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This study set out to investigate, using qualitative methodology, the experiences of spouses of people with Amyotrophic Lateral Sclerosis (ALS) during the coronavirus pandemic, with particular focus on spouse distress and cognitive and behavioral change in people with ALS (pwALS). Qualitative semi-structured interviews of nine spouses of pwALS living in England were conducted between 11/09/2020 and 20/04/2021, focusing on spouses' perspectives of how their lives and the lives of pwALS were affected by the pandemic and related lockdowns. Interviews were subject to thematic analysis. Four superordinate themes were identified from the spouses' interviews: (i) pandemic behaviors, which encompassed accounts of cautious behavior, relaxation of cautious behavior, and other people's attitudes to shielding the person with ALS; (ii) changes to daily life caused by the pandemic and progression of ALS; (iii) distress in spouses, which included anxiety, depression, and burden; and (iv) ALS-related behavioral impairment. Spouses also provided mixed accounts of telehealth care, pointing out its convenience but some felt that face-to-face appointments were preferable. While many reactions to the pandemic reported by spouses of pwALS may have been similar to those of the general population or other vulnerable groups, interviews indicated the potential for the pandemic to have made more apparent certain aspects of behavioral change in pwALS with which carers may require support. Clinicians need to acknowledge spouses' concerns about the potential limitations of remote clinical consultations, enquire about cognitive and behavioral change, and consider how input should be best provided in such limiting circumstances. [ABSTRACT FROM AUTHOR]

This systematic review provides an updated summary of the existing literature on the validity of screening tools for cognitive and behavioral impairment in people with Amyotrophic Lateral Sclerosis (pwALS), and also focuses on their reliability. The following cognitive and behavioral screening tools were assessed in this review: the Edinburgh Cognitive and Behavioral ALS Screen (ECAS); the ALS Cognitive Behavioral Screen (ALS-CBS), the Mini Addenbrooke's Cognitive Examination (Mini-ACE), the Beaumont Behavioral Interview (BBI); the MND Behavior Scale (MiND-B); and the ALS-FTD Questionnaire (ALS-FTD-Q). A search, using Medline, PsychINFO and Embase (21/09/2023), generated 37 results after exclusion criteria were applied. Evidence of internal consistency, item-total correlations, inter-rater reliability, clinical validity, convergent validity, and structural validity were extracted and assessed and risk of bias was evaluated. The cognitive component of the ECAS was the tool with most evidence of reliability and validity for the assessment of cognitive impairment in ALS. It is well-suited to accommodate physical symptoms of ALS. For behavioral assessment, the BBI or ALS-FTD-Q had the most evidence of reliability and validity. The BBI is more thorough, but the ALS-FTD-Q is briefer. There is good but limited evidence for the reliability and validity of cognitive and behavioral screens. Further evidence of clinical and convergent validity would increase confidence in their clinical and research use. [ABSTRACT FROM AUTHOR]

This study aimed to provide a preliminary assessment of objective and subjective neurocognitive functioning in individuals with functional motor symptoms (FMS) and/or functional seizures (FS). We tested the hypotheses that the FMS/FS group would display poorer objective attentional and executive functioning, altered social cognition, and reduced metacognitive accuracy. Individuals with FMS/FS (n = 16) and healthy controls (HCs, n = 17) completed an abbreviated CANTAB battery, and measures of intellectual functioning, subjective cognitive complaints, performance validity, and comorbid symptoms. Subjective performance ratings were obtained to assess local metacognitive accuracy. The groups were comparable in age (p = 0.45), sex (p = 0.62), IQ (p = 0.57), and performance validity (p-values = 0.10–0.91). We observed no impairment on any CANTAB test in this FMS/FS sample compared to HCs, although the FMS/FS group displayed shorter reaction times on the Emotional Bias task (anger) (p = 0.01, np2 = 0.20). The groups did not differ in subjective performance ratings (p-values 0.15). Whilst CANTAB attentional set-shifting performance (total trials/errors) correlated with subjective performance ratings in HCs (p-values<0.005, rs = −0.85), these correlations were non-significant in the FMS/FS sample (p-values = 0.10–0.13, rs-values = −0.46–0.50). The FMS/FS group reported more daily cognitive complaints than HCs (p = 0.006, g = 0.92), which were associated with subjective performance ratings on CANTAB sustained attention (p = 0.001, rs = −0.74) and working memory tests (p < 0.001, rs = −0.75), and with depression (p = 0.003, rs = 0.70), and somatoform (p = 0.003, rs = 0.70) and psychological dissociation (p-values<0.005, rs-values = 0.67–0.85). These results suggest a discordance between objective and subjective neurocognitive functioning in this FMS/FS sample, reflecting intact test performance alongside poorer subjective cognitive functioning. Further investigation of neurocognitive functioning in FND subgroups is necessary. [ABSTRACT FROM AUTHOR]

