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Brain : a Journal of Neurology Feb 2023Neurodevelopmental disorders are categorized and studied according to their manifestations as distinct syndromes. For instance, congenital prosopagnosia and dyslexia...
Neurodevelopmental disorders are categorized and studied according to their manifestations as distinct syndromes. For instance, congenital prosopagnosia and dyslexia have largely non-overlapping research literatures and clinical pathways for diagnosis and intervention. On the other hand, the high incidence of neurodevelopmental comorbidities or co-existing extreme strengths and weaknesses suggest that transdiagnostic commonalities may be greater than currently appreciated. The core-periphery model holds that brain regions within the stable core perceptual and motor regions are more densely connected to one another compared to regions in the flexible periphery comprising multimodal association regions. This model provides a framework for the interpretation of neural data in normal development and clinical disorders. Considering network-level commonalities reported in studies of neurodevelopmental disorders, variability in multimodal association cortex connectivity may reflect a shared origin of seemingly distinct neurodevelopmental disorders. This framework helps to explain both comorbidities in neurodevelopmental disorders and profiles of strengths and weaknesses attributable to competitive processing between cognitive systems within an individual.
Topics: Humans; Neurodevelopmental Disorders; Brain; Cerebral Cortex; Dyslexia; Prosopagnosia; Magnetic Resonance Imaging; Neural Pathways
PubMed: 36299249
DOI: 10.1093/brain/awac387 -
Cognitive Neuropsychology 2022Response times (RTs) are commonly used to assess cognitive abilities, though it is unclear whether face processing RTs predict recognition ability beyond accuracy. In...
Response times (RTs) are commonly used to assess cognitive abilities, though it is unclear whether face processing RTs predict recognition ability beyond accuracy. In the current study, we examined accuracy and RT on a widely used face matching assessment modified to collect meaningful RT data, the computerized Benton Facial Recognition Test (BFRT-c), and measured whether RTs predicted face recognition ability and developmental prosopagnosia (DP) vs. control group membership. 62 controls and 36 DPs performed the BFRT-c as well as validated measures of face recognition ability: the Cambridge Face Memory Test (CFMT) and a Famous Faces Memory Test (FFMT). In controls, BFRT-c accuracy robustly predicted CFMT (r = .49, < .001), FFMT (r = .43, < .001), and a CFMT-FFMT composite (r = .54, < .001), whereas BFRT-c RT was not significantly associated with these measures (all r's .21). We also found that BFRT-c accuracy significantly differed between DPs and controls, but RT failed to differentiate the groups.
Topics: Facial Recognition; Humans; Pattern Recognition, Visual; Prosopagnosia; Reaction Time; Recognition, Psychology
PubMed: 36202620
DOI: 10.1080/02643294.2022.2114824 -
Journal of Autism and Developmental... Dec 2023Autism traits are common exclusionary criteria in developmental prosopagnosia (DP) studies. We investigated whether autism traits produce qualitatively different face...
Autism traits are common exclusionary criteria in developmental prosopagnosia (DP) studies. We investigated whether autism traits produce qualitatively different face processing in 43 DPs with high vs. low autism quotient (AQ) scores. Compared to controls (n = 27), face memory and perception were similarly deficient in the high- and low-AQ DPs, with the high-AQ DP group additionally showing deficient face emotion recognition. Task-based fMRI revealed reduced occipito-temporal face selectivity in both groups, with high-AQ DPs additionally demonstrating decreased posterior superior temporal sulcus selectivity. Resting-state fMRI showed similar reduced face-selective network connectivity in both DP groups compared with controls. Together, this demonstrates that high- and low-AQ DP groups have very similar face processing deficits, with additional facial emotion deficits in high-AQ DPs.
Topics: Humans; Facial Recognition; Prosopagnosia; Autistic Disorder; Autism Spectrum Disorder; Magnetic Resonance Imaging; Pattern Recognition, Visual
PubMed: 36173532
DOI: 10.1007/s10803-022-05705-w -
Psychonomic Bulletin & Review Feb 2023Face recognition is strongly influenced by the processing of orientation structure in the face image. Faces are much easier to recognize when they are filtered to...
Face recognition is strongly influenced by the processing of orientation structure in the face image. Faces are much easier to recognize when they are filtered to include only horizontally oriented information compared with vertically oriented information. Here, we investigate whether preferences for horizontal information in faces are related to face recognition abilities in a typical sample (Experiment 1), and whether such preferences are lacking in people with developmental prosopagnosia (DP; Experiment 2). Experiment 1 shows that preferences for horizontal face information are linked to face recognition abilities in a typical sample, with weak evidence of face-selective contributions. Experiment 2 shows that preferences for horizontal face information are comparable in control and DP groups. Our study suggests that preferences for horizontal face information are related to variations in face recognition abilities in the typical range, and that these preferences are not aberrant in DP.
