-
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 -
Behavior Research Methods Feb 2022Tests of face processing are typically designed to identify individuals performing outside of the typical range; either prosopagnosic individuals who exhibit poor face...
Tests of face processing are typically designed to identify individuals performing outside of the typical range; either prosopagnosic individuals who exhibit poor face processing ability, or super recognisers, who have superior face processing abilities. Here we describe the development of the Oxford Face Matching Test (OFMT), designed to identify individual differences in face processing across the full range of performance, from prosopagnosia, through the range of typical performance, to super recognisers. Such a test requires items of varying difficulty, but establishing difficulty is problematic when particular populations (e.g., prosopagnosics, individuals with autism spectrum disorder) may use atypical strategies to process faces. If item difficulty is calibrated on neurotypical individuals, then the test may be poorly calibrated for atypical groups, and vice versa. To obtain items of varying difficulty, we used facial recognition algorithms to obtain face pair similarity ratings that are not biased towards specific populations. These face pairs were used as stimuli in the OFMT, and participants were required to judge whether the face images depicted the same individual or different individuals. Across five studies the OFMT was shown to be sensitive to individual differences in the typical population, and in groups of both prosopagnosic individuals and super recognisers. The test-retest reliability of the task was at least equivalent to the Cambridge Face Memory Test and the Glasgow Face Matching Test. Furthermore, results reveal, at least at the group level, that both face perception and face memory are poor in those with prosopagnosia, and are good in super recognisers.
Topics: Autism Spectrum Disorder; Facial Recognition; Humans; Individuality; Prosopagnosia; Reproducibility of Results
PubMed: 34131874
DOI: 10.3758/s13428-021-01609-2 -
Scientific Reports Nov 2020Developmental prosopagnosia (DP) is a condition characterised by lifelong face recognition difficulties. Recent neuroimaging findings suggest that DP may be associated...
Developmental prosopagnosia (DP) is a condition characterised by lifelong face recognition difficulties. Recent neuroimaging findings suggest that DP may be associated with aberrant structure and function in multimodal regions of cortex implicated in the processing of both facial and vocal identity. These findings suggest that both facial and vocal recognition may be impaired in DP. To test this possibility, we compared the performance of 22 DPs and a group of typical controls, on closely matched tasks that assessed famous face and famous voice recognition ability. As expected, the DPs showed severe impairment on the face recognition task, relative to typical controls. In contrast, however, the DPs and controls identified a similar number of voices. Despite evidence of interactions between facial and vocal processing, these findings suggest some degree of dissociation between the two processing pathways, whereby one can be impaired while the other develops typically. A possible explanation for this dissociation in DP could be that the deficit originates in the early perceptual encoding of face structure, rather than at later, post-perceptual stages of face identity processing, which may be more likely to involve interactions with other modalities.
Topics: Adult; Facial Recognition; Female; Humans; Male; Pattern Recognition, Visual; Prosopagnosia; Recognition, Psychology; Visual Perception; Voice Recognition
PubMed: 33184411
DOI: 10.1038/s41598-020-76819-3 -
Cognitive Research: Principles and... Feb 2022Some research indicates that face masks impair identification and other judgements such as trustworthiness. However, it is unclear whether those effects have abated over...
Some research indicates that face masks impair identification and other judgements such as trustworthiness. However, it is unclear whether those effects have abated over time as individuals adjust to widespread use of masks, or whether performance is related to individual differences in face recognition ability. This study examined the effect of masks and sunglasses on face matching and social judgements (trustworthiness, competence, attractiveness). In Experiment 1, 135 participants across three different time points (June 2020-July 2021) viewed unedited faces and faces with masks, sunglasses, or both. Both masks and sunglasses similarly decreased matching performance. The effect of masks on social judgements varied depending on the judgement and whether the face was depicted with sunglasses. There was no effect of timepoint on any measure, suggesting that the effects of masks have not diminished. In Experiment 2, 12 individuals with developmental prosopagnosia (DP) and 10 super-recognisers (SRs) completed the same tasks. The effect of masks on identity matching was reduced in SRs, whereas the effects of masks and sunglasses for the DP group did not differ from controls. These findings indicate that face masks significantly affect face perception, depending on the availability of other facial information, and are not modified by exposure.
