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Journal of Clinical Neuroscience :... Feb 2024Anosognosia, a neurological condition, involves a lack of awareness of one's neurological or psychiatric deficits. Anton Syndrome (AS), an unusual form of anosognosia,... (Review)
Review
INTRODUCTION
Anosognosia, a neurological condition, involves a lack of awareness of one's neurological or psychiatric deficits. Anton Syndrome (AS), an unusual form of anosognosia, manifests as bilateral vision loss coupled with denial of blindness. This systematic review delves into 64 studies encompassing 72 AS cases to explore demographics, clinical presentations, treatments, and outcomes.
MATERIALS AND METHODS
The study rigorously followed PRISMA guidelines, screening PubMed, Google Scholar, and Scopus databases without timeframe limitations. Only English human studies providing full text were included. Data underwent thorough assessment, examining patient demographics, etiological variables, and treatment modalities.
RESULTS
Sixty-four studies met the stringent inclusion criteria. Examining 72 AS cases showed a median age of 55 (6 to 96 years) with no gender preference. Hypertension (34.7 %) and visual anosognosia (90.3 %) were prevalent. Stroke (40.3 %) topped causes. Management included supportive (30.6 %) and causal approaches (30.6 %). Improvement was seen in 45.8 %, unchanged in 22.2 %, and deterioration in 11.1 %. Anticoagulation correlated with higher mortality (p < 0.05).
DISCUSSION
AS, an unusual manifestation of blindness, stems mainly from occipital lobe damage, often due to cerebrovascular incidents. The syndrome shares features with Dide-Botcazo syndrome and dates back to Roman times. Its causes range from strokes to rare conditions like multiple sclerosis exacerbation. Accurate diagnosis involves considering clinical presentations and imaging studies, distinguishing AS from similar conditions.
CONCLUSION
This comprehensive review sheds light on AS's complex landscape, emphasizing diverse etiologies, clinical features, and treatment options. Tailored treatments aligned with individual causes are crucial. The study's findings caution against blanket anticoagulation therapy, suggesting a nuanced approach. Further research is pivotal to refine diagnostics and optimize care for AS individuals.
Topics: Humans; Middle Aged; Aged; Aged, 80 and over; Blindness, Cortical; Brain; Stroke; Vision Disorders; Agnosia
PubMed: 38262263
DOI: 10.1016/j.jocn.2024.01.014 -
Cortex; a Journal Devoted To the Study... Jul 2024The goal of this preregistered scoping review is to create an overview of the research on developmental prosopagnosia (DP). Through analysis of all empirical studies of... (Review)
Review
The goal of this preregistered scoping review is to create an overview of the research on developmental prosopagnosia (DP). Through analysis of all empirical studies of DP in adults, we investigate 1) how DP is conceptualized and defined, 2) how individuals are classified with DP and 3) which aspects of DP are investigated in the literature. We reviewed 224 peer-reviewed studies of DP. Our analysis of the literature reveals that while DP is predominantly defined as a lifelong face recognition impairment in the absence of acquired brain injury and intellectual/cognitive problems, there is far from consensus on the specifics of the definition with some studies emphasizing e.g., deficits in face perception, discrimination and/or matching as core characteristics of DP. These differences in DP definitions is further reflected in the vast heterogeneity in classification procedures. Only about half of the included studies explicitly state how they classify individuals with DP, and these studies adopt 40 different assessment tools. The two most frequently studied aspects of DP are the role of holistic processing and the specificity of face processing, and alongside a substantial body of neuroimaging studies of DP, this paints a picture of a research field whose scientific interests and aims are rooted in cognitive neuropsychology and neuroscience. We argue that these roots - alongside the heterogeneity in DP definition and classification - may have limited the scope and interest of DP research unnecessarily, and we point to new avenues of research for the field.
Topics: Prosopagnosia; Humans; Facial Recognition; Recognition, Psychology
PubMed: 38795651
DOI: 10.1016/j.cortex.2024.04.011 -
Nursing Children and Young People Jul 2023Prosopagnosia or 'face blindness' is the inability to recognise people's faces. There are two types: congenital or developmental prosopagnosia, which is the most common,...
