt, R.; Barton, J.J.S. Prosopagnosia. In Encyclopedia of Behavioral Neuroscience, 2nd ed.; Della Sala, S., Ed.; Elsevier: Oxford, UK, 2022; pp. 597–604. [Google Scholar]
  2. Barton, J.J. Structure and function in acquired prosopagnosia: Lessons from a series of 10 patients with brain damage. J. Neuropsychol. 2008, 2, 197–225. [Google Scholar] [CrossRef] [PubMed]
  3. Dalrymple, K.A.; Palermo, R. Guidelines for studying developmental prosopagnosia in adults and children. Wiley Interdiscip. Rev. Cogn. Sci. 2016, 7, 73–87. [Google Scholar] [CrossRef]
  4. Volfart, A.; Rossion, B. The neuropsychological evaluation of face identity recognition. Neuropsychologia 2024, 198, 108865. [Google Scholar] [CrossRef]
  5. Kennerknecht, I.; Grueter, T.; Welling, B.; Wentzek, S.; Horst, J.; Edwards, S.; Grueter, M. First report of prevalence of non-syndromic hereditary prosopagnosia (HPA). Am. J. Med. Genet. A 2006, 140, 1617–1622. [Google Scholar] [CrossRef] [PubMed]
  6. Kennerknecht, I.; Ho, N.Y.; Wong, V.C. Prevalence of hereditary prosopagnosia (HPA) in Hong Kong Chinese population. Am. J. Med. Genet. A 2008, 146A, 2863–2870. [Google Scholar] [CrossRef]
  7. Bowles, D.C.; McKone, E.; Dawel, A.; Duchaine, B.; Palermo, R.; Schmalzl, L.; Rivolta, D.; Wilson, C.E.; Yovel, G. Diagnosing prosopagnosia: Effects of ageing, sex, and participant-stimulus ethnic match on the Cambridge Face Memory Test and Cambridge Face Perception Test. Cogn. Neuropsychol. 2009, 26, 423–455. [Google Scholar] [CrossRef]
  8. Barton, J.J.S.; Corrow, S.L. The problem of being bad at faces. Neuropsychologia 2016, 89, 119–124. [Google Scholar] [CrossRef]
  9. DeGutis, J.; Bahierathan, K.; Barahona, K.; Lee, E.; Evans, T.C.; Shin, H.M.; Mishra, M.; Likitlersuang, J.; Wilmer, J.B. What is the prevalence of developmental prosopagnosia? An empirical assessment of different diagnostic cutoffs. Cortex 2023, 161, 51–64. [Google Scholar] [CrossRef] [PubMed]
  10. Gerlach, C.; Nørkær, E.; Starrfelt, R.; Class, A.; Class, B. Is that the only chemistry?: A commentary on DeGutis et al. (2023): What is the prevalence of developmental prosopagnosia? An empirical assessment of different diagnostic cutoffs. Cortex 2024, 117, 385–388. [Google Scholar] [CrossRef]
  11. Burns, E.J. Improving the DSM-5 approach to cognitive impairment: Developmental prosopagnosia reveals the need for tailored diagnoses. Behav. Res. Methods 2024, 56, 7872–7891. [Google Scholar] [CrossRef] [PubMed]
  12. Minio-Paluello, I.; Porciello, G.; Pascual-Leone, A.; Baron-Cohen, S. Face individual identity recognition: A potential endophenotype in autism. Mol. Autism 2020, 11, 81. [Google Scholar] [CrossRef]
  13. Shah, P.; Bird, G.; Cook, R. Face processing in autism: Reduced integration of cross-feature dynamics. Cortex 2016, 75, 113–119. [Google Scholar] [CrossRef]
  14. Stantić, M.; Ichijo, E.; Catmur, C.; Bird, G. Face memory and face perception in autism. Autism 2022, 26, 276–280. [Google Scholar] [CrossRef] [PubMed]
  15. Wilson, C.E.; Freeman, P.; Brock, J.; Burton, A.M.; Palermo, R. Facial identity recognition in the broader autism phenotype. PLoS ONE 2010, 5, e12876. [Google Scholar] [CrossRef] [PubMed]
  16. Chen, Y.; Ekstrom, T. Perception of faces in schizophrenia: Subjective (self-report) vs. objective (psychophysics) assessments. J. Psychiatr. Res. 2016, 76, 136–142. [Google Scholar] [CrossRef] [PubMed]