Topics: Eyeglasses; Facial Recognition; Humans; Individuality; Masks; Sociological Factors
PubMed: 35171394
DOI: 10.1186/s41235-022-00371-z -
PloS One 2021Anterior temporal lobectomy (ATL) is the most common surgical treatment for drug-resistant temporal lobe epilepsy (TLE). Right ATL has been reported to reduce facial...
Anterior temporal lobectomy (ATL) is the most common surgical treatment for drug-resistant temporal lobe epilepsy (TLE). Right ATL has been reported to reduce facial memory ability in patients with TLE, as indicated by poor performance on the Warrington Recognition Memory Test for Faces (RMF), which is commonly used to evaluate visual memory in these patients. However, little is known about whether patients with TLE exhibit difficulties in identifying faces in daily life after ATL. The aim of this study was to investigate facial memory ability and self-awareness of face identification difficulties in patients with TLE after ATL. Sixteen patients with TLE after right ATL, 14 patients with TLE after left ATL, and 29 healthy controls were enrolled in this study. We developed the multiview face recognition test (MFRT), which comprises a learning phase (one or three frontal face images without external facial feature information) and a recognition phase (frontal, oblique, or noise-masked face images). Facial memory abilities were examined in all participants using the MFRT and RMF, and self-awareness of difficulties in face identification was evaluated using the 20-item prosopagnosia index (PI20), which has been widely used to assess developmental prosopagnosia. The MFRT performance in patients with TLE after ATL was significantly worse than that in healthy controls regardless of the resected side, whereas the RMF scores in patients with TLE were significantly worse than those in healthy controls only after right ATL. The MFRT performance in patients with TLE after both left and right ATL was more influenced by working memory load than that in healthy controls. The PI20 scores revealed that patients with TLE after left ATL were aware of their difficulties in identifying faces. These findings suggest that patients with TLE not only after right ATL but also after left ATL might have difficulties in face identification.
Topics: Adult; Anterior Temporal Lobectomy; Case-Control Studies; Epilepsy, Temporal Lobe; Facial Recognition; Female; Humans; Male; Memory Disorders; Neuropsychological Tests; Postoperative Complications
PubMed: 33793593
DOI: 10.1371/journal.pone.0248785 -
Royal Society Open Science Nov 2021The Twenty Item Prosopagnosia Index (PI20) is a self-report questionnaire used for quantifying prosopagnosic traits. This scale is intended to help researchers identify...
The Twenty Item Prosopagnosia Index (PI20) is a self-report questionnaire used for quantifying prosopagnosic traits. This scale is intended to help researchers identify cases of developmental prosopagnosia by providing standardized self-report evidence to complement diagnostic evidence obtained from objective computer-based tasks. In order to respond appropriately to items, prosopagnosics must have some insight that their face recognition is well below average, while non-prosopagnosics need to understand that their relative face recognition ability falls within the typical range. There has been considerable debate about whether participants have the necessary insight into their face recognition abilities to respond appropriately. In the present study, we sought to determine whether the PI20 provides meaningful evidence of face recognition impairment. In keeping with the intended use of the instrument, we used PI20 scores to identify two groups: high-PI20 scorers (those with self-reported face recognition difficulties) and low-PI20 scorers (those with no self-reported face recognition difficulties). We found that participant groups distinguished on the basis of PI20 scores clearly differed in terms of their mean performance on objective measures of face recognition ability. We also found that high-PI20 scorers were more likely to achieve levels of face recognition accuracy associated with developmental prosopagnosia.
PubMed: 34737872
DOI: 10.1098/rsos.202062 -
BMJ Case Reports Dec 2020We illustrate a case of post-traumatic recurrent transient prosopagnosia in a paediatric patient with a right posterior inferior temporal gyrus haemorrhage seen on...
