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Neuropsychologia Jun 2024Facial identity recognition (FIR) is arguably the ultimate form of recognition for the adult human brain. Even if the term prosopagnosia is reserved for exceptionally... (Review)
Review
Facial identity recognition (FIR) is arguably the ultimate form of recognition for the adult human brain. Even if the term prosopagnosia is reserved for exceptionally rare brain-damaged cases with a category-specific abrupt loss of FIR at adulthood, subjective and objective impairments or difficulties of FIR are common in the neuropsychological population. Here we provide a critical overview of the evaluation of FIR both for clinicians and researchers in neuropsychology. FIR impairments occur following many causes that should be identified objectively by both general and specific, behavioral and neural examinations. We refute the commonly used dissociation between perceptual and memory deficits/tests for FIR, since even a task involving the discrimination of unfamiliar face images presented side-by-side relies on cortical memories of faces in the right-lateralized ventral occipito-temporal cortex. Another frequently encountered confusion is between specific deficits of the FIR function and a more general impairment of semantic memory (of people), the latter being most often encountered following anterior temporal lobe damage. Many computerized tests aimed at evaluating FIR have appeared over the last two decades, as reviewed here. However, despite undeniable strengths, they often suffer from ecological limitations, difficulties of instruction, as well as a lack of consideration for processing speed and qualitative information. Taking into account these issues, a recently developed behavioral test with natural images manipulating face familiarity, stimulus inversion, and correct response times as a key variable appears promising. The measurement of electroencephalographic (EEG) activity in the frequency domain from fast periodic visual stimulation also appears as a particularly promising tool to complete and enhance the neuropsychological assessment of FIR.
Topics: Humans; Facial Recognition; Neuropsychological Tests; Prosopagnosia; Recognition, Psychology; Electroencephalography
PubMed: 38522782
DOI: 10.1016/j.neuropsychologia.2024.108865 -
Scientific Reports Mar 2024Congenital Prosopagnosia (CP) is an innate impairment in face perception with heterogeneous characteristics. It is still unclear if and to what degree holistic...
Congenital Prosopagnosia (CP) is an innate impairment in face perception with heterogeneous characteristics. It is still unclear if and to what degree holistic processing of faces is disrupted in CP. Such disruption would be expected to lead to a focus on local features of the face. In this study, we used binocular rivalry (BR) to implicitly measure face perception in conditions that favour holistic or local processing. The underlying assumption is that if stimulus saliency affects the perceptual dominance of a given stimulus in BR, one can deduce how salient a stimulus is for a given group (here: participants with and without CP) based on the measured perceptual dominance. A further open question is whether the deficit in face processing in CP extends to the processing of the facial display of emotions. In experiment 1, we compared predominance of upright and inverted faces displaying different emotions (fearful, happy, neutral) vs. houses between participants with CP (N = 21) and with normal face perception (N = 21). The results suggest that CP observers process emotions in faces automatically but rely more on local features than controls. The inversion of faces, which is supposed to disturb holistic processing, affected controls in a more pronounced way than participants with CP. In experiment 2, we introduced the Thatcher effect in BR by inverting the eye and mouth regions of the presented faces in the hope of further increasing the effect of face inversion. However, our expectations were not borne out by the results. Critically, both experiments showed that inversion effects were more pronounced in controls than in CP, suggesting that holistic face processing is less relevant in CP. We find BR to be a useful implicit test for assessing visual processing specificities in neurological participants.
Topics: Humans; Facial Recognition; Prosopagnosia; Pattern Recognition, Visual; Visual Perception; Photic Stimulation
PubMed: 38509151
DOI: 10.1038/s41598-024-55023-7 -
Scientific Reports Mar 2024Developmental prosopagnosia (DP) is characterised by deficits in face identification. However, there is debate about whether these deficits are primarily perceptual, and...
Developmental prosopagnosia (DP) is characterised by deficits in face identification. However, there is debate about whether these deficits are primarily perceptual, and whether they extend to other face processing tasks (e.g., identifying emotion, age, and gender; detecting faces in scenes). In this study, 30 participants with DP and 75 controls completed a battery of eight tasks assessing four domains of face perception (identity; emotion; age and gender; face detection). The DP group performed worse than the control group on both identity perception tasks, and one task from each other domain. Both identity perception tests uniquely predicted DP/control group membership, and performance on two measures of face memory. These findings suggest that deficits in DP may arise from issues with face perception. Some non-identity tasks also predicted DP/control group membership and face memory, even when face identity perception was accounted for. Gender perception and speed of face detection consistently predicted unique variance in group membership and face memory; several other tasks were only associated with some measures of face recognition ability. These findings indicate that face perception deficits in DP may extend beyond identity perception. However, the associations between tasks may also reflect subtle aspects of task demands or stimuli.
