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Brain : a Journal of Neurology Sep 2020The concept of the right temporal variant of frontotemporal dementia (rtvFTD) is still equivocal. The syndrome accompanying predominant right anterior temporal atrophy...
The concept of the right temporal variant of frontotemporal dementia (rtvFTD) is still equivocal. The syndrome accompanying predominant right anterior temporal atrophy has previously been described as memory loss, prosopagnosia, getting lost and behavioural changes. Accurate detection is challenging, as the clinical syndrome might be confused with either behavioural variant FTD (bvFTD) or Alzheimer's disease. Furthermore, based on neuroimaging features, the syndrome has been considered a right-sided variant of semantic variant primary progressive aphasia (svPPA). Therefore, we aimed to demarcate the clinical and neuropsychological characteristics of rtvFTD versus svPPA, bvFTD and Alzheimer's disease. Moreover, we aimed to compare its neuroimaging profile against svPPA, which is associated with predominant left anterior temporal atrophy. Of 619 subjects with a clinical diagnosis of frontotemporal dementia or primary progressive aphasia, we included 70 subjects with a negative amyloid status in whom predominant right temporal lobar atrophy was identified based on blinded visual assessment of their initial brain MRI scans. Clinical symptoms were assessed retrospectively and compared with age- and sex-matched patients with svPPA (n = 70), bvFTD (n = 70) and Alzheimer's disease (n = 70). Prosopagnosia, episodic memory impairment and behavioural changes such as disinhibition, apathy, compulsiveness and loss of empathy were the most common initial symptoms, whereas during the disease course, patients developed language problems such as word-finding difficulties and anomia. Distinctive symptoms of rtvFTD compared to the other groups included depression, somatic complaints, and motor/mental slowness. Aside from right temporal atrophy, the imaging pattern showed volume loss of the right ventral frontal area and the left temporal lobe, which represented a close mirror image of svPPA. Atrophy of the bilateral temporal poles and the fusiform gyrus were associated with prosopagnosia in rtvFTD. Our results highlight that rtvFTD has a unique clinical presentation. Since current diagnostic criteria do not cover specific symptoms of the rtvFTD, we propose a diagnostic tree to be used to define diagnostic criteria and call for an international validation.
Topics: Aged; Case-Control Studies; Cohort Studies; Female; Frontotemporal Dementia; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Retrospective Studies; Temporal Lobe
PubMed: 32830218
DOI: 10.1093/brain/awaa225 -
Behavior Research Methods Dec 2023Developmental prosopagnosia is characterized by severe, lifelong difficulties when recognizing facial identity. Unfortunately, the most common diagnostic assessment...
Developmental prosopagnosia is characterized by severe, lifelong difficulties when recognizing facial identity. Unfortunately, the most common diagnostic assessment (Cambridge Face Memory Test) misses 50-65% of individuals who believe that they have this condition. This results in such excluded cases' absence from scientific knowledge, effect sizes of impairment potentially overestimated, treatment efficacy underrated, and may elicit in them a negative experience of research. To estimate their symptomology and group-level impairments in face processing, we recruited a large cohort who believes that they have prosopagnosia. Matching prior reports, 56% did not meet criteria on the Cambridge Face Memory Test. However, the severity of their prosopagnosia symptoms and holistic perception deficits were comparable to those who did meet criteria. Excluded cases also exhibited face perception and memory impairments that were roughly one standard deviation below neurotypical norms, indicating the presence of objective problems. As the prosopagnosia index correctly classified virtually every case, we propose it should be the primary method for providing a diagnosis, prior to subtype categorization. We present researchers with a plan on how they can analyze these excluded prosopagnosia cases in their future work without negatively impacting their traditional findings. We anticipate such inclusion will enhance scientific knowledge, more accurately estimate effect sizes of impairments and treatments, and identify commonalities and distinctions between these different forms of prosopagnosia. Owing to their atypicalities in visual perception, we recommend that the prosopagnosia index should be used to screen out potential prosopagnosia cases from broader vision research.
Topics: Humans; Prosopagnosia; Recognition, Psychology; Visual Perception; Facial Recognition; Pattern Recognition, Visual
PubMed: 36459376
DOI: 10.3758/s13428-022-02017-w -
Medicina 2021Proposapnosia is a type of visual agnosia characterized by the inability to recognize people's faces. There are basically two variants, apperceptive and associative. The...