Topics: Humans; Facial Recognition; Prosopagnosia; Recognition, Psychology; Pattern Recognition, Visual
PubMed: 36002717
DOI: 10.3758/s13423-022-02163-4 -
Journal of Neurosurgery. Case Lessons Sep 2021Disruptions of the inferior longitudinal fasciculus (ILF) in the nondominant temporal lobe can lead to the rare but significant higher visual-processing disturbance of...
BACKGROUND
Disruptions of the inferior longitudinal fasciculus (ILF) in the nondominant temporal lobe can lead to the rare but significant higher visual-processing disturbance of prosopagnosia. Here, the authors describe a 57-year-old right hand-dominant female with a large breast cancer brain metastasis in the right temporal lobe who underwent resection and subsequent Gamma Knife radiosurgery. She presented with difficulty with facial recognition, but following surgical intervention, the prosopagnosia became more profound.
OBSERVATIONS
Even in nondominant cortex, significant deficits can arise when operating near higher visual-processing centers, including the ILF.
LESSONS
This case highlights the utility of imaging-based tractography obtained from preoperative imaging for resective surgical planning even when operating in areas that do not involve what is traditionally considered elegant areas of the brain. To optimize neurological outcomes in metastatic tumor resection, awareness and diffusion tensor imaging of neighboring, displaced white matter tracts may prevent permanent deficits in higher visual processing.
PubMed: 35855187
DOI: 10.3171/CASE21313 -
Journal of Neurosurgery. Case Lessons Sep 2021Prosopagnosia is a rare neurological condition characterized by the impairment of face perception with preserved visual processing and cognitive functioning and is...
Prosopagnosia following nonlanguage dominant inferior temporal lobe low-grade glioma resection in which the inferior longitudinal fasciculus was disrupted preoperatively: illustrative case.
BACKGROUND
Prosopagnosia is a rare neurological condition characterized by the impairment of face perception with preserved visual processing and cognitive functioning and is associated with injury to the fusiform gyrus and inferior longitudinal fasciculus (ILF). Reports of this clinical impairment following resection of right temporal lobe diffuse gliomas in the absence of contralateral injury are exceedingly scarce and not expected as a complication of surgery.
OBSERVATIONS
The authors describe the case of a young female patient found to have an incidental diffuse glioma in the right inferior temporal lobe despite evidence of preoperative ILF disruption by the tumor. Following resection of the lesion, despite the preoperative disruption to the ILF by the tumor, the patient developed prosopagnosia. There was no evidence of contralateral, left-sided ILF injury.
LESSONS
Given the significant functional impairment associated with prosopagnosia, neurosurgeons should be aware of the exceedingly rare possibility of a visual-processing deficit following unilateral and, in this case, right-sided inferior temporal lobe glioma resections. More investigation is needed to determine whether preoperative testing can determine dominance of facial-processing networks for patients with lesions in the right inferior posterior temporooccipital lobe and whether intraoperative mapping could help prevent this complication.
PubMed: 35855186
DOI: 10.3171/CASE21277 -
Surgical Neurology International 2022Prosopagnosia is a rare form of apraxia, in which a person has normal memory and vision, but has impaired cognition of human faces that are manifested through symptoms...
BACKGROUND
Prosopagnosia is a rare form of apraxia, in which a person has normal memory and vision, but has impaired cognition of human faces that are manifested through symptoms such as not being able to recognize the face of a familiar person, one has known or not being able to remember the face of a person. Here, we report the case of a patient with transient prosopagnosia associated with brain metastasis from epidermal growth factor receptor (EGFR)-mutated lung adenocarcinoma who was treated with tyrosine kinase inhibitors (TKIs).
CASE DESCRIPTION
A 52-year-old right-handed man with lung adenocarcinoma was introduced to our department because brain metastasis. On admission, he complained that he could not recognize his wife's face, but he could recall her face based on her voice. MRI revealed a right temporo-occipital enhancing lesion with perifocal edema and dissemination that were indicative of brain metastasis from lung adenocarcinoma. Two weeks after open biopsy, he was started on TKI therapy with osimertinib at a dosage of 80 mg/day. An MRI scan taken 1 month later revealed shrinkage of the metastasis. In addition, he had recovered from transient prosopagnosia and returned to normal life.
CONCLUSION
In this study, the TKI osimertinib was administered to a patient with brain metastasis of EGFR-mutated lung adenocarcinoma who presented with prosopagnosia, and the patient's lesion shrunk and his symptoms were reversed with this treatment.
PubMed: 35855154
DOI: 10.25259/SNI_500_2022 -
Neuropsychologia Sep 2022Many studies have attempted to identify the perceptual underpinnings of developmental prosopagnosia (DP). The majority have focused on whether holistic and configural...