  17. Ekstrom, T.; Maher, S.; Shinn, A.; Ongur, D.; Chen, Y. Face identity discrimination in schizophrenia: Impairments to faces with high exposure in society. Schizophr. Res. 2016, 171, 237–238. [Google Scholar] [CrossRef]
  18. Kühn, C.D.; Gerlach, C.; Andersen, K.B.; Poulsen, M.; Starrfelt, R. Face recognition in developmental dyslexia: Evidence for dissociation between faces and words. Cogn. Neuropsychol. 2021, 38, 107–115. [Google Scholar] [CrossRef] [PubMed]
  19. Robotham, R.J.; Starrfelt, R. Face and Word Recognition Can Be Selectively Affected by Brain Injury or Developmental Disorders. Front. Psychol. 2017, 8, 1547. [Google Scholar] [CrossRef] [PubMed]
  20. Sigurdardottir, H.M.; Ívarsson, E.; Kristinsdóttir, K.; Kristjánsson, Á. Impaired recognition of faces and objects in dyslexia: Evidence for ventral stream dysfunction? Neuropsychology 2015, 29, 739–750. [Google Scholar] [CrossRef]
  21. Demirci, E.; Erdogan, A. Is emotion recognition the only problem in ADHD? effects of pharmacotherapy on face and emotion recognition in children with ADHD. Atten. Defic. Hyperact. Disord. 2016, 8, 197–204. [Google Scholar] [CrossRef] [PubMed]
  22. Romani, M.; Vigliante, M.; Faedda, N.; Rossetti, S.; Pezzuti, L.; Guidetti, V.; Cardona, F. Face memory and face recognition in children and adolescents with attention deficit hyperactivity disorder: A systematic review. Neurosci. Biobehav. Rev. 2018, 89, 1–12. [Google Scholar] [CrossRef] [PubMed]
  23. Hong, D.; Scaletta Kent, J.; Kesler, S. Cognitive profile of Turner syndrome. Dev. Disabil. Res. Rev. 2009, 15, 270–278. [Google Scholar] [CrossRef] [PubMed]
  24. Bate, S.; Cook, S.J.; Mole, J.; Cole, J. First report of generalized face processing difficulties in möbius sequence. PLoS ONE 2013, 8, e62656. [Google Scholar] [CrossRef] [PubMed]
  25. Svart, N.; Starrfelt, R. Is It Just Face Blindness? Exploring Developmental Comorbidity in Individuals with Self-Reported Developmental Prosopagnosia. Brain Sci. 2022, 12, 230. [Google Scholar] [CrossRef] [PubMed]
  26. Corrow, S.L.; Dalrymple, K.A.; Barton, J.J. Prosopagnosia: Current perspectives. Eye Brain 2016, 8, 165–175. [Google Scholar] [CrossRef] [PubMed]
  27. Barton, J.J.S.; Albonico, A.; Susilo, T.; Duchaine, B.; Corrow, S.L. Object recognition in acquired and developmental prosopagnosia. Cogn. Neuropsychol. 2019, 36, 54–84. [Google Scholar] [CrossRef] [PubMed]
  28. Bate, S.; Adams, A.; Bennetts, R.; Line, H. Developmental prosopagnosia with concurrent topographical difficulties: A case report and virtual reality training programme. Neuropsychol. Rehabil. 2019, 29, 1290–1312. [Google Scholar] [CrossRef]
  29. Ishihara, S. Ishihara’s Tests for Colour Deficiency; Kanehara & Co.: Tokyo, Japan, 1996. [Google Scholar]
  30. Anaki, D.; Kaufman, Y.; Freedman, M.; Moscovitch, M. Associative (prosop)agnosia without (apparent) perceptual deficits: A case-study. Neuropsychologia 2007, 45, 1658–1671. [Google Scholar] [CrossRef] [PubMed]
  31. Schultz, R.R.; Bertolucci, P.H.F. Congenital prosopagnosia: A case report. Dement. Neuropsychol. 2011, 5, 54–57. [Google Scholar] [CrossRef]
  32. Wechsler, D. Wechsler Adult Intelligence Scale–Fourth Edition (WAIS–IV). In Norwegian Version; Pearson: Stockholm, Sweden, 2011. [Google Scholar]
  33. Wilson, B.; Cockburn, J.; Halligan, P. Development of a behavioral test of visuospatial neglect. Arch. Phys. Med. Rehabil. 1987, 68, 98–102. [Google Scholar] [PubMed]