Topics: Humans; Facial Recognition; Prosopagnosia; Emotions; Pattern Recognition, Visual
PubMed: 38503841
DOI: 10.1038/s41598-024-57176-x -
Cortex; a Journal Devoted To the Study... Apr 2024Developmental prosopagnosia (DP) is characterised by difficulties recognising face identities and is associated with diverse co-occurring object recognition...
Developmental prosopagnosia (DP) is characterised by difficulties recognising face identities and is associated with diverse co-occurring object recognition difficulties. The high co-occurrence rate and heterogeneity of associated difficulties in DP is an intrinsic feature of developmental conditions, where co-occurrence of difficulties is the rule, rather than the exception. However, despite its name, cognitive and neural theories of DP rarely consider the developmental context in which these difficulties occur. This leaves a large gap in our understanding of how DP emerges in light of the developmental trajectory of face recognition. Here, we argue that progress in the field requires re-considering the developmental origins of differences in face recognition abilities, rather than studying the end-state alone. In practice, considering development in DP necessitates a re-evaluation of current approaches in recruitment, design, and analyses.
Topics: Humans; Prosopagnosia; Facial Recognition; Visual Perception; Pattern Recognition, Visual
PubMed: 38460488
DOI: 10.1016/j.cortex.2024.02.006 -
Brain Communications 2024Loss of facial recognition or prosopagnosia has been well-recognized for over a century. It has been categorized as developmental or acquired depending on whether the...
Loss of facial recognition or prosopagnosia has been well-recognized for over a century. It has been categorized as developmental or acquired depending on whether the onset is in early childhood or beyond, and acquired cases can have degenerative or non-degenerative aetiologies. Prosopagnosia has been linked to involvement of the fusiform gyri, mainly in the right hemisphere. The literature on prosopagnosia comprises case reports and small case series. We aim to assess demographic, clinical and imaging characteristics and neurological and neuropathological disorders associated with a diagnosis of prosopagnosia in a large cohort. Patients were categorized as developmental versus acquired; those with acquired prosopagnosia were further subdivided into degenerative versus non-degenerative, based on neurological aetiology. We assessed regional involvement on [F] fluorodeoxyglucose-PET and MRI of the right and left frontal, temporal, parietal and occipital lobes. The Intake and Referral Center at the Mayo Clinic identified 487 patients with possible prosopagnosia, of which 336 met study criteria for probable or definite prosopagnosia. Ten patients, 80.0% male, had developmental prosopagnosia including one with Niemann-Pick type C and another with a forkhead box G1 gene mutation. Of the 326 with acquired prosopagnosia, 235 (72.1%) were categorized as degenerative, 91 (27.9%) as non-degenerative. The most common degenerative diagnoses were posterior cortical atrophy, primary prosopagnosia syndrome, Alzheimer's disease dementia and semantic dementia, with each diagnosis accounting for >10% of this group. The most common non-degenerative diagnoses were infarcts (ischaemic and haemorrhagic), epilepsy-related and primary brain tumours, each accounting for >10%. We identified a group of patients with non-degenerative transient prosopagnosia in which facial recognition loss improved or resolved over time. These patients had migraine-related prosopagnosia, posterior reversible encephalopathy syndrome, delirium, hypoxic encephalopathy and ischaemic infarcts. On [F] fluorodeoxyglucose-PET, the temporal lobes proved to be the most frequently affected regions in 117 patients with degenerative prosopagnosia, while in 82 patients with non-degenerative prosopagnosia, MRI revealed the right temporal and right occipital lobes as most affected by a focal lesion. The most common pathological findings in those with degenerative prosopagnosia were frontotemporal lobar degeneration with hippocampal sclerosis and mixed Alzheimer's and Lewy body disease pathology. In this large case series of patients diagnosed with prosopagnosia, we observed that facial recognition loss occurs across a wide range of acquired degenerative and non-degenerative neurological disorders, most commonly in males with developmental prosopagnosia. The right temporal and occipital lobes, and connecting fusiform gyrus, are key areas. Multiple different pathologies cause degenerative prosopagnosia.