The study evaluates the technology of fluoroscopy-based hip navigation that has shown to improve implant positioning in total hip arthroplasty (THA). Premier Healthcare data for patients undergoing manual THA or fluoroscopy-based hip navigation THA between 1 January 2016-30 September 2021, were analyzed 90- and 365-day post-THA. The primary outcome was inpatient readmission. Secondary outcomes were operating room (OR) time, length of stay, discharge status, and hospital costs. Baseline covariate differences were balanced using fine stratification and analyzed using generalized linear models. Among 4,080 fluoroscopy-based hip navigation THA and 429,533 manual THA balanced patients, hip-related readmission rates were statistically significantly lower for the fluoroscopy-based hip navigation THA cohort vs. manual THA for both 90-day (odd ratio [95% CI]: 0.69 [0.52 to 0.91] and 365-day (0.63 [0.49 to 0.81] follow-up. OR time was higher with fluoroscopy-based hip navigation THA vs. manual THA (134.65 vs. 132.04 minutes); however, fluoroscopy-based hip navigation THA patients were more likely to be discharged to home (93.73% vs. 90.11%) vs. manual THA. Hospital costs were not different between cohorts at 90- and 365-day post-operative. Fluoroscopy-based hip navigation THA resulted in fewer readmissions, greater discharge to home, and similar hospital costs compared to manual THA. Computer-assisted solutions are becoming more ubiquitous in surgical procedures. However, most of the current research is limited to small sample size and limited economic endpoints. The current study utilizes hospital billing data and fine stratification methodology to address the issue around sample size and covariate balancing. Hospital billing data provide a large sample across the US along with hospital costs that can be broken into different categories. Fine stratification methodology allows for covariate balancing while keeping all the samples. It is particularly advantageous when the treated or exposed group represents less than 5% of the entire cohort for a given study, as was observed in this study. Covariate balancing was done on patient, provider (hospital) and surgeon characteristics to minimize confounding. Furthermore, a generalized linear model was utilized to minimize any residual confounding. The study evaluated both short term (3-month) and long term (1-year) outcomes. Study suggested clinical benefits in using computer-assisted fluoroscopy-based hip navigation system in THA compared to manual THA as well as showed cost parity between computer-assisted fluoroscopy-based hip navigation system in THA and manual THA. [ABSTRACT FROM AUTHOR]

The impact of robotic-assisted total knee arthroplasty (rTKA) vs. traditional, manual TKA (mTKA) on hospital costs is not well documented and is analyzed herein. Patients in the Premier billing Healthcare Database undergoing elective rTKA or mTKA ("index') in the in- or outpatient setting for knee osteoarthritis between Oct 1st, 2015, to September 30th, 2021, were identified. Variables included patient demographics and comorbidities and hospital characteristics. Matched rTKA vs. mTKA cohorts were created using direct (on provider characteristics, age, gender, race and Elixhauser index) and propensity score matching (fixation type, comorbidities). Index and 90-day inflation-adjusted costs and healthcare utilization (HCU) were analyzed for both cohorts, using generalized linear models. 16,714 rTKA patients were matched to 51,199 mTKA patients. Average 90-day hospital cost reached $17,932 and were equivalent for both cohorts (rTKA vs. mTKA: $132 (95% confidence interval; -$19 to $284). There was a 2.7% (95%CI: 2.2%-3.3%) increase in home or home health discharge, and a 0.4% (95%CI: 0%-0.8%) decrease in 90-day hospital knee related re-visit in the rTKA vs. mTKA group. Cost-neutrality of rTKA vs. mTKA was observed, with a potential for lowered immediate post-operative HCU in the rTKA vs. mTKA cohorts. [ABSTRACT FROM AUTHOR]

