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Cureus Sep 2023Kluver-Bucy Syndrome (KBS) is a rare neuropsychiatric disorder characterized by hyperorality, hypersexuality, bulimia, visual agnosia, and amnesia due to lesions... (Review)
Review
Kluver-Bucy Syndrome (KBS) is a rare neuropsychiatric disorder characterized by hyperorality, hypersexuality, bulimia, visual agnosia, and amnesia due to lesions affecting bilateral temporal lobes. It is attributed to a multitude of causes, including stroke, herpes simplex encephalitis, Alzheimer's disease, and head trauma. Current treatments for KBS include symptomatic management with antipsychotics, mood stabilizers, carbamazepine, and selective serotonin reuptake inhibitors. The bibliometric analysis was done to reflect the relevance and understanding of KBS in recent literature. The SCOPUS database was utilized to conduct a search for all articles with the terms "Kluver-Bucy" and "Kluver Bucy" from January 1, 1955 (the first available articles from the search) to February 1, 2023. The parameters included in this analysis were article title, citation numbers, citations per year, authors, institutions, publishing journals, country of origin, Source Normalized Impact per Paper, and Scopus CiteScore. Since 1937, when Kluver-Bucy Syndrome was first defined, the publications on KBS have steadily increased, with up to six publications a year in 2002. The most common institutions were SUNY Upstate Medical University, VA Medical Center, and the State University of New York (SUNY) System. Seven of these papers were published in . Almost 75% of the articles were published in journals of medicine and neuroscience. This is the first bibliometric analysis to evaluate the most influential publications about Kluver-Bucy Syndrome. A majority of the research is case-based and there is a dearth of clinical trials to identify the exact pathophysiology and physiotherapy management, possibly owing to the rarity of the disease. Our research suggests that there may be a significant overlap between Sanfilippo syndrome and KBS, suggesting that refined guidelines for establishing diagnosis may be required for children. Our study could bring a renewed interest in this field and lead to additional research focused on understanding the pathophysiology of KBS in order to promote the development of novel diagnostics and treatment.
PubMed: 37854727
DOI: 10.7759/cureus.45382 -
Neuropsychologia Nov 2023We present a comprehensive review of the rare syndrome visual form agnosia (VFA). We begin by documenting its history, including the origins of the term, and the first... (Review)
Review
We present a comprehensive review of the rare syndrome visual form agnosia (VFA). We begin by documenting its history, including the origins of the term, and the first case study labelled as VFA. The defining characteristics of the syndrome, as others have previously defined it, are then described. The impairments, preserved aspects of visual perception, and areas of brain damage in 21 patients who meet these defining characteristics are described in detail, including which tests were used to verify the presence or absence of key symptoms. From this, we note important similarities along with notable areas of divergence between patients. Damage to the occipital lobe (20/21), an inability to recognise line drawings (19/21), preserved colour vision (14/21), and visual field defects (16/21) were areas of consistency across most cases. We found it useful to distinguish between shape and form as distinct constructs when examining perceptual abilities in VFA patients. Our observations suggest that these patients often exhibit difficulties in processing simplified versions of form. Deficits in processing orientation and size were uncommon. Motion perception and visual imagery were not widely tested for despite being typically cited as defining features of the syndrome - although in the sample described, motion perception was never found to be a deficit. Moreover, problems with vision (e.g., poor visual acuity and the presence of hemianopias/scotomas in the visual fields) are more common than we would have thought and may also contribute to perceptual impairments in patients with VFA. We conclude that VFA is a perceptual disorder where the visual system has a reduced ability to synthesise lines together for the purposes of making sense of what images represent holistically.
Topics: Humans; Pattern Recognition, Visual; Visual Perception; Vision, Ocular; Visual Fields; Vision Disorders; Agnosia
PubMed: 37634886
DOI: 10.1016/j.neuropsychologia.2023.108666 -
Cell Death Discovery Mar 2024Pancreatic cancer is a malignant tumor of the digestive system. It is highly aggressive, easily metastasizes, and extremely difficult to treat. This study aimed to...