Proposapnosia is a type of visual agnosia characterized by the inability to recognize people's faces. There are basically two variants, apperceptive and associative. The "Tortoni effect" is a phenomenon described by Bekinschtein et al a few years ago in waiters from Buenos Aires, who used this tool to remember the orders of each member of a table. We present a case of prosopagnosia associated with bilateral temporo-occipital injury secondary to head trauma, initially manifested by the lack of face recognition with the use of an associative strategy similar to that described in the "Tortoni effect" as compensation, in a 62-year-old female who suffered a severe head injury. A few months after this event, the patient had difficulty in recognizing familiar people, a fact evidenced by her relatives when at a restaurant table, they changed their seats, remained silent momentarily, and right after the patient kept naming them by their previous location. The magnetic resonance imaging of the brain revealed blunt sequelae lesions in the bilateral temporo-occipital region. Acquired prosopagnosia due to focal lesions in the temporo-occipital region, generally bilateral and right, and less frequently left, is a rare condition. The strategy used in the "Tortoni effect" was one of the initial manifestations of the condition in our patient. Carrying out an ecological neuropsychological test that considers this strategy could be useful in the screening and early detection of this entity.
Topics: Brain; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Neuropsychological Tests; Prosopagnosia
PubMed: 34633963
DOI: No ID Found -
Molecular Autism Oct 2020Face individual identity recognition skill is heritable and independent of intellectual ability. Difficulties in face individual identity recognition are present in...
BACKGROUND
Face individual identity recognition skill is heritable and independent of intellectual ability. Difficulties in face individual identity recognition are present in autistic individuals and their family members and are possibly linked to oxytocin polymorphisms in families with an autistic child. While it is reported that developmental prosopagnosia (i.e., impaired face identity recognition) occurs in 2-3% of the general population, no prosopagnosia prevalence estimate is available for autism. Furthermore, an autism within-group approach has not been reported towards characterizing impaired face memory and to investigate its possible links to social and communication difficulties.
METHODS
The present study estimated the prevalence of prosopagnosia in 80 autistic adults with no intellectual disability, investigated its cognitive characteristics and links to autism symptoms' severity, personality traits, and mental state understanding from the eye region by using standardized tests and questionnaires.
RESULTS
More than one third of autistic participants showed prosopagnosia. Their face memory skill was not associated with their symptom's severity, empathy, alexithymia, or general intelligence. Face identity recognition was instead linked to mental state recognition from the eye region only in autistic individuals who had prosopagnosia, and this relationship did not depend on participants' basic face perception skills. Importantly, we found that autistic participants were not aware of their face memory skills.
LIMITATIONS
We did not test an epidemiological sample, and additional work is necessary to establish whether these results generalize to the entire autism spectrum.
CONCLUSIONS
Impaired face individual identity recognition meets the criteria to be a potential endophenotype in autism. In the future, testing for face memory could be used to stratify autistic individuals into genetically meaningful subgroups and be translatable to autism animal models.
Topics: Adolescent; Adult; Aged; Autistic Disorder; Case-Control Studies; Endophenotypes; Facial Recognition; Female; Humans; Intelligence; Male; Memory; Middle Aged; Personality; Prevalence; Prosopagnosia; Recognition, Psychology; Young Adult
PubMed: 33081830
DOI: 10.1186/s13229-020-00371-0 -
Neuropsychologia Dec 2021Numerous neurological, developmental, and psychiatric conditions demonstrate impaired face recognition, which can be socially debilitating. These impairments can be...
Numerous neurological, developmental, and psychiatric conditions demonstrate impaired face recognition, which can be socially debilitating. These impairments can be caused by either deficient face perception or face memory mechanisms. Though there are well-validated, sensitive measures of face memory impairments, it currently remains unclear which assessments best measure face perception impairments. A sensitive, validated face perception measure could help with diagnosing causes of face recognition deficits and be useful in characterizing individual differences in unimpaired populations. Here, we compared the computerized Benton Face Recognition Test (BFRT-c) and Cambridge Face Perception Test (CFPT) in their ability to differentiate developmental prosopagnosics (DPs, N = 30) and age-matched controls (N = 30). Participants completed the BFRT-c, CFPT, and two additional face perception assessments: the University of Southern California Face Perception Test (USCFPT) and a novel same/different face matching test (SDFMT). Participants were also evaluated on objective and subjective face recognition tasks including the Cambridge Face Memory Test, famous faces test, and Prosopagnosia Index-20. We performed a logistic regression with the perception tests predicting DP vs. control group membership and used multiple linear regressions to predict continuous objective and subjective face recognition memory. Our results show that the BFRT-c performed as well as, if not better than, the CFPT, and that both tests clearly outperformed the USCFPT and SDFMT. Further, exploratory analyses revealed that face lighting-change conditions better predicted DP group membership and face recognition abilities than viewpoint-change conditions. Together, these results support the combined use of the BFRT-c and CFPT to best assess face perception impairments.
