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Frontiers in Psychiatry 2023Fahr's disease (FD) is a rare disorder, characterized by basal ganglia calcification and presenting with movement disorders, speech impairment, cognitive deficits, and...
Fahr's disease (FD) is a rare disorder, characterized by basal ganglia calcification and presenting with movement disorders, speech impairment, cognitive deficits, and neuropsychiatric symptoms. Psychotic disorders related to FD are barely described in the literature, and knowledge is missing concerning pathophysiology, course, and management. Here, we report on the long-term follow-up of a patient who had three acute episodes of FD-psychosis characterized by bizarre delusions and behavioral disorganization, without hallucinations. Genetic and metabolic causes of FD were ruled out. In all three episodes, olanzapine monotherapy rapidly and completely resolved psychosis, without inducing metabolic syndrome and extrapyramidal symptoms. In addition to the acute decompensations, the patient presented a tame, introverted, industrious, and perfectionistic personality, which we could interpret as the "" described for many other basal ganglia disorders. Moreover, bizarre appearance, reduced affectivity, abulia, concrete speech, and stiff motricity in the context of a mild asymmetric extrapyramidal syndrome characterized the mental status. The cognitive profile was initially marked by executive difficulties and partial agnosia, with an IQ of 86. In the course of 10 years, the patient suffered from an ischemic stroke in the left superior temporal gyrus, which provoked a decline in memory and executive functions, without any impact on the psychiatric picture. Antiphospholipid antibody syndrome emerged as the underlying cause; thus, for the first time in the literature, an overlap of FD and antiphospholipid antibody syndrome is described here. This case report stresses once more the need for better integration of psychiatry and neurology and for the investigation of secondary causes of late-onset psychosis.
PubMed: 38016060
DOI: 10.3389/fpsyt.2023.1268982 -
Cortex; a Journal Devoted To the Study... Sep 2023It has been suggested that Gerstmann's syndrome is the result of subcortical disconnection rather than emerging from damage of a multifunctional brain region within the...
It has been suggested that Gerstmann's syndrome is the result of subcortical disconnection rather than emerging from damage of a multifunctional brain region within the parietal lobe. However, patterns of white matter tract disconnection following parietal damage have been barely investigated. This single case study allows characterising Gerstmann's syndrome in terms of disconnected networks. We report the case of a left parietal patient affected by Gerstmann's tetrad: agraphia, acalculia, left/right orientation problems, and finger agnosia. Lesion mapping, atlas-based estimation of probability of disconnection, and DTI-based tractography revealed that the lesion was mainly located in the superior parietal lobule, and it caused disruption of both intraparietal tracts passing through the inferior parietal lobule (e.g., tracts connecting the angular, supramarginal, postcentral gyri, and the superior parietal lobule) and fronto-parietal long tracts (e.g., the superior longitudinal fasciculus). The lesion site appears to be located more superiorly as compared to the cerebral regions shown active by other studies during tasks impaired in the syndrome, and it reached the subcortical area potentially critical in the emergence of the syndrome, as hypothesised in previous studies. Importantly, the reconstruction of tracts connecting regions within the parietal lobe indicates that this critical subcortical area is mainly crossed by white matter tracts connecting the angular gyrus and the superior parietal lobule. Taken together, these findings suggest that this case study might be considered as empirical evidence of Gerstmann's tetrad caused by disconnection of intraparietal white matter tracts.
Topics: Humans; Gerstmann Syndrome; White Matter; Parietal Lobe; Brain; Agnosia
PubMed: 37478549
DOI: 10.1016/j.cortex.2023.05.016 -
Cortex; a Journal Devoted To the Study... Oct 2023People with aphantasia have a markedly impaired ability to form visual images in the mind's eye. Here, by testing people with and without aphantasia, we examine the...
People with aphantasia have a markedly impaired ability to form visual images in the mind's eye. Here, by testing people with and without aphantasia, we examine the relationship between visual imagery and face processing. We show that aphantasics have weaker face recognition than people with visual imagery, using both self-report (Prosopagnosia Index) and behavioural measures (Cambridge Face Memory Test). However, aphantasics nonetheless have a fully intact ability to construct facial composites from memory (i.e., composites produced using EFIT6 by aphantasics and imagers were rated as equally accurate in terms of their resemblance to a target face). Additionally, we show that aphantasics were less able than imagers to see the resemblance between composites and a target face, suggestive of potential issues with face matching (perception). Finally, we show that holistic and featural methods of composite construction using EFIT6 produce equally accurate composites. Our results suggest that face recognition, but not face composite construction, is facilitated by the ability to represent visual properties as 'pictures in the mind'. Our findings have implications for the study of aphantasia, and also for forensic settings, where face composite systems are commonly used to aid criminal investigations.