Many studies have attempted to identify the perceptual underpinnings of developmental prosopagnosia (DP). The majority have focused on whether holistic and configural processing mechanisms are impaired in DP. However, previous work suggests that there is substantial heterogeneity in holistic and configural processing within the DP population; further, there is disagreement as to whether any deficits are face-specific or reflect a broader perceptual deficit. This study used a data-driven approach to examine whether there are systematic patterns of variability in DP that reflect different underpinning perceptual deficits. A group of individuals with DP (N = 37) completed a cognitive battery measuring holistic/configural and featural processing in faces and non-face objects. A two-stage cluster analysis on data from the Cambridge Face Perception Test identified two subgroups of DPs. Across several tasks, the first subgroup (N = 21) showed typical patterns of holistic/configural processing (measured via inversion effects); the second (N = 16) was characterised by reduced or abolished inversion effects compared to age-matched control participants (N = 91). The subgroups did not differ on tasks measuring upright face matching, object matching, non-face holistic processing, or composite effects. These findings indicate two separable pathways to face recognition impairment, one characterised by impaired configural processing and the other potentially by impaired featural processing. Comparisons to control participants provide some preliminary evidence that the deficit in featural processing may extend to some non-face stimuli. Our results demonstrate the utility of examining both the variability between and consistency across individuals with DP as a means of illuminating our understanding of face recognition in typical and atypical populations.
Topics: Facial Recognition; Humans; Pattern Recognition, Visual; Photic Stimulation; Prosopagnosia; Recognition, Psychology
PubMed: 35839963
DOI: 10.1016/j.neuropsychologia.2022.108332 -
Cortex; a Journal Devoted To the Study... Sep 2022Developmental prosopagnosia (DP) is a neurodevelopmental condition characterized by lifelong face recognition difficulties. To date, it remains unclear whether or not...
Developmental prosopagnosia (DP) is a neurodevelopmental condition characterized by lifelong face recognition difficulties. To date, it remains unclear whether or not individuals with DP experience impaired recognition of facial expressions. It has been proposed that DPs may have sufficient perceptual ability to correctly interpret facial expressions when tasks are relatively easy (e.g., the stimuli are unambiguous and viewing conditions are optimal), but exhibit subtle impairments when tested under more challenging conditions. In the present study, we sought to take advantage of the COVID-19 pandemic to test this view. It is well-established that the surgical-type masks worn during the pandemic hinder the recognition and interpretation of facial emotion in typical participants. Relative to typical participants, we hypothesized that DPs may be disproportionately impaired when asked to interpret the facial emotion of people wearing face masks. We compared the ability of 34 DPs and 60 age-matched typical controls to recognize facial emotions i) when the whole face is visible, and ii) when the lower portion of the face is covered with a surgical mask. When expression stimuli were viewed without a mask, the DPs and typical controls exhibited similar levels of performance. However, when expression stimuli were shown with a mask, the DPs showed signs of subtle expression recognition deficits. The DPs were particularly prone to mislabeling masked expressions of happiness as emotion neutral. These results add to a growing body of evidence that under some conditions, DPs do exhibit subtle deficits of expression recognition.
Topics: COVID-19; Facial Expression; Facial Recognition; Humans; Pandemics; Prosopagnosia; Recognition, Psychology
PubMed: 35728295
DOI: 10.1016/j.cortex.2022.05.008 -
Frontiers in Neurology 2022Creutzfeldt-Jakob disease (CJD) is a rare form of rapidly progressive, neurodegenerative disease that results from the misfolding and accumulation of an aberrant,...
Creutzfeldt-Jakob disease (CJD) is a rare form of rapidly progressive, neurodegenerative disease that results from the misfolding and accumulation of an aberrant, disease-associated prion protein (PrPD). CJD affects 1-1.5 cases per million per year with the sporadic-type accounting for an estimated 85% of these cases. Sporadic CJD (sCJD) is further subdivided into five subtypes based on genetic polymorphisms; the rarest subtype, sCJDVV1, occurs at a rate of 1 case per one-hundredth million population per year. Clinical characteristics of the sCJDVV1 subtype have been reported to show, early age of onset (44 years), average disease duration of 21 months, absent PSWCs on electroencephalography (EEG), and MRI hyperintensities in the cerebral cortex with usual negative signal in the basal ganglia or thalamus. We present a case of the sCJDVV1 subtype with uncommon features. Contrary to current data on sCJDVV1, our patient presented with an unusual age at onset (61 years) and longer disease duration (32 months). The highly sensitive and specific real-time quaking-induced conversion (RT-QuIC) assay was negative. Presenting clinical symptoms included paranoid thoughts and agitation, rapidly progressive memory decline, prosopagnosia, and late development of myoclonus and mutism. Other findings showed positive antithyroid peroxidase antibodies (anti-TPO), and absent PSWCs on EEG. High-dose steroid therapy treatment was administered based on positive anti-TPO findings, which failed to elicit any improvement and the patient continued to decline. To our knowledge, only four cases with the sCJDVV1 subtype, including our patient, have been reported to have a negative result on RT-QuIC. This may suggest varied sensitivity across sCJD subtypes. However, given the rarity of our patient's subtype, and the relatively novel RT-QuIC, current data are based on a small number of cases and larger cohorts of confirmed VV1 cases with RT-QuIC testing need to be reported.
PubMed: 35614914
DOI: 10.3389/fneur.2022.875370