Topics: Facial Recognition; Head; Humans; Memory Disorders; Pattern Recognition, Visual; Prosopagnosia; Recognition, Psychology
PubMed: 34673046
DOI: 10.1016/j.neuropsychologia.2021.108067 -
Brain Sciences Feb 2023Understanding how the human brain recognizes faces is a primary scientific goal in cognitive neuroscience. Given the limitations of the monkey model of human face... (Review)
Review
Understanding how the human brain recognizes faces is a primary scientific goal in cognitive neuroscience. Given the limitations of the monkey model of human face recognition, a key approach in this endeavor is the recording of electrophysiological activity with electrodes implanted inside the brain of human epileptic patients. However, this approach faces a number of challenges that must be overcome for meaningful scientific knowledge to emerge. Here we synthesize a 10 year research program combining the recording of intracerebral activity (StereoElectroEncephaloGraphy, SEEG) in the ventral occipito-temporal cortex (VOTC) of large samples of participants and fast periodic visual stimulation (FPVS), to objectively define, quantify, and characterize the neural basis of human face recognition. These large-scale studies reconcile the wide distribution of neural face recognition activity with its (right) hemispheric and regional specialization and extend face-selectivity to anterior regions of the VOTC, including the ventral anterior temporal lobe (VATL) typically affected by magnetic susceptibility artifacts in functional magnetic resonance imaging (fMRI). Clear spatial dissociations in category-selectivity between faces and other meaningful stimuli such as landmarks (houses, medial VOTC regions) or written words (left lateralized VOTC) are found, confirming and extending neuroimaging observations while supporting the validity of the clinical population tested to inform about normal brain function. The recognition of face identity - arguably the ultimate form of recognition for the human brain - beyond mere differences in physical features is essentially supported by selective populations of neurons in the right inferior occipital gyrus and the lateral portion of the middle and anterior fusiform gyrus. In addition, low-frequency and high-frequency broadband iEEG signals of face recognition appear to be largely concordant in the human association cortex. We conclude by outlining the challenges of this research program to understand the neural basis of human face recognition in the next 10 years.
PubMed: 36831897
DOI: 10.3390/brainsci13020354 -
Journal of Vision Aug 2019Despite extensive investigation, the causes and nature of developmental prosopagnosia (DP)-a severe face identification impairment in the absence of acquired brain...
Despite extensive investigation, the causes and nature of developmental prosopagnosia (DP)-a severe face identification impairment in the absence of acquired brain injury-remain poorly understood. Drawing on previous work showing that individuals identified as being neurotypical (NT) show robust individual differences in where they fixate on faces, and recognize faces best when the faces are presented at this location, we defined and tested four novel hypotheses for how atypical face-looking behavior and/or retinotopic face encoding could impair face recognition in DP: (a) fixating regions of poor information, (b) inconsistent saccadic targeting, (c) weak retinotopic tuning, and (d) fixating locations not matched to the individual's own face tuning. We found no support for the first three hypotheses, with NTs and DPs consistently fixating similar locations and showing similar retinotopic tuning of their face perception performance. However, in testing the fourth hypothesis, we found preliminary evidence for two distinct phenotypes of DP: (a) Subjects characterized by impaired face memory, typical face perception, and a preference to look high on the face, and (b) Subjects characterized by profound impairments to both face memory and perception and a preference to look very low on the face. Further, while all NTs and upper-looking DPs performed best when faces were presented near their preferred fixation location, this was not true for lower-looking DPs. These results suggest that face recognition deficits in a substantial proportion of people with DP may arise not from aberrant face gaze or compromised retinotopic tuning, but from the suboptimal matching of gaze to tuning.
Topics: Adult; Attention; Eye Movements; Facial Recognition; Female; Humans; Male; Middle Aged; Prosopagnosia; Saccades
PubMed: 31426085
DOI: 10.1167/19.9.7 -
Brain Communications 2019Developmental prosopagnosia is a disorder characterized by profound and lifelong difficulties with face recognition in the absence of sensory or intellectual deficits or...
Developmental prosopagnosia is a disorder characterized by profound and lifelong difficulties with face recognition in the absence of sensory or intellectual deficits or known brain injury. While there has been a surge in research on developmental prosopagnosia over the last decade and a half, the cognitive mechanisms behind the disorder and its neural underpinnings remain elusive. Most recently it has been proposed that developmental prosopagnosia may be a manifestation of widespread disturbance in neural migration which affects both face responsive brain regions as well as other category-sensitive visual areas. We present a combined behavioural and functional MRI study of face, object and word processing in a group of developmental prosopagnosics ( = 15). We show that developmental prosopagnosia is associated with reduced activation of core ventral face areas during perception of faces. The reductions were bilateral but tended to be more pronounced in the left hemisphere. As the first study to address category selectivity for word processing in developmental prosopagnosia, we do not, however, find evidence for reduced activation of the visual word form area during perception of orthographic material. We also find no evidence for reduced activation of the lateral occipital complex during perception of objects. These imaging findings correspond well with the behavioural performance of the developmental prosopagnosics, who show severe impairment for faces but normal reading and recognition of line drawings. Our findings suggest that a general deficit in neural migration across ventral occipito-temporal cortex is not a viable explanation for developmental prosopagnosia. The finding of left hemisphere involvement in our group of developmental prosopagnosics was at first surprising. However, a closer look at existing studies shows similar, but hitherto undiscussed, findings. These left hemisphere abnormalities seen in developmental prosopagnosia contrasts with lesion and imaging studies suggesting primarily right hemisphere involvement in acquired prosopagnosia, and this may reflect that the left hemisphere is important for the development of a normal face recognition network.