Topics: Humans; Facial Recognition; Imagery, Psychotherapy; Prosopagnosia; Recognition, Psychology; Self Report; Visual Perception
PubMed: 37597266
DOI: 10.1016/j.cortex.2023.06.015 -
Geriatrie Et Psychologie... Sep 2023Alzheimer's disease leads to an alteration of decision-making abilities which may increase risk-taking behaviours, particularly associated anosognosia. Anticipating the...
Alzheimer's disease leads to an alteration of decision-making abilities which may increase risk-taking behaviours, particularly associated anosognosia. Anticipating the progression of the disease raises a number of questions, particularly in relation to aging in place. Our qualitative study aimed to identify the arguments used by older patients with Alzheimer's disease when choosing a place to age. The study included 22 older adults, living at home, and diagnosed as mild dementia. The patients' arguments in favour of ageing in place were based mainly on the preservation of internal security, through the familiarity of places and relations as well as the maintenance of their independence and their lifestyle habits, allowing stability in their daily lives. Despite the identification of memory loss, the associated risks were minimized or hidden from the reflection on the choice of the place to age.
Topics: Humans; Aged; Alzheimer Disease; Independent Living; Memory Disorders; Agnosia
PubMed: 38093573
DOI: 10.1684/pnv.2023.1119 -
European Journal of Neurology Oct 2023This study was undertaken to assess the most sensitive combination of tests to detect peripersonal unilateral neglect (UN) after stroke.
BACKGROUND AND PURPOSE
This study was undertaken to assess the most sensitive combination of tests to detect peripersonal unilateral neglect (UN) after stroke.
METHODS
The present study is a secondary analysis of a previously reported multicentric study of 203 individuals with right hemisphere damage (RHD), mainly subacute stroke, 11 weeks postonset on average, and 307 healthy controls. A battery of seven tests, providing 19 age- and education-adjusted z-scores, were given: the bells test, line bisection, figure copying, clock drawing, overlapping figures test, and reading and writing. Statistical analyses used a logistic regression and a receiver operating characteristic (ROC) curve after adjustment on demographic variables.
RESULTS
A combination of four z-scores based on the following three tests provided good discrimination of patients with RHD from matched healthy controls: the starting point and the difference between the number of omissions on left and right sides from the bells test, rightward deviation in bisection of long lines (20 cm), and left-sided omissions in a reading task. The area under the ROC curve was 0.865 (95% confidence interval = 0.83-0.901), with sensitivity = 0.68, specificity = 0.95, accuracy = 0.85, positive predictive value = 0.90, and negative predictive value = 0.82.
CONCLUSIONS
The most sensitive and parsimonious combination of tests to detect UN after stroke relies on four scores from three simple tests (bells test, line bisection, and reading). Future study is warranted to assess its ability to account for the functional difficulties of UN in daily life in the patient's actual environment.
Topics: Humans; Perceptual Disorders; Stroke; Agnosia; Predictive Value of Tests; ROC Curve; Neuropsychological Tests; Functional Laterality
PubMed: 37405828
DOI: 10.1111/ene.15965 -
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 -
Parkinsonism & Related Disorders Jun 2024Anosognosia, defined as reduced awareness of one's deficit or symptom, is common in Huntington's disease (HD) and detectable at each disease stage. The impact of...
INTRODUCTION
Anosognosia, defined as reduced awareness of one's deficit or symptom, is common in Huntington's disease (HD) and detectable at each disease stage. The impact of anosognosia on self-reporting in HD populations is critical to understand given growing use of patient-reported outcomes in HD clinical care and research. We aimed to determine the influence of anosognosia on patient-reported outcome measures assessing psychiatric symptoms and quality of life in HD.
METHODS
We enrolled HD patients to complete a battery of patient-reported and rater-administered measures, including the Anosognosia Scale, at baseline and 6 months later. Patient-reported outcome measures included NeuroQoL short forms for depression, anxiety, satisfaction with social roles and activities, and positive affect and well-being and Patient-Reported Outcomes Measurement Information System short forms for emotional distress-anger and sleep-related impairment. Anosognosia Scale-Difference Score indexed patient-clinician agreement on patient motor, cognitive, and behavioral abilities. We conducted multivariable linear regression analyses to quantify the association of baseline anosognosia with 6-month patient-reported outcomes.
