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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 -
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 -
Brain Sciences Jan 2024Existing evidence suggests that developmental prosopagnosia (DP) is a surprisingly prevalent condition, with some individuals describing lifelong difficulties with...
Existing evidence suggests that developmental prosopagnosia (DP) is a surprisingly prevalent condition, with some individuals describing lifelong difficulties with facial identity recognition. Together with case reports of multiple family members with the condition, this evidence suggests that DP is inherited in at least some instances. Here, we offer some novel case series that further support the heritability of the condition. First, we describe five adult siblings who presented to our lab with symptoms of DP. Second, for the first known time in the literature, we describe a pair of adult identical twins who contacted us in the belief that they both experience DP. The condition was confirmed in three of the five siblings (with minor symptoms observed in the remaining two) and in both twins. Supplementary assessments suggested that all individuals also experienced some degree of difficulty with facial identity perception, but that object recognition was preserved. These findings bolster the evidence supporting the heritability of DP and suggest that it can be a specific impairment in some cases.
PubMed: 38248264
DOI: 10.3390/brainsci14010049 -
Neuroscience and Biobehavioral Reviews Mar 2024Face-selective regions in the human ventral occipito-temporal cortex (VOTC) have been defined for decades mainly with functional magnetic resonance imaging. This... (Review)
Review
Face-selective regions in the human ventral occipito-temporal cortex (VOTC) have been defined for decades mainly with functional magnetic resonance imaging. This face-selective VOTC network is traditionally divided in a posterior 'core' system thought to subtend face perception, and regions of the anterior temporal lobe as a semantic memory component of an extended general system. In between these two putative systems lies the anterior fusiform gyrus and surrounding sulci, affected by magnetic susceptibility artifacts. Here we suggest that this methodological gap overlaps with and contributes to a conceptual gap between (visual) perception and semantic memory for faces. Filling this gap with intracerebral recordings and direct electrical stimulation reveals robust face-selectivity in the anterior fusiform gyrus and a crucial role of this region, especially in the right hemisphere, in identity recognition for both familiar and unfamiliar faces. Based on these observations, we propose an integrated theoretical framework for human face (identity) recognition according to which face-selective regions in the anterior fusiform gyrus join the dots between posterior and anterior cortical face memories.
Topics: Humans; Prosopagnosia; Temporal Lobe; Facial Recognition; Recognition, Psychology; Magnetic Resonance Imaging; Pattern Recognition, Visual; Brain Mapping; Photic Stimulation
PubMed: 38191080
DOI: 10.1016/j.neubiorev.2024.105535 -
Tijdschrift Voor Psychiatrie 2023Artificial intelligence (AI) has evolved enormously over the past decade and is increasingly being applied to a range of domains, including psychiatry. AI encompasses... (Review)
Review
BACKGROUND
Artificial intelligence (AI) has evolved enormously over the past decade and is increasingly being applied to a range of domains, including psychiatry. AI encompasses several modalities, including artificial neural networks (ANNs), referring to computer models partly based on the workings of the brain. ANNs have existed since the ’50s, but only became ‘mainstream’ since the 2010s. The fact that they are inspired by the workings of the brain raises the question of whether they can also be used to model the (dys)functioning of the brain. This question led to the advent of the research field ‘computational psychiatry’.
AIM
This article aims at providing an accessible introduction to artificial neural networks, and potential applications hereof in contemporary psychiatric practice.
METHOD
Literature review with some examples.
RESULTS
In this article we try to outline with some concrete examples what artificial neural networks are and how they can be used to model mechanisms in the brain. We successively discuss ANNs as a model of the human visual system, as a model of prosopagnosia and as a model of auditory hallucinations and finally as a model of autism spectrum disorder. We also describe a number of limitations of this approach.
CONCLUSION
A computer model that models the entire brain is challenging at present, but current models can help in testing hypotheses concerning possible mechanisms that give rise to a wide range of neuropsychiatric conditions.
Topics: Humans; Artificial Intelligence; Autism Spectrum Disorder; Brain; Neural Networks, Computer; Psychiatry
PubMed: 38174402
DOI: No ID Found -
Brain Sciences Sep 2023Faces play a crucial role in social interactions. Developmental prosopagnosia (DP) refers to the lifelong difficulty in recognizing faces despite the absence of obvious... (Review)
Review
Faces play a crucial role in social interactions. Developmental prosopagnosia (DP) refers to the lifelong difficulty in recognizing faces despite the absence of obvious signs of brain lesions. In recent decades, the neural substrate of this condition has been extensively investigated. While early neuroimaging studies did not reveal significant functional and structural abnormalities in the brains of individuals with developmental prosopagnosia (DPs), recent evidence identifies abnormalities at multiple levels within DPs' face-processing networks. The current work aims to provide an overview of the convergent and contrasting findings by examining twenty-five years of neuroimaging literature on the anatomo-functional correlates of DP. We included 55 original papers, including 63 studies that compared the brain structure (MRI) and activity (fMRI, EEG, MEG) of healthy control participants and DPs. Despite variations in methods, procedures, outcomes, sample selection, and study design, this scoping review suggests that morphological, functional, and electrophysiological features characterize DPs' brains, primarily within the ventral visual stream. Particularly, the functional and anatomical connectivity between the Fusiform Face Area and the other face-sensitive regions seems strongly impaired. The cognitive and clinical implications as well as the limitations of these findings are discussed in light of the available knowledge and challenges in the context of DP.
PubMed: 37891769
DOI: 10.3390/brainsci13101399 -
Cureus Sep 2023Prosopagnosia describes the inability to recognize others by their faces, which may be hereditary or acquired. Acquired cases result from intracranial lesions such as...
Prosopagnosia describes the inability to recognize others by their faces, which may be hereditary or acquired. Acquired cases result from intracranial lesions such as intracranial hemorrhage or ischemia. This case demonstrates acquired prosopagnosia secondary to an intracranial hemorrhage and thus exemplifies the importance of early symptom recognition for appropriate diagnosis and management. A 58-year-old female presented to the emergency department with a chief complaint of the worst headache of her life along with nausea and vomiting. She also reported that she was unable to recognize her children in photos and although she knew her husband was with her, she did not recognize his face. Physical examination revealed no focal motor deficits. Computed tomography angiography of the brain revealed intracerebral hemorrhage of the right occipital lobe. Acquired prosopagnosia can be the only presenting symptom of intracranial pathology. It is most commonly caused by intracranial hemorrhage, as shown in this case report. This demonstrates a unique symptom of posterior circulation strokes that are commonly misdiagnosed in the emergency department.
PubMed: 37842404
DOI: 10.7759/cureus.45128