PubMed: 32954273
DOI: 10.1093/braincomms/fcz034 -
Revista de Neurologia Jul 2020Congenital amusia is a specific condition in which the individual is unable to recognise tonal variations in a piece of musical. This cannot be explained by a previous... (Review)
Review
INTRODUCTION
Congenital amusia is a specific condition in which the individual is unable to recognise tonal variations in a piece of musical. This cannot be explained by a previous brain injury, hearing loss, cognitive deficit, socio-affective disorder or lack of environmental stimulation. The current estimated prevalence is 1.5% of the world population, with a significant genetic component among those who suffer from it. It has been claimed that certain cognitive abilities in the emotional, spatial and language fields may be affected in people with amusia.
AIM
To review the literature describing the effects on non-musical skills that may coexist in individuals with congenital amusia.
DEVELOPMENT
Several neuroimaging studies have observed morphological and functional changes in the temporal lobe, as well as in the white matter connections between the superior temporal gyrus and the inferior frontal gyrus. From these affected regions, there may be a deficit in cognitive skills related to adjacent areas.
CONCLUSIONS
Congenital amusia has been associated with poor performance in different non-musical cognitive skills, such as visuospatial processing, language processing, reading difficulties, face recognition and emotional aspects.
Topics: Affective Symptoms; Auditory Perceptual Disorders; Dyslexia; Female; Humans; Language Development Disorders; Male; Neural Pathways; Prosopagnosia; Psychomotor Performance; Spatial Navigation; Temporal Lobe; White Matter
PubMed: 32627163
DOI: 10.33588/rn.7102.2020066 -
Brain : a Journal of Neurology Dec 2019Damage to the right fusiform face area can disrupt the ability to recognize faces, a classic example of how damage to a specialized brain region can disrupt a...
Damage to the right fusiform face area can disrupt the ability to recognize faces, a classic example of how damage to a specialized brain region can disrupt a specialized brain function. However, similar symptoms can arise from damage to other brain regions, and face recognition is now thought to depend on a distributed brain network. The extent of this network and which regions are critical for facial recognition remains unclear. Here, we derive this network empirically based on lesion locations causing clinically significant impairments in facial recognition. Cases of acquired prosopagnosia were identified through a systematic literature search and lesion locations were mapped to a common brain atlas. The network of brain regions connected to each lesion location was identified using resting state functional connectivity from healthy participants (n = 1000), a technique termed lesion network mapping. Lesion networks were overlapped to identify connections common to lesions causing prosopagnosia. Reproducibility was assessed using split-half replication. Specificity was assessed through comparison with non-specific control lesions (n = 135) and with control lesions associated with symptoms other than prosopagnosia (n = 155). Finally, we tested whether our facial recognition network derived from clinically evident cases of prosopagnosia could predict subclinical facial agnosia in an independent lesion cohort (n = 31). Our systematic literature search identified 44 lesions causing prosopagnosia, only 29 of which intersected the right fusiform face area. However, all 44 lesion locations fell within a single brain network defined by connectivity to the right fusiform face area. Less consistent connectivity was found to other face-selective regions. Surprisingly, all 44 lesion locations were also functionally connected, through negative correlation, with regions in the left frontal cortex. This connectivity pattern was highly reproducible and specific to lesions causing prosopagnosia. Positive connectivity to the right fusiform face area and negative connectivity to left frontal regions were independent predictors of prosopagnosia and predicted subclinical facial agnosia in an independent lesion cohort. We conclude that lesions causing prosopagnosia localize to a single functionally connected brain network defined by connectivity to the right fusiform face area and to left frontal regions. Implications of these findings for models of facial recognition deficits are discussed.
Topics: Brain; Brain Mapping; Humans; Image Processing, Computer-Assisted; Magnetic Resonance Imaging; Nerve Net; Prosopagnosia; Reproducibility of Results
PubMed: 31740940
DOI: 10.1093/brain/awz332