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Brain and Behavior Jul 2021Behavioral and cognitive changes can be observed across all Huntington disease (HD) stages. Our multicenter and retrospective study investigated the association between...
INTRODUCTION
Behavioral and cognitive changes can be observed across all Huntington disease (HD) stages. Our multicenter and retrospective study investigated the association between cognitive and behavioral scale scores in manifest HD, at three different yearly timepoints.
METHODS
We analyzed cognitive and behavioral domains by the Unified Huntington's Disease Rating Scale (UHDRS) and by the Problem Behaviors Assessment Short Form (PBA-s), at three different yearly times of life (t0 or baseline, t1 after one year, t2 after two years), in 97 patients with manifest HD (mean age 48.62 ± 13.1), from three ENROLL-HD Centers. In order to test the disease progression, we also examined patients' motor and functional changes by the UHDRS, overtime.
RESULTS
The severity of apathy and of perseveration/obsession was associated with the severity of the cognitive decline (p < .0001), regardless of the yearly timepoint. The score of irritability significantly and positively correlated with perseveration errors in the verbal fluency test at t0 (r = .34; p = .001), while the psychosis significantly and negatively correlated with the information processing speed at t0 (r = -.21; p = .038) and significantly and positively correlated with perseveration errors in the verbal fluency test at t1 (r = .35; p < .0001). The disease progression was confirmed by the significant worsening of the UHDRS-Total Motor Score (TMS) and of the UHDRS-Total Functional Capacity (TFC) scale score after two-year follow-up (p < .0001).
CONCLUSION
Although the progression of abnormal behavioral manifestations cannot be predicted in HD, the severity of apathy and perseveration/obsessions are significantly associated with the severity of the cognitive function impairment, thus contributing, together, to the disease development and to patients' loss of independence, in addition to the neurological manifestations. This cognitive-behavior pattern determines a common underlying deficit depending on a dysexecutive syndrome.
Topics: Adult; Cognition; Cross-Sectional Studies; Humans; Huntington Disease; Middle Aged; Problem Behavior; Retrospective Studies; Severity of Illness Index
PubMed: 34110097
DOI: 10.1002/brb3.2151 -
The Journal of Physician Assistant... Dec 2022The COVID-19 pandemic has disrupted almost every aspect of life globally, with higher education one of many direct targets. Institutions and educators have been faced...
The COVID-19 pandemic has disrupted almost every aspect of life globally, with higher education one of many direct targets. Institutions and educators have been faced with urgent crises of how to conduct business as usual while maintaining expectations of high standards and uncompromised goals. As physician assistant (PA) educators at Seton Hall University, we rallied and brainstormed approaches to daily instruction to keep students on track and faculty both effective and sane. We tapped technological resources offered by our university, learned countless new skills, and adapted classroom activities to online virtual platforms. Creativity and flexibility became the norm as we modified the way we did everything while remaining laser focused on the ultimate objectives. Our intention was to provide insight, motivation, and, at a minimum, an example of how to do things differently when faced with roadblocks. We were inspired by an editorial by Mary Jo Bondy, DHEd, MHS, PA-C, former CEO of the Physician Assistant Education Association, in which she called for collective innovation, collaboration, publication, and circulation of stories. With that charge, we assembled a detailed account of how a didactic team within our faculty maneuvered the challenges created by COVID-19, in hopes of adding to the arsenal of small triumphs in this uncharted territory.
Topics: Female; Humans; Physician Assistants; COVID-19; Pandemics; Faculty; Learning
PubMed: 36409244
DOI: 10.1097/JPA.0000000000000472 -
Cognition Nov 2020When humans and other animals make repeated choices, they tend to repeat previously chosen actions independently of their reward history. This paper locates the origin...
When humans and other animals make repeated choices, they tend to repeat previously chosen actions independently of their reward history. This paper locates the origin of perseveration in a trade-off between two computational goals: maximizing rewards and minimizing the complexity of the action policy. We develop an information-theoretic formalization of policy complexity and show how optimizing the trade-off leads to perseveration. Analysis of two data sets reveals that people attain close to optimal trade-offs. Parameter estimation and model comparison supports the claim that perseveration quantitatively agrees with the theoretically predicted functional form (a softmax function with a frequency-dependent action bias).