Introduction: We aimed to examine susceptibility to dissociation and the impact of dissociation on interoceptive processing in individuals with functional neurological disorder (FND). We hypothesised that dissociative states would be elevated and interoceptive accuracy and awareness impaired at baseline in people with FND, and that such differences would be exacerbated following acute dissociation. Methods: Nineteen adults with FND were compared to 20 healthy controls. A modified heart-beat tracking task measured interoceptive accuracy and awareness (confidence) before and after a validated dissociation induction procedure. An exteroceptive processing control task was included. Mann–Whitney tests and r-values (effect size) were computed for between-group comparisons. Results: The FND group displayed elevated dissociation at baseline (p = 0.001, r = 0.528) compared to controls which increased following dissociation-induction (p < 0.001, r = 0.663). Interoceptive accuracy did not differ between groups at baseline (p = 0.967, r = 0.009); however, the FND group had lower accuracy scores post-induction (p = 0.021, r = 0.379). A negative correlation (trend) between change scores for dissociation and interoceptive accuracy was noted (rs = −0.411, p = 0.057). Confidence ratings on interoceptive and exteroceptive processing tasks were lower in the FND group (p-values < 0.05 or <0.01, r-values 0.331–0.489). Conclusions: Individuals with FND experienced greater susceptibility to dissociation, metacognitive deficits and impaired interoceptive accuracy than controls after acute dissociation. [ABSTRACT FROM AUTHOR]

Background: There is a lack of knowledge about what factors may impede or facilitate engagement in psychological interventions in people with motor neuron disease (pwMND) and how such interventions can be adapted to best meet the needs of this population. Objectives: To explore the needs and preferences of pwMND with respect to psychological interventions, and how best to adapt such interventions for pwMND. Methods: A series of semi-structured interviews (n = 22) and workshops (n = 3) were conducted with pwMND (n = 15), informal caregivers of pwMND (n = 10), and MND healthcare professionals (n = 12). These explored preferences and concerns that would need to be considered when delivering a psychological intervention for pwMND. Three areas were explored: (i) perceived factors that may hinder or facilitate pwMND engaging with psychological interventions; (ii) ways in which such interventions could be adapted to meet the individual needs of pwMND; and (iii) views regarding the main psychological issues that would need to be addressed. Workshops and interviews were audio recorded and transcribed and thematic analysis was used to inductively derive themes. Findings: Data could be classified within four overarching themes: unfamiliar territory; a series of losses; variability and difficulty meeting individual needs; and informal support. Conclusions: Flexibility, tailoring interventions to the individual needs of pwMND, and encouraging autonomy are key attributes for psychological interventions with pwMND. Psychological interventions such as Acceptance and Commitment Therapy (ACT) could be acceptable for pwMND if adapted to their specific needs. [ABSTRACT FROM AUTHOR]

Objectives: Cognitive and behavioral change in Amyotrophic Lateral Sclerosis (ALS) is well-accepted. Several screening tools have been developed to detect such changes. Further guidance on their use may come from a consideration of the rigor with which they were validated. This systematic review set out to critically appraise and present published data pertaining to the validation of six screening tools used to diagnose cognitive and/or behavioral change in patients with ALS. Methods: The screening tools considered in this search included: The Edinburgh Cognitive and Behavioural ALS Screen (ECAS), The ALS Cognitive Behavioural Screen (ALS-CBS), The Motor Neuron Disease Behavioural Scale (MiND-B), The Frontal Behavioural Inventory ALS Version, The ALS Frontotemporal Dementia Questionnaire (ALS-FTD-Q), and The Beaumont Behavioural Inventory (BBI). MEDLINE, EMBASE, and PsycINFO were searched until 4th week of June 2017. Results: Fourteen eligible studies were included in the review. Papers either reported data concerning convergent validity or clinical validity. Validation data concerning the ECAS showed this screening tool to have strong clinical validity, although further work needs to consider how its use will affect diagnosis rates according to current diagnostic guidelines. When screening for behavioral change only, more limited information is available; the BBI may offer greater potential than the ALS-FTD-Q for detecting mild impairment as it assesses a wider range of behavioral changes. Conclusions: Scores of sensitivity, specificity, positive predictive values, and negative predictive values should be given considerable importance when considering which screening tools to incorporate into current clinical practice. [ABSTRACT FROM AUTHOR]

This article presents the revised consensus criteria for the diagnosis of frontotemporal dysfunction in amyotrophic lateral sclerosis (ALS) based on an international research workshop on frontotemporal dementia (FTD) and ALS held in London, Canada in June 2015. Since the publication of the Strong criteria, there have been considerable advances in the understanding of the neuropsychological profile of patients with ALS. Not only is the breadth and depth of neuropsychological findings broader than previously recognised – – including deficits in social cognition and language – but mixed deficits may also occur. Evidence now shows that the neuropsychological deficits in ALS are extremely heterogeneous, affecting over 50% of persons with ALS. When present, these deficits significantly and adversely impact patient survival. It is the recognition of this clinical heterogeneity in association with neuroimaging, genetic and neuropathological advances that has led to the current re-conceptualisation that neuropsychological deficits in ALS fall along a spectrum. These revised consensus criteria expand upon those of 2009 and embrace the concept of the frontotemporal spectrum disorder of ALS (ALS-FTSD). [ABSTRACT FROM AUTHOR]