PubMed: 38419734
DOI: 10.1093/braincomms/fcae002 -
Internal Medicine (Tokyo, Japan) Feb 2024A 73-year-old woman with posterior cortical atrophy (PCA) presented with progressive apperceptive visual agnosia, alexia, agraphia, ventral simultanagnosia,...
A 73-year-old woman with posterior cortical atrophy (PCA) presented with progressive apperceptive visual agnosia, alexia, agraphia, ventral simultanagnosia, prosopagnosia, and allocentric (stimulus-centered) left-sided hemispatial neglect. All of these symptoms were attributed to damage to the bilateral occipito-temporal cortices, consistent with ventral variant PCA. While the Pittsburgh compound B uptake was extensively distributed throughout the occipito-parietal (dorsal) and occipito-temporal (ventral) areas, the THK5351 (ligand binding to tau aggregates/astrocyte gliosis) accumulation was limited to the ventral area. These findings suggest that local accumulation of tau proteins and/or astrocyte gliosis over the occipito-temporal cortices can result in ventral variant PCA.
PubMed: 38369357
DOI: 10.2169/internalmedicine.2844-23 -
Neuro-ophthalmology (Aeolus Press) 2024Vision specialists will benefit from increased awareness of posterior cortical atrophy (PCA) syndrome. Failure to adequately identify the chief complaint as a visual...
Vision specialists will benefit from increased awareness of posterior cortical atrophy (PCA) syndrome. Failure to adequately identify the chief complaint as a visual symptom may lead to incorrect diagnosis or diagnostic delay. A previously healthy, 59-year-old woman presented with a 5-year history of 'losing her stuff'. Upon psychiatric and neuro-ophthalmological evaluation, this symptom was better recognised as a feature of visual agnosia and simultanagnosia. She also presented with multiple previously unrecognised symptoms indicative of higher visual processing dysfunction, such as alexia without agraphia, ocular motor apraxia, optic ataxia, prosopagnosia, akinetopsia and topographagnosia, so further assessment to investigate for PCA was carried out. After a work-up including cognitive assessment, brain structural/functional imaging, and laboratory tests she was diagnosed with visual-variant Alzheimer's disease. Patients with PCA merit a detailed review of their symptoms, as well as the use of office tests such as cognitive evaluation tools, different types of perimetry, colour vision tests, and non-delayed psychiatric consultation for correct management and assessment. This report will emphasise five key aspects to be considered when evaluating patients with PCA.
PubMed: 38357623
DOI: 10.1080/01658107.2023.2257311 -
Cortex; a Journal Devoted To the Study... Mar 2024Despite severe everyday problems recognising faces, some individuals with developmental prosopagnosia (DP) can achieve typical accuracy scores on laboratory face...
Despite severe everyday problems recognising faces, some individuals with developmental prosopagnosia (DP) can achieve typical accuracy scores on laboratory face recognition tests. To address this, studies sometimes also examine response times (RTs), which tend to be longer in DPs relative to control participants. In the present study, 24 potential (according to self-report) DPs and 110 age-matched controls completed the Cambridge Face and Bicycle Memory Tests, old new faces task, and a famous faces test. We used accuracy and the Balanced Integration Score (BIS), a measure that adjusts accuracy for RTs, to classify our sample at the group and individual levels. Subjective face recognition ability was assessed using the PI20 questionnaire and semi structured interviews. Fifteen DPs showed a major impairment using BIS compared with only five using accuracy alone. Logistic regression showed that a model incorporating the BIS measures was the most sensitive for classifying DP and showed highest area under the curve (AUC). Furthermore, larger between-group effect sizes were observed for a derived global (averaged) memory measure calculated using BIS versus accuracy alone. BIS is thus an extremely sensitive novel measure for attenuating speed-accuracy trade-offs that can otherwise mask impairment measured only by accuracy in DP.
Topics: Humans; Prosopagnosia; Facial Recognition; Self Report; Surveys and Questionnaires; Reaction Time; Pattern Recognition, Visual
PubMed: 38330779
DOI: 10.1016/j.cortex.2023.12.011 -
Brain Sciences Jan 2024It is still a matter of debate whether developmental prosopagnosia is a disorder selective to faces or whether object recognition is also affected. In a previous study,...