Topics: Animals; Decision Making; Humans; Reward
PubMed: 32679270
DOI: 10.1016/j.cognition.2020.104394 -
Scientific Reports Jun 2023Irritability, anger, and impulsivity have important associations with psychological well-being. However, studying the internal relationships between such emotional...
Irritability, anger, and impulsivity have important associations with psychological well-being. However, studying the internal relationships between such emotional constructs is challenging, largely because of the lack of precise operational definitions and extensively validated measurement tools. The aim of this study was to examine relationships between the above emotional constructs and how they relate to satisfaction with life and perceived negative impact on different life domains. Participants (N = 471) completed a self-report questionnaire online. Correlational analyses showed that higher levels of irritability and trait anger were associated with lower life satisfaction. Impulsivity displayed complex relationships with life satisfaction, with some aspects (sensation seeking) showing a positive relationship and others (urgency, lack of perseverance) showing a negative relationship. A two-factor Confirmatory Factor Analysis treating irritability and anger as separate constructs showed a better fit compared with a one-factor model, indicating that irritability and anger should be treated as separate constructs. An exploratory moderation analysis showed that higher irritability predicted increased anger only for participants scoring average to high on urgency (a facet of impulsivity). Our findings increase the understanding of the relationship between these dispositional constructs and supports the conceptualization of irritability and anger as related but distinct constructs.
Topics: Humans; Anger; Impulsive Behavior; Irritable Mood; Surveys and Questionnaires; Self Report
PubMed: 37369760
DOI: 10.1038/s41598-023-37557-4 -
Neuropsychology Mar 2021Right brain-damaged patients may show omissions and/or additional marks in target cancellation. The latter is classified as perseverative behavior and has been...
Right brain-damaged patients may show omissions and/or additional marks in target cancellation. The latter is classified as perseverative behavior and has been attributed to defective response inhibition or attentional disengagement deficit. This study aimed at (a) verifying that consecutive (immediate) and return (temporally distant) motor perseverations could be due to different mechanisms; (b) investigating the relationships among different types of perseveration (e.g., consecutive, return, scribble), spatial neglect and the impairment in specific components of executive functioning. Seventeen right brain-damaged patients underwent letter, star, bell, and apple cancellation tasks. A global index for each type of perseveration found and Mean Position of Hits, as a neglect index, were calculated. The following components of executive functioning were evaluated: motor programming (Frontal Assessment Battery [FAB] subtest), inhibitory control FAB, interference sensitivity (FAB and Stroop color-word interference test), set-shifting (Weigl sorting test, Phonemic/semantic alternate fluencies), and working memory (Backward Digit span). Ten patients out of 17 showed some degree of perseveration. Regularized linear regression analyses demonstrated that interference sensitivity and Stroop test performances were related to return perseverations and backward digit to scribble ones. No significant relationships were found for consecutive perseverations and between neglect and any type of perseverations. The present study showed that return perseverations might have a distinct etiology from consecutive ones, being related to an inability to update and shift between action programs according to the visual stimuli. A finer classification of perseverations could help in unveiling the neuropsychological mechanisms underlying each type of behavior. (PsycInfo Database Record (c) 2021 APA, all rights reserved).
Topics: Adult; Aged; Aged, 80 and over; Brain Injuries; Brain Ischemia; Brain Neoplasms; Cerebral Cortex; Executive Function; Female; Functional Laterality; Humans; Intracranial Hemorrhages; Male; Memory, Short-Term; Middle Aged; Neuropsychological Tests; Perceptual Disorders; Prospective Studies; Psychomotor Performance; Stroop Test
PubMed: 33970664
DOI: 10.1037/neu0000730 -
Epilepsy & Behavior : E&B Apr 2022In a comparative cross-sectional study, 26 adult individuals with clinically typical, genetically confirmed Dravet syndrome (DS) and an equal number of individuals with...