  34. Riddoch, M.J.; Humphreys, G.W. Birmingham Object Recognition Battery; Ruthledge: New York, NY, USA, 1993. [Google Scholar]
  35. Warrington, E.; James, M. The Visual Object and Space Perception Battery; Pearson: London, UK, 1991. [Google Scholar]
  36. Benedict, R. Brief Visuospatial Memory Test—Revised: Professional Manual; Psychological Assessment Resources: Lutz, FL, USA, 1997. [Google Scholar]
  37. Trahan, D.; Larrabee, G. Continuous Visual Memory Test; Psychological Assessment Resources: Odessa, FL, USA, 1988. [Google Scholar]
  38. Wechsler, D. Wechsler Memory Scale-Third Edition (WMS-III); Psychological Corporation: San Antonio, TX, USA, 1997. [Google Scholar]
  39. Delis, D.; Kramer, J.; Kaplan, E.; Ober, B.A. California Verbal Learning Test—Second Edition: Adult Version; Psychological Corporation: San Antonio, TX, USA, 2000. [Google Scholar]
  40. Conners. Conners’ CPT 3: Manual; Multi-Health Systems: North Tonawanda, NY, USA, 2014. [Google Scholar]
  41. Delis, D.; Kaplan, E.; Kramer, J. Delis-Kaplan Executive Function System (D-KEFS); Psychological Corporation: San Antonio, TX, USA, 2001. [Google Scholar]
  42. Roth, R.M.; Gioia, G.A. Behavior Rating Inventory of Executive Function-Adult Version; Psychological Assessment Resources: Lutz, FL, USA, 2005. [Google Scholar]
  43. Constantino, J.N.; Gruber, C.P. Social Responsiveness Scale-2 (SRS-2). Norwegian Version; Hogrefe: Stockholm, Sweden, 2019. [Google Scholar]
  44. Duchaine, B.; Nakayama, K. The Cambridge Face Memory Test: Results for neurologically intact individuals and an investigation of its validity using inverted face stimuli and prosopagnosic participants. Neuropsychologia 2006, 44, 576–585. [Google Scholar] [CrossRef]
  45. McKone, E.; Hall, A.; Pidcock, M.; Palermo, R.; Wilkinson, R.B.; Rivolta, D.; Yovel, G.; Davis, J.M.; O’Connor, K.B. Face ethnicity and measurement reliability affect face recognition performance in developmental prosopagnosia: Evidence from the Cambridge Face Memory Test–Australian. Cogn. Neuropsychol. 2011, 28, 109–146. [Google Scholar] [CrossRef]
  46. Gerlach, C.; Barton, J.J.S.; Albonico, A.; Malaspina, M.; Starrfelt, R. Contrasting domain-general and domain-specific accounts in cognitive neuropsychology: An outline of a new approach with developmental prosopagnosia as a case. Behav. Res. Methods 2022, 54, 2829–2842. [Google Scholar] [CrossRef] [PubMed]
  47. Duchaine, B.; Germine, L.; Nakayama, K. Family resemblance: Ten family members with prosopagnosia and within-class object agnosia. Cogn. Neuropsychol. 2007, 24, 419–430. [Google Scholar] [CrossRef]
  48. Shah, P.; Gaule, A.; Sowden, S.; Bird, G.; Cook, R. The 20-item prosopagnosia index (PI20): A self-report instrument for identifying developmental prosopagnosia. R. Soc. Open Sci. 2015, 2, 140343. [Google Scholar] [CrossRef]
  49. Gray, K.L.; Bird, G.; Cook, R. Robust associations between the 20-item prosopagnosia index and the Cambridge Face Memory Test in the general population. R. Soc. Open Sci. 2017, 4, 160923. [Google Scholar] [CrossRef] [PubMed]
  50. Mishra, M.V.; Fry, R.M.; Saad, E.; Arizpe, J.M.; Ohashi, Y.-G.B.; DeGutis, J.M. Comparing the sensitivity of face matching assessments to detect face perception impairments. Neuropsychologia 2021, 163, 108067. [Google Scholar] [CrossRef] [PubMed]