RESULTS
Of 79 patients with complete Anosognosia Scale data at baseline, 25 (31.6 %) met the scale's criterion for anosognosia. In the regression analyses, baseline Difference Score improved prediction of 6-month patient-reported outcomes for depression, anxiety, anger, and positive affect and well-being (χ(1) value range for likelihood ratio tests contrasting models with and without Difference Score: 13.1-20.9, p-values <0.001). Patients with more anosognosia self-reported less severe psychiatric symptoms and more positive affect and well-being.
CONCLUSION
Study results suggest that anosognosia influences patient-reported outcomes for psychiatric symptoms and quality of life in HD populations.
Topics: Humans; Huntington Disease; Male; Female; Middle Aged; Patient Reported Outcome Measures; Quality of Life; Adult; Agnosia; Aged; Depression
PubMed: 38614044
DOI: 10.1016/j.parkreldis.2024.106969 -
Acta Psychologica May 2024Developmental prosopagnosia (DP) is a condition that indicates the inability to recognize individuals by their faces from birth, without any history of brain damage. The...
Developmental prosopagnosia (DP) is a condition that indicates the inability to recognize individuals by their faces from birth, without any history of brain damage. The assessment of face recognition ability and diagnosis of DP involve the use of face tests such as the Cambridge Face Memory Test (CFMT) and the Cambridge Face Perception Test, along with self-reported measures like the 20-Item Prosopagnosia Index (PI20). Face recognition accuracy is affected by anxiety. However, previous studies on the relationship between face recognition ability and anxiety have not used the PI20 measure. This study aimed to investigate the relationship between self-reported measures of face recognition ability and anxiety tendencies among healthy young individuals for DP diagnosis and its implications. We used a face recognition test, involving the PI20, CFMT, Visual Perception Test for Agnosia-Famous Face Test (VPTA-FFT), and State-Trait Anxiety Inventory (STAI). We assessed the performance of 116 Japanese young adults (75 females, median age of 20.7 years, with a standard deviation of 1.2). Subsequently, we conducted a statistical analysis to examine the relationship between the outcomes of the face recognition tests and STAI scores using Pearson correlation analysis and single correlation coefficients. The results showed a positive correlation between state anxiety and PI20 (r = 0.308, p = 0.007), and a weak positive correlation was also observed between trait anxiety and PI20 (r = 0.268, p = 0.04). In contrast, there was no correlation between CFMT and VPTA-FFT with respect to STAI. The results of the hierarchical multiple regression analysis also suggested that the correlation between the performance on the PI20 (self-report) and objective measures of face recognition performance (the CFMT and the VPTA-FFT) are driven by differences in anxiety. This study is the first to explore the relationship between face recognition abilities and anxiety using the PI20 self-report measure. There are implications for future research on the diagnosis of DP and the relationship between anxiety and face recognition.
Topics: Female; Young Adult; Humans; Adult; Facial Recognition; Prosopagnosia; Recognition, Psychology; Anxiety; Self Report; Pattern Recognition, Visual
PubMed: 38537601
DOI: 10.1016/j.actpsy.2024.104237 -
PeerJ 2023Developmental prosopagnosia is a relatively common visuo-cognitive condition, characterised by impaired facial identity recognition. Impairment severity appears to...
Developmental prosopagnosia is a relatively common visuo-cognitive condition, characterised by impaired facial identity recognition. Impairment severity appears to reside on a continuum, however, it is unknown whether instances of milder deficits reflect the successful use of spontaneous (typical) face recognition strategies, or the application of extraneous compensatory cues to recognition. Here, we explore this issue in two studies. First, 23 adults with developmental prosopagnosia were asked about their use of spontaneous versus compensatory face recognition techniques in everyday life, using a series of closed- and open-ended questions. Second, the same participants performed a computerised famous face recognition task where they were asked to provide reasons why they could make any successful identifications. Findings from both studies suggest that people with developmental prosopagnosia can successfully, and quite frequently, use compensatory strategies to recognition, and that these cues support the majority of instances of preserved familiar face recognition. In contrast, 16 of the 23 participants were able to spontaneously recognise familiar faces on at least some occasions, but there were vast individual differences in frequencies of success. These findings have important implications for our conceptualisation of the condition, as well as for diagnostic practice.
Topics: Adult; Humans; Cues; Facial Recognition; Prosopagnosia; Recognition, Psychology; Male; Female; Middle Aged
PubMed: 37483961
DOI: 10.7717/peerj.15497 -
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