In a comparative cross-sectional study, 26 adult individuals with clinically typical, genetically confirmed Dravet syndrome (DS) and an equal number of individuals with early onset, problematic epilepsy, and intellectual disability (ID) of comparable severity were included. The aim of the study was to find out whether patients with DS could be clearly distinguished from the comparison group with regard to neurological and behavioral symptoms. Significant differences were found in that individuals with DS clearly more frequently exhibited a symptom cluster characterized by bradykinesia, hypomimia, hypophonia, (spastic) increased muscle tone, ataxia, sthenic perseveration, and a special interest in colors. To these symptoms must be added, according to the findings of previous examinations, mastication, camptocormia/antecollis on the one hand, and the tendency to visual hallucinations on the other hand, in order to define one neuropsychiatric phenotype of DS in adulthood. To these symptoms must be added, according to the findings of previous investigations, crouch gait with camptocormia/antecollis on the one hand, and the tendency to visual hallucinations on the other hand, in order to define one outlined neuropsychiatric phenotype of DS in adulthood.
Topics: Adult; Cross-Sectional Studies; Epilepsies, Myoclonic; Epileptic Syndromes; Humans; Phenotype; Spasms, Infantile
PubMed: 35203016
DOI: 10.1016/j.yebeh.2022.108601 -
JAMA Network Open Aug 2023Little is known about temporal trends in children's well-being and how the COVID-19 pandemic may have influenced the well-being of young Australians. Certain demographic...
IMPORTANCE
Little is known about temporal trends in children's well-being and how the COVID-19 pandemic may have influenced the well-being of young Australians. Certain demographic groups may be more vulnerable to experiencing declines in well-being.
OBJECTIVE
To examine well-being trends over 6 consecutive years among South Australian students and explore the influence of sociodemographic characteristics.
DESIGN, SETTING, AND PARTICIPANTS
Longitudinal analyses of annual (2017 to 2022) cross-sectional data of students in grades 4 through 9 (n = 40 392 to 56 897 per year) attending South Australian government schools from the Well-being and Engagement Collection (WEC) census.
EXPOSURES
Calendar year (2017-2022) and sociodemographic characteristics (sex, school grade, parental education, language spoken at home, residential region) from school enrollment records.
MAIN OUTCOMES AND MEASURES
Students self-reported life satisfaction, optimism, happiness, cognitive engagement, emotional regulation, perseverance, worry, and sadness.
RESULTS
Over 6 years (2017 to 2022), a total of 119 033 students (mean [SD] age, 12.1 y; 51.4% male) participated in this study. Most well-being measures declined over time, with consistent worsening of well-being from 2020 onward. For example, compared with 2017, sadness was 0.26 (95% CI, 0.25-0.27) points higher in 2020 (standardized mean difference [SMD], 0.27) and remained elevated by more than 0.26 points (SMD, 0.27) in 2021 and 2022. At almost every time point, greatest well-being was reported by students of male sex (except cognitive engagement and perseverance), in earlier school grades, with highest parental education, speaking a language other than English at home, and residing in outer regional and remote settings (for satisfaction, optimism, and emotional regulation). Sociodemographic differences in well-being were generally consistent over time; however, sex differences widened from 2020 for all indicators except cognitive engagement and perseverance. For example, between 2017 and 2022, sadness increased by 0.27 (95% CI, 0.25-0.29) more points among females than males (SMD, 0.28).
CONCLUSIONS AND RELEVANCE
In this longitudinal analysis of annual census data, there were downward trends in students' well-being, especially since 2020. The largest sociodemographic disparities were observed for students of female sex, those in later school grades, and those with lowest parental education. Urgent and equitable support for the well-being of all young people, particularly those facing disparity, is imperative.
Topics: Child; Female; Adolescent; Male; Humans; Australia; Cross-Sectional Studies; Pandemics; COVID-19; South Australia
PubMed: 37606925
DOI: 10.1001/jamanetworkopen.2023.30098 -
Disability and Rehabilitation.... May 2023Mainstream intelligent personal assistants (IPAs, e.g., Amazon Echo and Google Home) offer an unprecedented opportunity to enhance agency and wellbeing among vulnerable... (Randomized Controlled Trial)
Randomized Controlled Trial
PURPOSE
Mainstream intelligent personal assistants (IPAs, e.g., Amazon Echo and Google Home) offer an unprecedented opportunity to enhance agency and wellbeing among vulnerable groups across health and social care. However, unintended consequences and barriers to use are possible.