Pancreatic cancer is a malignant tumor of the digestive system. It is highly aggressive, easily metastasizes, and extremely difficult to treat. This study aimed to analyze the genes that might regulate pancreatic cancer migration to provide an essential basis for the prognostic assessment of pancreatic cancer and individualized treatment. A CRISPR knockout library directed against 915 murine genes was transfected into TB 32047 cell line to screen which gene loss promoted cell migration. Next-generation sequencing and PinAPL.py- analysis was performed to identify candidate genes. We then assessed the effect of serine/threonine kinase 11 (STK11) knockout on pancreatic cancer by wound-healing assay, chick agnosia (CAM) assay, and orthotopic mouse pancreatic cancer model. We performed RNA sequence and Western blotting for mechanistic studies to identify and verify the pathways. After accelerated Transwell migration screening, STK11 was identified as one of the top candidate genes. Further experiments showed that targeted knockout of STK11 promoted the cell migration and increased liver metastasis in mice. Mechanistic analyses revealed that STK11 knockout influences blood vessel morphogenesis and is closely associated with the enhanced expression of phosphodiesterases (PDEs), especially PDE4D, PDE4B, and PDE10A. PDE4 inhibitor Roflumilast inhibited STK11-KO cell migration and tumor size, further demonstrating that PDEs are essential for STK11-deficient cell migration. Our findings support the adoption of therapeutic strategies, including Roflumilast, for patients with STK11-mutated pancreatic cancer in order to improve treatment efficacy and ultimately prolong survival.
PubMed: 38461159
DOI: 10.1038/s41420-024-01890-y -
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 -
Neuropsychologia Jun 2024Facial identity recognition (FIR) is arguably the ultimate form of recognition for the adult human brain. Even if the term prosopagnosia is reserved for exceptionally... (Review)
Review
Facial identity recognition (FIR) is arguably the ultimate form of recognition for the adult human brain. Even if the term prosopagnosia is reserved for exceptionally rare brain-damaged cases with a category-specific abrupt loss of FIR at adulthood, subjective and objective impairments or difficulties of FIR are common in the neuropsychological population. Here we provide a critical overview of the evaluation of FIR both for clinicians and researchers in neuropsychology. FIR impairments occur following many causes that should be identified objectively by both general and specific, behavioral and neural examinations. We refute the commonly used dissociation between perceptual and memory deficits/tests for FIR, since even a task involving the discrimination of unfamiliar face images presented side-by-side relies on cortical memories of faces in the right-lateralized ventral occipito-temporal cortex. Another frequently encountered confusion is between specific deficits of the FIR function and a more general impairment of semantic memory (of people), the latter being most often encountered following anterior temporal lobe damage. Many computerized tests aimed at evaluating FIR have appeared over the last two decades, as reviewed here. However, despite undeniable strengths, they often suffer from ecological limitations, difficulties of instruction, as well as a lack of consideration for processing speed and qualitative information. Taking into account these issues, a recently developed behavioral test with natural images manipulating face familiarity, stimulus inversion, and correct response times as a key variable appears promising. The measurement of electroencephalographic (EEG) activity in the frequency domain from fast periodic visual stimulation also appears as a particularly promising tool to complete and enhance the neuropsychological assessment of FIR.