We illustrate a case of post-traumatic recurrent transient prosopagnosia in a paediatric patient with a right posterior inferior temporal gyrus haemorrhage seen on imaging and interictal electroencephalogram abnormalities in the right posterior quadrant. Face recognition area mapping with magnetoencephalography (MEG) and functional MRI (fMRI) was performed to clarify the relationship between the lesion and his prosopagnosia, which showed activation of the right fusiform gyrus that colocalised with the lesion. Lesions adjacent to the right fusiform gyrus can result in seizures presenting as transient prosopagnosia. MEG and fMRI can help to attribute this unique semiology to the lesion.
Topics: Brain Mapping; Cerebral Hemorrhage; Child; Electroencephalography; Facial Recognition; Humans; Magnetic Resonance Imaging; Male; Neurosurgical Procedures; Prosopagnosia; Seizures; Temporal Lobe; Treatment Outcome
PubMed: 33370980
DOI: 10.1136/bcr-2020-237228 -
Scientific Reports Dec 2019Developmental prosopagnosia (DP) is a neurodevelopmental condition characterised by difficulties recognising and discriminating faces. It is currently unclear whether...
Developmental prosopagnosia (DP) is a neurodevelopmental condition characterised by difficulties recognising and discriminating faces. It is currently unclear whether the perceptual impairments seen in DP are restricted to identity information, or also affect the perception of other facial characteristics. To address this question, we compared the performance of 17 DPs and matched controls on two sensitive sex categorisation tasks. First, in a morph categorisation task, participants made binary decisions about faces drawn from a morph continuum that blended incrementally an average male face and an average female face. We found that judgement precision was significantly lower in the DPs than in the typical controls. Second, we used a sex discrimination task, where female or male facial identities were blended with an androgynous average face. We manipulated the relative weighting of each facial identity and the androgynous average to create four levels of signal strength. We found that DPs were significantly less sensitive than controls at each level of difficulty. Together, these results suggest that the visual processing difficulties in DP extend beyond the extraction of facial identity and affects the extraction of other facial characteristics. Deficits of facial sex categorisation accord with an apperceptive characterisation of DP.
Topics: Adult; Discrimination, Psychological; Facial Recognition; Female; Humans; Male; Middle Aged; Photic Stimulation; Prosopagnosia; Sex Characteristics; Task Performance and Analysis
PubMed: 31836836
DOI: 10.1038/s41598-019-55569-x -
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 -
Psychological Research Jul 2021An imbalance between top-down and bottom-up processing on perception (specifically, over-reliance on top-down processing) can lead to anomalous perception, such as...
An imbalance between top-down and bottom-up processing on perception (specifically, over-reliance on top-down processing) can lead to anomalous perception, such as illusions. One factor that may be involved in anomalous perception is visual mental imagery, which is the experience of "seeing" with the mind's eye. There are vast individual differences in self-reported imagery vividness, and more vivid imagery is linked to a more sensory-like experience. We, therefore, hypothesized that susceptibility to anomalous perception is linked to individual imagery vividness. To investigate this, we adopted a paradigm that is known to elicit the perception of faces in pure visual noise (pareidolia). In four experiments, we explored how imagery vividness contributes to this experience under different response instructions and environments. We found strong evidence that people with more vivid imagery were more likely to see faces in the noise, although removing suggestive instructions weakened this relationship. Analyses from the first two experiments led us to explore confidence as another factor in pareidolia proneness. We, therefore, modulated environment noise and added a confidence rating in a novel design. We found strong evidence that pareidolia proneness is correlated with uncertainty about real percepts. Decreasing perceptual ambiguity abolished the relationship between pareidolia proneness and both imagery vividness and confidence. The results cannot be explained by incidental face-like patterns in the noise, individual variations in response bias, perceptual sensitivity, subjective perceptual thresholds, viewing distance, testing environments, motivation, gender, or prosopagnosia. This indicates a critical role of mental imagery vividness and perceptual uncertainty in anomalous perceptual experience.
Topics: Humans; Illusions; Imagination; Individuality; Uncertainty; Visual Perception
PubMed: 32476064
DOI: 10.1007/s00426-020-01364-7