  51. Dennett, H.W.; McKone, E.; Tavashmi, R.; Hall, A.; Pidcock, M.; Edwards, M.; Duchaine, B. The Cambridge Car Memory Test: A task matched in format to the Cambridge Face Memory Test, with norms, reliability, sex differences, dissociations from face memory, and expertise effects. Behav. Res. Methods 2012, 44, 587–605. [Google Scholar] [CrossRef] [PubMed]
  52. Geskin, J.; Behrmann, M. Congenital prosopagnosia without object agnosia? A literature review. Cogn. Neuropsychol. 2018, 35, 4–54. [Google Scholar] [CrossRef]
  53. Guilmette, T.J.; Sweet, J.J.; Hebben, N.; Koltai, D.; Mahone, E.M.; Spiegler, B.J.; Stucky, K.; Westerveld, M.; Conference, P. American Academy of Clinical Neuropsychology consensus conference statement on uniform labeling of performance test scores. Clin. Neuropsychol. 2020, 34, 437–453. [Google Scholar] [CrossRef] [PubMed]
  54. Crawford, J.R.; Garthwaite, P.H. Comparison of a single case to a control or normative sample in neuropsychology: Development of a Bayesian approach. Cogn. Neuropsychol. 2007, 24, 343–372. [Google Scholar] [CrossRef]
  55. Crawford, J.R.; Garthwaite, P.H.; Porter, S. Point and interval estimates of effect sizes for the case-controls design in neuropsychology: Rationale, methods, implementations, and proposed reporting standards. Cogn. Neuropsychol. 2010, 27, 245–260. [Google Scholar] [CrossRef] [PubMed]
  56. Gray, K.L.H.; Biotti, F.; Cook, R. Evaluating object recognition ability in developmental prosopagnosia using the Cambridge Car Memory Test. Cogn. Neuropsychol. 2019, 36, 89–96. [Google Scholar] [CrossRef] [PubMed]
  57. Gerlach, C.; Starrfelt, R. Evidence for a Classical Dissociation between Face and Object Recognition in Developmental Prosopagnosia. Brain Sci. 2024, 14, 107. [Google Scholar] [CrossRef]
  58. Nikolas, M.A.; Marshall, P.; Hoelzle, J.B. The role of neurocognitive tests in the assessment of adult attention-deficit/hyperactivity disorder. Psychol. Assess. 2019, 31, 685–698. [Google Scholar] [CrossRef] [PubMed]
  59. Morand-Beaulieu, S.; Leclerc, J.B.; Valois, P.; Lavoie, M.E.; O’Connor, K.P.; Gauthier, B. A Review of the Neuropsychological Dimensions of Tourette Syndrome. Brain Sci. 2017, 7, 106. [Google Scholar] [CrossRef] [PubMed]
  60. Andersen, P.N.; Egeland, J.; Oie, M. Learning and memory impairments in children and adolescents with attention-deficit/hyperactivity disorder. J. Learn. Disabil. 2013, 46, 453–460. [Google Scholar] [CrossRef]
  61. Yardley, L.; McDermott, L.; Pisarski, S.; Duchaine, B.; Nakayama, K. Psychosocial consequences of developmental prosopagnosia: A problem of recognition. J. Psychosom. Res. 2008, 65, 445–451. [Google Scholar] [CrossRef]
  62. Murray, E.; Hills, P.J.; Bennetts, R.J.; Bate, S. Identifying Hallmark Symptoms of Developmental Prosopagnosia for Non-Experts. Sci. Rep. 2018, 8, 1690. [Google Scholar] [CrossRef]
  63. Adams, A.; Hills, P.J.; Bennetts, R.J.; Bate, S. Coping strategies for developmental prosopagnosia. Neuropsychol. Rehabil. 2020, 30, 1996–2015. [Google Scholar] [CrossRef] [PubMed]
  64. Baron-Cohen, S.; Wheelwright, S.; Hill, J.; Raste, Y.; Plumb, I. The “Reading the Mind in the Eyes” Test revised version: A study with normal adults, and adults with Asperger syndrome or high-functioning autism. J. Child Psychol. Psychiatry 2001, 42, 241–251. [Google Scholar] [CrossRef] [PubMed]