Topics: Humans; Facial Recognition; Neuropsychological Tests; Prosopagnosia; Recognition, Psychology; Electroencephalography
PubMed: 38522782
DOI: 10.1016/j.neuropsychologia.2024.108865 -
Frontiers in Endocrinology 2020Emotional deprivation can lead to growth faltering of infants and children. The mechanism(s) involved differ in that for infants, the major metabolic problem is... (Review)
Review
Emotional deprivation can lead to growth faltering of infants and children. The mechanism(s) involved differ in that for infants, the major metabolic problem is inadequate energy intake for growth. In young children, it is likely that the emotional deprivation causes a syndrome not only of growth faltering, but with bizarre behaviors, especially with regard to food: hoarding, gorging and vomiting, hyperphagia, drinking from the toilet, and eating from garbage pails. Other disturbed behaviors include, poor sleep, night wanderings, and pain agnosia. The pathophysiology appears to be reversible hypopituitarism, at least for the growth hormone and hypothalamic-pituitary- adrenal axes. The review begins with an historical perspective concerning stress, children and growth and then moves to the issue of hospitalism, where young infants failed to thrive (and died) due to inadequate stimulation and energy intake. Refeeding programs at the end of World Wars I and II noted that some children did not thrive despite an adequate energy intake. It appeared that in addition taking care of their emotional needs permitted super-physiologic (catch-up) growth. Next came the first notions from clinical investigation that hypopituitarism might be the mechanism of growth faltering. Studies that address this mechanism from a number of observational and clinical research studies are reviewed in depth to show that the hypopituitarism was relieved upon removal from the deprivational environment and occurred much too quickly to be due to adequate energy alone. These findings are then compared to those from malnourished children and adoptees from emerging countries, especially those from orphanages where their psychosocial needs were unmet despite adequate caloric intake. Together, these various conditions define one aspect of the field of psychoneuroendocrinology.
Topics: Affective Symptoms; Child; Failure to Thrive; Humans; Hypopituitarism
PubMed: 33117295
DOI: 10.3389/fendo.2020.596144 -
Cortex; a Journal Devoted To the Study... Apr 2024Developmental prosopagnosia (DP) is characterised by difficulties recognising face identities and is associated with diverse co-occurring object recognition...
Developmental prosopagnosia (DP) is characterised by difficulties recognising face identities and is associated with diverse co-occurring object recognition difficulties. The high co-occurrence rate and heterogeneity of associated difficulties in DP is an intrinsic feature of developmental conditions, where co-occurrence of difficulties is the rule, rather than the exception. However, despite its name, cognitive and neural theories of DP rarely consider the developmental context in which these difficulties occur. This leaves a large gap in our understanding of how DP emerges in light of the developmental trajectory of face recognition. Here, we argue that progress in the field requires re-considering the developmental origins of differences in face recognition abilities, rather than studying the end-state alone. In practice, considering development in DP necessitates a re-evaluation of current approaches in recruitment, design, and analyses.
Topics: Humans; Prosopagnosia; Facial Recognition; Visual Perception; Pattern Recognition, Visual
PubMed: 38460488
DOI: 10.1016/j.cortex.2024.02.006 -
Frontiers in Neuroinformatics 2021Deep neural networks, inspired by information processing in the brain, can achieve human-like performance for various tasks. However, research efforts to use these...
Deep neural networks, inspired by information processing in the brain, can achieve human-like performance for various tasks. However, research efforts to use these networks as models of the brain have primarily focused on modeling healthy brain function so far. In this work, we propose a paradigm for modeling neural diseases with deep learning and demonstrate its use in modeling posterior cortical atrophy (PCA), an atypical form of Alzheimer's disease affecting the visual cortex. We simulated PCA in deep convolutional neural networks (DCNNs) trained for visual object recognition by randomly injuring connections between artificial neurons. Results showed that injured networks progressively lost their object recognition capability. Simulated PCA impacted learned representations hierarchically, as networks lost object-level representations before category-level representations. Incorporating this paradigm in computational neuroscience will be essential for developing models of the brain and neurological diseases. The paradigm can be expanded to incorporate elements of neural plasticity and to other cognitive domains such as motor control, auditory cognition, language processing, and decision making.
PubMed: 34867256
DOI: 10.3389/fninf.2021.748370 -
European Review For Medical and... Jan 2022COVID-19 infection can cause impairments in many cognitive areas. The aim of the present study was to evaluate the cognitive functions of patients who had been infected...
OBJECTIVE
COVID-19 infection can cause impairments in many cognitive areas. The aim of the present study was to evaluate the cognitive functions of patients who had been infected with COVID-19.
PATIENTS AND METHODS
The demographic and infection-related characteristics of patients who had been infected with COVID-19 were determined. Their cranial magnetic resonance imaging (MRI) and electroencephalography (EEG) findings were recorded. The Mini-Mental State Evaluation (MMSE), clock drawing test, forward and backward digit span tests, visual memory test, and Frontal Assessment Battery were applied to the patients. Finger agnosia and ideomotor apraxia were also determined.