Prosopagnosia or 'face blindness' is the inability to recognise people's faces. There are two types: congenital or developmental prosopagnosia, which is the most common, and acquired prosopagnosia, which may occur secondary to brain tumours, stroke or other brain disorders. The authors of this article explored if mask wearing as a result of the restrictions imposed by the coronavirus disease 2019 (COVID-19) pandemic may affect social and developmental outcomes in children, including the development of prosopagnosia. Limited research on this topic is available and, although some relevant publications were found, no definitive evidence of mask-induced prosopagnosia in children was identified. However, nurses should be aware of this issue and discuss coping strategies to support children with the condition. Longitudinal studies on outcomes in children from different age groups who grew up during the COVID-19 pandemic will provide further insight.
Topics: Child; Humans; Prosopagnosia; Pandemics; Recognition, Psychology; Child Health; COVID-19; Blindness
PubMed: 36688257
DOI: 10.7748/ncyp.2023.e1480 -
Neuro-ophthalmology (Aeolus Press) 2024Vision specialists will benefit from increased awareness of posterior cortical atrophy (PCA) syndrome. Failure to adequately identify the chief complaint as a visual...
Vision specialists will benefit from increased awareness of posterior cortical atrophy (PCA) syndrome. Failure to adequately identify the chief complaint as a visual symptom may lead to incorrect diagnosis or diagnostic delay. A previously healthy, 59-year-old woman presented with a 5-year history of 'losing her stuff'. Upon psychiatric and neuro-ophthalmological evaluation, this symptom was better recognised as a feature of visual agnosia and simultanagnosia. She also presented with multiple previously unrecognised symptoms indicative of higher visual processing dysfunction, such as alexia without agraphia, ocular motor apraxia, optic ataxia, prosopagnosia, akinetopsia and topographagnosia, so further assessment to investigate for PCA was carried out. After a work-up including cognitive assessment, brain structural/functional imaging, and laboratory tests she was diagnosed with visual-variant Alzheimer's disease. Patients with PCA merit a detailed review of their symptoms, as well as the use of office tests such as cognitive evaluation tools, different types of perimetry, colour vision tests, and non-delayed psychiatric consultation for correct management and assessment. This report will emphasise five key aspects to be considered when evaluating patients with PCA.
PubMed: 38357623
DOI: 10.1080/01658107.2023.2257311 -
Orthopaedic Surgery Oct 2023Composite tissue loss involving the distal finger pulp and the nail is a common but challenging finger injury to restore. This study introduces a reconstruction...
OBJECTIVE
Composite tissue loss involving the distal finger pulp and the nail is a common but challenging finger injury to restore. This study introduces a reconstruction procedure for a distal finger pulp and nail defect using a partial toenail flap transfer.
METHODS
Twenty digits, including 16 thumbs, two index fingers, and two middle fingers, with composite soft tissue defects were treated with a partial toenail flap transfer from October 2015 to January 2020. Shortening revision of the great toe phalanx, a V-Y advancement flap of the toe pulp, and a local pedicle flap from a second toe transfer were used to cover the donor sites, and no skin grafts were required. Functionality was evaluated using the validated Spanish version of the Quick-DASH scale. The aesthetics of both the reconstructed and donor sites were evaluated using the Vancouver Scar Scale (VSS). The static two-point discrimination (2-PD) of the finger pulp was used as a measure of tactile agnosia.
RESULTS
All donor site wounds healed well. The average follow-up time was 23.6 months (6-39 months). The mean Quick-DASH functional score was 7.1. The VSS scores were 4.02 ± 0.29 and 4.00 ± 0.38 for the reconstructed and donor sites, respectively. The static 2-PD of finger pulp was 4.5 ± 0.76 mm. The patients were satisfied with finger motion, sensory function, and aesthetic contour.
CONCLUSIONS
Partial toenail flap transfer is the recommended treatment to regain motion, sensation, function, and a satisfactory aesthetic appearance when considering repairing a composite soft tissue distal finger defect with accompanying loss of the perionychium, particularly in the thumb, index finger, or middle finger.