  65. Higgins, W.C.; Ross, R.M.; Langdon, R.; Polito, V. The “Reading the Mind in the Eyes” Test Shows Poor Psychometric Properties in a Large, Demographically Representative U.S. Sample. Assessment 2023, 30, 1777–1789. [Google Scholar] [CrossRef]
  66. Leckman, J.F.; Riddle, M.A.; Hardin, M.T.; Ort, S.I.; Swartz, K.L.; Stevenson, J.; Cohen, D.J. The Yale Global Tic Severity Scale: Initial testing of a clinician-rated scale of tic severity. J. Am. Acad. Child Adolesc. Psychiatry 1989, 28, 566–573. [Google Scholar] [CrossRef] [PubMed]
  67. Kessler, R.C.; Adler, L.; Ames, M.; Demler, O.; Faraone, S.; Hiripi, E.; Howes, M.J.; Jin, R.; Secnik, K.; Spencer, T.; et al. The World Health Organization Adult ADHD Self-Report Scale (ASRS): A short screening scale for use in the general population. Psychol. Med. 2005, 35, 245–256. [Google Scholar] [CrossRef] [PubMed]
  68. Ward, M.F.; Wender, P.H.; Reimherr, F.W. The Wender Utah Rating Scale: An aid in the retrospective diagnosis of childhood attention deficit hyperactivity disorder. Am. J. Psychiatry 1993, 150, 885–890. [Google Scholar] [CrossRef] [PubMed]
Table 1. ON’s neuropsychological profile.
Table 1. ON’s neuropsychological profile.
MeasureRaw ScoreNorm-Based ScoreQualitative Descriptive Label
General cognitive ability
 WAIS-IV
  Fullscale IQ 111 aHigh average
  Verbal Comprehension Index 102 aAverage
  Perceptual Reasoning Index 120 aAbove average
  Processing Speed Index 112 aHigh average
  Working Memory Index 105 aAverage
Visual perception
 BORB
  Length match291.3 bWithin normal expectations
  Size match27−0.1 bWithin normal expectations
  Orientation match260.5 bWithin normal expectations
  Position of gap match34−0.3 bWithin normal expectations
  Picture Naming (animate)151 bWithin normal expectations
  Picture Naming (inanimate)200.6 bWithin normal expectations
 VOSP
  Object perception
    Screening test 200.2 bWithin normal expectations
    Incomplete letters 200.9 bWithin normal expectations
    Silhouettes 260.7 bWithin normal expectations
    Object decision 16−1.6 bBelow average
    Progressive silhouettes 90.3 bWithin normal expectations
  Space perception
    Dot counting 100.3 bWithin normal expectations
    Position discrimination 200.4 bWithin normal expectations
    Number location 100.6 bWithin normal expectations
    Cube analysis 9−0.3 bWithin normal expectations
 Star Cancellation Test54/
54		--
Memory
 BVMT-R
  Trial 1860 cHigh average
  Total Recall 3061 cHigh average
  Delayed Recall1159 cHigh average
  Recognition Discrimination6>16%ileWithin normal expectations
  Index
 CVMT
  Total Score75−0.8 bLow average
  Delayed Recognition4−0.5 bAverage
  Visual discrimination7/7−-
 CVLT-II
  Trial 15−1 bLow average
  List B trial 1 4−1 bLow average
  Trials 1–5 Total Score4652 cAverage
  Short Delay Free Recall100.5 bAverage
  Long Delay Free Recall100 bAverage
  Recognition Hits140 bAverage
  Recognition False Positives4−0.5 bAverage
  Forced Recognition16/
16		--
 WMS-III
    Logical Memory I—Recall4812 dHigh average
    Logical Memory II
     Recall3012 dHigh average
     Recognition27/
30		--
  Spatial Span1914 dAbove average
 CCMT43−1 bLow average
Executive and attentional control
 BRIEF-A Self Report Form
  Behavioral Regulation Index 76 cExceptionally high
  Metacognitive Index 81 cExceptionally high
  Global Executive Composite 81 cExceptionally high