RESULTS
The study included 176 patients [100 female (56.8%), 76 male (43.2%), mean age 66.09±13.96 years]. About half of the patients were hospitalized for symptoms of COVID-19 infection (n=82, 46.6%). One third of these patients required intensive care (n=26, 14.8%). While 50 (45.9%) of the 109 patients diagnosed with dementia before infection were hospitalized, 32 (47.8%) of the 67 patients without a diagnosis of dementia required hospitalization (p=0.46). The most common neurological finding during COVID-19 infection was insomnia (n=36, 20.5%). The MMSE and visual memory test scores of the patients who were hospitalized for severe respiratory distress were lower than those whose treatment at home was completed (respectively 17.92±7.69/20.59±7.01, p=0.02; 2.53 ±1.73/3.69±2.80, p=0.01). The patients with moderate to severe cognitive impairment had significantly higher CRP levels at admission than the others (37.52±43.09/20.93±31.74, p=0.01, respectively).
CONCLUSIONS
Cognitive damage in COVID-19 infection may be caused by ACE receptor density in the pial, hippocampal, and amygdala areas. In addition, the reason why people with severe dementia have a milder infection might be explained by the atrophy in these areas.
Topics: Aged; Aged, 80 and over; C-Reactive Protein; COVID-19; Cognitive Dysfunction; Critical Care; Cross-Sectional Studies; Dementia; Electroencephalography; Female; Hospitalization; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Neuropsychological Tests; SARS-CoV-2; Severity of Illness Index; Sleep Initiation and Maintenance Disorders
PubMed: 35113443
DOI: 10.26355/eurrev_202201_27894 -
Frontiers in Rehabilitation Sciences 2023Benign paroxysmal positional vertigo (BPPV) is a common condition with disabling symptoms that is diagnosed and effectively treated at the bedside. Our encounter with...
INTRODUCTION
Benign paroxysmal positional vertigo (BPPV) is a common condition with disabling symptoms that is diagnosed and effectively treated at the bedside. Our encounter with patients experiencing prolonged BPPV who may not have received appropriate physical therapy prompted us to explore barriers to the diagnosis and treatment for BPPV among physical therapists, which has not been extensively investigated. We hypothesize that a potential barrier may be a lack of understanding of subtle symptoms of BPPV that deviate from the classical presentation. The gold standard for diagnosing definite BPPV is subjective dizziness or vertigo with nystagmus in response to positional testing. There are variants of BPPV including subjective BPPV (subjective dizziness or vertigo without nystagmus) and vestibular agnosia (nystagmus without subjective dizziness or vertigo) that do not meet the diagnostic criteria for definite BPPV but are equally responsive to the same repositioning maneuvers. The purpose of this project was to survey physical therapists for their understanding of BPPV including subjective BPPV and vestibular agnosia.
METHODS
A panel of experts created a 16-question survey, designed for physical therapists, with three categories: (1), inquiring if they treat persons with BPPV, (2) three clinical vignettes for definite BPPV, subjective BPPV, and BPPV with vestibular agnosia, and (3) demographic information. Data collection occurred at two large physical therapy meetings, one of which was a national professional meeting and the other was a professional continuing medical education course geared towards advancing vestibular rehabilitation skills.
RESULTS
There were 426 people who completed the survey, 364 of whom treat BPPV in their practice. In the first clinical vignette created to assess the respondents' understanding of definite BPPV, 229 (62%) of respondents would always assess a patient for BPPV based on complaints of a "room spinning" vertigo from head movement. When asked if the complaint was lingering "lightheadedness or feelings of imbalance" from head movement, only 158 (43%) reported they would perform positional testing to reassess. In the BPPV variant vignettes, 187 (51%) identified the patient with subjective BPPV as having BPPV and 305 (85%) identified the patient with vestibular agnosia as having BPPV.
DISCUSSION
The results of this survey demonstrate gaps in knowledge regarding BPPV across practice settings and experience, with opportunities to bridge these gaps to improve treatment for BPPV.
PubMed: 37662546
DOI: 10.3389/fresc.2023.1228453