PubMed: 37644638
DOI: 10.1111/os.13829 -
Neuropsychologia Oct 2023Despite 25 years of research on the topic, there is still no consensus on whether prism adaptation is an effective therapy for visuospatial neglect. We have addressed... (Meta-Analysis)
Meta-Analysis Review
Despite 25 years of research on the topic, there is still no consensus on whether prism adaptation is an effective therapy for visuospatial neglect. We have addressed this question through a meta-analysis of the most well-controlled studies on the topic. Our main meta-analytic model included studies with a placebo/sham/treatment-as-usual control group from which data from right hemisphere stroke patients and left-sided neglect could be aggregated. The short-term treatment effects on the two commonly used standard tests for neglect, the conventional Behavioural Inattention Test (BIT-C) and cancellation test scores were combined into one random effect model justified by the fact that 89% of the BIT-C score is determined by cancellation tasks. With this approach, we were able to obtain a larger and more homogeneous dataset than previous meta-analyses: sixteen studies including 430 patients. No evidence for beneficial effects of prism adaptation was found. The secondary meta-analysis including data from the Catherine Bergego Scale, a functional measure of activities of daily living, also found no evidence for the therapeutic effects of prism adaptation, although half as many studies were available for this analysis. The results were consistent after the removal of influential outliers, after studies with high risk-of-bias were excluded, and when an alternative measure of effect size was considered. These results do not support the routine use of prism adaptation as a therapy for spatial neglect.
Topics: Humans; Activities of Daily Living; Adaptation, Physiological; Perceptual Disorders; Stroke; Agnosia
PubMed: 37149126
DOI: 10.1016/j.neuropsychologia.2023.108566 -
Frontiers in Neurology 2023Loss of time is a major obstacle to efficient stroke treatment. Our telestroke path intends to optimize prehospital triage using a video link connecting ambulance...
Real-time video analysis allows the identification of large vessel occlusion in patients with suspected stroke: feasibility trial of a "telestroke" pathway in Northwestern Switzerland.
BACKGROUND AND AIM
Loss of time is a major obstacle to efficient stroke treatment. Our telestroke path intends to optimize prehospital triage using a video link connecting ambulance personnel and a stroke physician. The objectives were as follows: (1) To identify patients suffering a stroke and (2) in particular large vessel occlusion (LVO) strokes as candidates for endovascular treatment. We have chosen the Rapid Arterial Occlusion Evaluation (RACE) scale for this purpose.
METHODS
This analysis aimed to verify the feasibility of prehospital stroke identification by video assessment. In this prospective telestroke cohort study, we included 97 subjects, in which the RACE score (items: facial palsy, arm and leg motor function, head and gaze deviation, and aphasia or agnosia) was applied, and the assessment videotaped by a trained member of the Emergency Medical Services (EMS) in the field using a mobile device. Each recorded patient video was independently assessed by three experienced stroke physicians from a certified stroke center and compared to the neuroimaging gold standard. Within this feasibility study, the stroke code was not altered by the outcome of the RACE assessment, and all patients underwent the standard procedures within the emergency unit.
RESULTS
We analyzed 97 patients (median age 78 years, 53% women), of whom 51 (52.6%) suffered an acute stroke, 12 (23.5%) of which were due to an LVO and 46 patients had symptoms mimicking a stroke. The sensitivity of stroke identification was 77.8%, and specificity was 53.6%. In regard to the identification of an LVO, sensitivity was 69.4% and specificity was 84.3%. The inter-rater agreement in the RACE-score assessment was ICC = 0.82 (intraclass-correlation coefficient).
CONCLUSION
These results confirm our hypothesis that the local telestroke concept is feasible. It allows correct (i) stroke and (ii) LVO identification in the majority of the cases and thus has the potential to assist in efficient prehospital triage.
PubMed: 37941577
DOI: 10.3389/fneur.2023.1232401 -
The International Tinnitus Journal Mar 2024Deep functional and structural neuroimaging of a series of Gerstmann's syndrome patients required high accuracy, and our results avoided false overlaps of heterogeneous...