 CCPT-3
  d′-57 cHigh average
  Omissions-55 cAverage
  Commissions-53 cAverage
  Perseverations-55 cAverage
  Hit Reaction Time-39 cLow average
  Hit Reaction Time SD-39 cLow average
  Variability-44 cAverage
  Hit Reaction Time Block-43 cAverage
  Change
  Hit Reaction Time-32 cBelow average
  Interstimulus Interval Change
 D-KEFS Tower Test Total1811 dAverage
 Achievement
Social cognition
  SRS-2 Self Report Form5450 cWithin normal levels
Face processing
  CFPT66−2.1 bExceptionally low
  CFMT34−3.5 bExceptionally low
  CFMT-Aus25−4 bExceptionally low
  PI-20914.8 bExceptionally high
Note. The following norm-based scores were applied: a IQ or index scores (M = 100, SD = 15), b z scores (M = 0, SD = 1), c T scores (M = 50, SD = 10), d scaled scores (M = 10, SD = 3), and percentile ranks. Norm-based scores for performance-based tests were adjusted so that lower scores indicate lower performance and higher scores indicate higher performance. For questionnaires, higher scores indicate higher symptom levels. BORB = Birmingham Object Recognition Battery; BRIEF-A = Behavior Rating Inventory of Executive Function—adult version; BVMT-R = Brief Visuospatial Memory Test—Revised; CCMT = Cambridge Car Memory Test; CFMT = Cambridge Face Memory Test; CFMT-Aus = Cambridge Face Memory Test—Australian; CFPT = Cambridge Face Perception Test; CCPT-3 = Conners Continuous Performance Test—third edition; CVLT-II = California Verbal Learning Test—second edition; CVMT = Continuous Visual Memory Test; D-KEFS = Delis–Kaplan Executive Function System; PI-20 = 20-Item Prosopagnosia Index; SRS = Social Responsiveness Scale—second edition; WAIS-IV = Wechsler Adult Intelligence Scale—fourth edition; WMS-III = Wechsler Memory Scale—third edition.
Table 2. Scores for controls (N = 61) from [46] and ON.
Table 2. Scores for controls (N = 61) from [46] and ON.
Controls
Min		Controls
Max		Controls
Mean		Controls
SD		ONON
z Score		ON
z Score CI		Two-Tailed p
CFMT457259.027.1734−3.49(−4.16–-2.82)0.001
CCMT316952.059.1643−0.99(−1.29–-0.68)0.331
CFPT129239.0813.10662.06(1.61–2.5)0.046
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Emhjellen, P.E.; Starrfelt, R.; Raudeberg, R.; Hassel, B. Assessment of Developmental Prosopagnosia in an Individual with Tourette Syndrome and Attention Deficit Hyperactivity Disorder: A Case Report. Brain Sci. 2025, 15, 56. https://doi.org/10.3390/brainsci15010056

AMA Style

Emhjellen PE, Starrfelt R, Raudeberg R, Hassel B. Assessment of Developmental Prosopagnosia in an Individual with Tourette Syndrome and Attention Deficit Hyperactivity Disorder: A Case Report. Brain Sciences. 2025; 15(1):56. https://doi.org/10.3390/brainsci15010056

Chicago/Turabian Style

Emhjellen, Petter Espeseth, Randi Starrfelt, Rune Raudeberg, and Bjørnar Hassel. 2025. "Assessment of Developmental Prosopagnosia in an Individual with Tourette Syndrome and Attention Deficit Hyperactivity Disorder: A Case Report" Brain Sciences 15, no. 1: 56. https://doi.org/10.3390/brainsci15010056

APA Style

Emhjellen, P. E., Starrfelt, R., Raudeberg, R., & Hassel, B. (2025). Assessment of Developmental Prosopagnosia in an Individual with Tourette Syndrome and Attention Deficit Hyperactivity Disorder: A Case Report. Brain Sciences, 15(1), 56. https://doi.org/10.3390/brainsci15010056

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