MATERIALS AND METHODS
We conducted a mixed-methods semi-randomized controlled trial among individuals with intellectual disability (ID), providing IPAs to an intervention group ( = 22), but not a control group ( = 22). Semi-structured interviews on device use and daily life were conducted with individuals with ID. Observation surveys were also collected from support staff. Key themes were identified using thematic analysis. We also collected quantitative agency and wellbeing data. A separate group of 40 individuals who had already received IPAs were additionally assessed, as well as their support staff.
RESULTS
Four themes were identified: (1) social value, (2) entertainment, (3) perceived agency, (4) challenges, perseverance, training/support needs. Opinions regarding IPAs were overwhelmingly positive. Most individuals qualitatively reported improved sense of agency and IPAs enabled many individuals to access features associated with wellbeing, but there was no significant change in the quantitative measure. Some individuals experienced challenges related to pronouncing and remembering IPA phrases; however, perseverance was common.
CONCLUSIONS
This study increases our understanding of the ways smart speakers can be used to enhance life quality among individuals with ID, and the nature of barriers faced. In conclusion, IPAs are cost-effective complementary support for vulnerable populations, but additional training is required to realize all potential benefits.Implications for rehabilitationIndividuals with intellectual disability (ID) in supported living are able to use mainstream smart-speakers to access features associated with supporting wellbeing and social communication.They also reported that smart speakers made them feel better able to do things for themselves.A number of individuals with ID and their support staff identified a need for training and support to get the most out of smart speakers in future.
Topics: Humans; Intellectual Disability; Quality of Life; Communication
PubMed: 33378247
DOI: 10.1080/17483107.2020.1864670 -
Physiology & Behavior Oct 2019Rodent self-grooming is a behavior that, besides its cleaning function, can be led by arousing experiences. A putative trait-like nature of this behavior was studied....
Rodent self-grooming is a behavior that, besides its cleaning function, can be led by arousing experiences. A putative trait-like nature of this behavior was studied. With the aim of providing information about how grooming behavior can reflect different behavioral processes, an individual differences approach was adopted. Fifty nine male Wistar-derived rats were submitted to five 30-min long behavioral tests. These tests were selected based on the behavioral processes they entangle. Elevated plus-maze (EPM): anxiety, exploration/habituation, arousal/dearousal; Marble burying (MB): active/passive coping; Operant extinction (EXT): frustration, perseveration; Conditioned fear context (CFC): fearfulness, active/passive coping; Novelty after restraint (NAR): stress induced behavior. Orthogonal (Varimax) factor analyses were performed within each test in order to select the most representative measures. To the selected variables from all tests a Direct Oblimin factor analysis was applied. A three factor solution was found after the application of the Cattell's scree test. This solution accounted for 44.2% of the variance. By looking at the loading variables, some conclusions could be drawn. On Factor 1 loaded time spent grooming in three tests and the measure of extinction resistance. We considered this factor to evidence a trait-like nature of grooming and a relationship between it and perseveration. On Factor 2 loaded freezing in the CFC, SAPs in the EPM and grooming duration in the EXT. We considered this factor to correspond to anxiety. On Factor 3, moderate to high loadings were found for crossings in the NAR test and for grooming duration in this test and in the EPM. A lower loading on this factor was also found for the number of buried marbles. We considered this factor as related to dearousal. The present results suggest important relationships both (1) within grooming measures recorded in different behavioral tests and (2) among grooming and other behaviors observed in the tests. These relationships are in accordance with a trait-like nature for self-grooming and shed some light to how grooming behavior interplays with anxiety, dearousal and perseveration.
Topics: Adaptation, Psychological; Animals; Anxiety; Arousal; Exploratory Behavior; Extinction, Psychological; Fear; Grooming; Individuality; Instinct; Male; Rats; Rats, Wistar; Stress, Psychological
PubMed: 31226313
DOI: 10.1016/j.physbeh.2019.112585 -
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke... Jan 2024
PubMed: 38246754
DOI: 10.3760/cma.j.cn115330-20231115-00216