It is still a matter of debate whether developmental prosopagnosia is a disorder selective to faces or whether object recognition is also affected. In a previous study, based on a small sample of developmental prosopagnosics (DPs; N = 10), we found impairments in both domains although the difficulties were most pronounced for faces. Importantly, impairments with faces and objects were systematically related. We suggested that that the seemingly disproportional impairment for faces in DP was likely to reflect differences between stimulus categories in visual similarity. Here, we aimed to replicate these findings in a larger, independent sample of DPs (N = 21) using the same experimental paradigms. Contrary to our previous results, we found no disproportional effect of visual similarity on performance with faces or objects in the new DP group when compared to controls (N = 21). The new DP group performed within the control range, and significantly better than the old DP-group, on sensitive and demanding object recognition tasks, and we can demonstrate a classical dissociation between face and object recognition at the group level. These findings are perhaps the strongest evidence yet presented for a face-specific deficit in developmental prosopagnosia.
PubMed: 38275527
DOI: 10.3390/brainsci14010107 -
Frontiers in Neurology 2023Frontotemporal lobe disorders (FTD) are amongst the most common brain neurodegenerative disorders. Their relatively covert, frequently subtle presentations and diverse...
BACKGROUND
Frontotemporal lobe disorders (FTD) are amongst the most common brain neurodegenerative disorders. Their relatively covert, frequently subtle presentations and diverse etiologies, pose major challenges in diagnosis and treatments. Recent studies have yielded insights that the etiology in the majority are due to environmental and sporadic causes, rather than genetic in origin.
AIMS
To retrospectively examine the cognitive and behavioral impairments in the veteran population to garner the range of differing syndrome presentations and etiological subcategories with a specific focus on frontotemporal lobe disorders.
METHODOLOGY
The design is a retrospective, observational registry, case series with the collection of epidemiological, clinical, cognitive, laboratory and radiological data on people with cognitive and behavioral disorders. Inclusion criteria for entry were veterans evaluated exclusively at Orlando VA Healthcare System, neurology section, receiving a diagnosis of FTD by standard criteria, during the observation period dated from July 2016 to March 2021. Frontotemporal disorders (FTD) were delineated into five clinical 5 subtypes. Demographic, cardiovascular risk factors, cognitive, behavioral neurological, neuroimaging data and presumed etiological categories, were collected for those with a diagnosis of frontotemporal disorder.
RESULTS
Of the 200 patients with FTD, further cognitive, behavioral neurological evaluation with standardized, metric testing was possible in 105 patients. Analysis of the etiological groups revealed significantly different younger age of the traumatic brain injury (TBI) and Gulf War Illness (GWI) veterans who also had higher Montreal Cognitive Assessment (MOCA) scores. The TBI group also had significantly more abnormalities of hypometabolism, noted on the PET brain scans. Behavioral neurological testing was notable for the findings that once a frontotemporal disorder had been diagnosed, the four different etiological groups consistently had abnormal FRSBE scores for the 3 principal frontal presentations of (i) abulia/apathy, (ii) disinhibition, and (iii) executive dysfunction as well as abnormal Frontal Behavioral Inventory (FBI) scores with no significant difference amongst the etiological groups. The most common sub-syndromes associated with frontotemporal syndromes were the Geschwind-Gastaut syndrome (GGS), Klüver-Bucy syndrome (KBS), involuntary emotional expression disorder (IEED), cerebellar cognitive affective syndrome (CCA), traumatic encephalopathy syndrome (TES) and prosopagnosia. Comparisons with the three principal frontal lobe syndrome clusters (abulia, disinhibition, executive dysfunction) revealed a significant association with abnormal disinhibition FRSBE T-scores with the GGS. The regression analysis supported the potential contribution of disinhibition behavior that related to this complex, relatively common behavioral syndrome in this series. The less common subsyndromes in particular, were notable, as they constituted the initial overriding, presenting symptoms and syndromes characterized into 16 separate conditions.
CONCLUSION
By deconstructing FTD into the multiple sub-syndromes and differing etiologies, this study may provide foundational insights, enabling a more targeted precision medicine approach for future studies, both in treating the sub-syndromes as well as the underlying etiological process.
PubMed: 38264092
DOI: 10.3389/fneur.2023.1305071