Deep functional and structural neuroimaging of a series of Gerstmann's syndrome patients required high accuracy, and our results avoided false overlaps of heterogeneous brain lesions by handling each case of our study subjects separately as an individual case regarding functional and neuroimaging tests. Six patients with Gerstmann tetrad (one with dominant acalculia, one with dominant left and right disorientation, two with writing disabilities and two with finger agnosia) and 6 control subjects with close ages were recruited in the current study. In the main phase, we assessed brain activation in response to experimental and interventional settings using neuroimaging techniques (FMRI-Functional Magnetic Resonance Imagingwhere twelve pictures were taken on a Dell inspiration 3T all-body scanner with sequences of echo pictures, 80o angled, TE 35 ms) of the subject's brain to declare lesions existence and locations that might result in one of the four cognitive impairment domains of Gerstman's syndrome tetrad. We assessed statistically significant differences of patient images vs. control images as well as the images of patients presenting specific symptomatic cognitive dysfunction domain vs. the images of patients presenting the three other domains. Neuroimages were analyzed using multiple databases such as T1 weighted and free sequence types. Gerstmann's syndrome is mainly connected to injury in the dominant parietal lobe, so images comparisons and analysis were only restricted to the left parietal lobe region. P values <0.05were only considered as statistically significant difference in comparisons of functional tests time and accuracy of patients vs. in addition to comparisons of brain images parameters of patient group vs. control group and specific symptomatic domain patients vs. other symptomatic domains patients. Regarding functional testing, Patients group took significantly higher time compared to control group. Regarding brain images parameters, patients in each domain showed significantly different lesions compared to other domains. Moreover, control subjects showed no lesions in the left parietal lobe compared to significant lesions in the patient groups. These results oppose the theory of Gerstmann that a common brain structural injury may result in the combination of all of the four symptomatic dysfunction domains. This may be due to the fact that Gerstmann examined incomplete cases which represent a considerable criticism to his scientific basis. Moreover, he excluded patients with speech difficulties and apraxia.
Topics: Male; Humans; Gerstmann Syndrome; Case-Control Studies; Brain; Magnetic Resonance Imaging; Speech Disorders
PubMed: 38507641
DOI: 10.5935/0946-5448.20230038 -
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 -
BMC Neurology Jul 2023Creutzfeldt-Jakob disease (CJD), is a deadly degenerative condition of the central nervous system marked by rapidly progressive dementia. Magnetic resonance imaging...
BACKGROUND
Creutzfeldt-Jakob disease (CJD), is a deadly degenerative condition of the central nervous system marked by rapidly progressive dementia. Magnetic resonance imaging (MRI) abnormalities in the cerebral cortex, basal ganglia, thalamus, and cerebellum could indicate severe acute diseases caused by a variety of factors. Although their MRI patterns may resemble those of CJD, clinical history, additional MRI findings, and laboratory testing are all necessary to provide a reliable difference. Here, we report a misdiagnosed case of probable VV1 subtype of sporadic CJD (sCJD) in which follow-up MRI supported the diagnosis.
CASE PRESENTATION
A 41-year-old male patient attended the Neuropsychiatry Department with rapidly progressive dementia, akinetic mutism, and difficulty walking and speaking. His problem began with forgetfulness, disorganized behavior, and disorganized speech 7 months earlier which progressed rapidly and was accompanied by aphasia, apraxia, agnosia, and akinetic mutism in the last 2 months. On neurologic examination, hypertonia, hyperreflexia, frontal ataxia, bradykinesia, gait apraxia, and aphasia were noted. Based on clinical features and rapid symptoms progression the likely diagnosis of CJD was suspected. MRI and electroencephalography (EEG) were advised. MRI revealed features of diffuse cortical injury of both cerebral hemispheres also involving bilateral corpus striatum with evidence of cerebral volume loss. EEG showed lateralized periodic theta slow waves on the right side. According to the CDC's diagnostic criteria for CJD, the diagnosis of probable sCJD was established. Supportive care and symptomatic treatment are provided for the patient. After a 1-month follow up the patient's condition deteriorated significantly. The time-lapse from the first reported symptom to death was about 13 months.
CONCLUSION
The need of addressing CJD in patients presenting with rapidly progressive dementia is highlighted in this case report. In the early stages of the disease, interpretation of MRI results might cause diagnostic difficulties; therefore, follow-up MRI is critical in obtaining the correct diagnosis.
Topics: Male; Humans; Adult; Creutzfeldt-Jakob Syndrome; Akinetic Mutism; Magnetic Resonance Imaging; Electroencephalography; Diagnostic Errors
PubMed: 37464286
DOI: 10.1186/s12883-023-03318-z