Despite recent interest in cognitive changes in patients with amyotrophic lateral sclerosis (ALS), investigations of language function looking at the level of word, sentence and discourse processing are relatively scarce. Data were obtained from 26 patients with sporadic ALS and 26 healthy controls matched for age, education, gender, anxiety, depression and executive function performance. Standardized language tasks included confrontation naming, semantic access, and syntactic comprehension. Quantitative production analysis (QPA) was used to analyse connected speech samples of the Cookie Theft picture description task. Results showed that the ALS patients were impaired on standardized measures of grammatical comprehension and action/verb semantics. At the level of discourse, ALS patients were impaired on measures of syntactic complexity and fluency; however, the latter could be better explained by disease related factors. Discriminant analysis revealed that syntactic measures differentiated ALS patients from controls. In conclusion, patients with ALS exhibit deficits in receptive and expressive language on tasks of comprehension and connected speech production, respectively. Our findings suggest that syntactic processing deficits seem to be the predominant feature of language impairment in ALS and that these deficits can be detected by relatively simple language tests. [ABSTRACT FROM AUTHOR]

Our objective was to systematically review and critically evaluate the evidence for psychotherapy and pharmacotherapy interventions for reducing distress or improving well-being in people with amyotrophic lateral sclerosis (pwALS). Online bibliographic databases and clinical trial registers were searched and an assessment of study quality was conducted. Seven thousand two hundred and twenty-three studies were identified, of which five met inclusion criteria (four completed and one in progress). All studies examined psychotherapeutic interventions, and no studies investigated pharmacotherapy. Two studies adopted a randomized controlled trial design, one a controlled trial design and two a cohort design. Sample sizes were small in all studies (overalln =145). The quality of completed studies was generally poor, with evidence that all were at potential risk of bias in numerous areas. Improvements in well-being were found with expressive disclosure (compared to no disclosure), cognitive behavioural therapy/counselling (compared to non-randomized pharmacotherapy) and hypnosis in the short term only, while no improvements were seen with a life review intervention. In conclusion, there is currently insufficient evidence to recommend the use of specific psychotherapy interventions for reducing distress or improving well-being in pwALS, and no evidence to support pharmacotherapy interventions. Research is urgently needed to address these significant gaps in the literature. [ABSTRACT FROM PUBLISHER]

Up to 50% of patients with amyotrophic lateral sclerosis (ALS) show mild to moderate cognitive-behavioural change alongside their progressive functional impairment. This study examines the relative impact of patients’ disease symptoms, behavioural change and current executive function and social cognition abilities on psychosocial outcomes in spouse caregivers of people with ALS. Thirty-five spouse caregivers rated their own levels of depression and anxiety, subjective burden and marital satisfaction. Caregivers also rated their partner’s everyday behaviour. The patients were assessed for disease severity and cognitive function, with composite scores derived for executive function and social cognition. Regression analyses revealed that caregiver burden was predicted by the severity of patients’ limb involvement and behavioural problems. Depression was predicted by patients’ limb involvement, while behavioural problems and patient age predicted caregiver anxiety. Current marital satisfaction was predicted by patient behavioural problems beyond the level of pre-illness marital satisfaction. In conclusion, the study highlights the potential impact of ALS patients’ functional impairment and behavioural change on ALS caregivers’ psychosocial functioning. Clinical communication with ALS families should emphasise both physical and psychological challenges presented by the disease. [ABSTRACT FROM PUBLISHER]

Our objective was to identify factors associated with acceptance of non-invasive ventilation (NIV) and gastrostomy in an exploratory population-based study. Seventy-eight people with ALS at least six months post-diagnosis, and 50 caregivers, were recruited from the South-East ALS Register. Baseline physical, cognitive and psychological measures were obtained. Three-monthly follow-ups monitored whether patients had accepted or refused NIV or gastrostomy. Following an intervention decision, post-decision interviews repeated baseline measures and included further intervention-specific questionnaires. Results showed that 32 people with ALS made at least one intervention decision and of these 10 decided about both NIV and gastrostomy. While illness factors predicted those needing to make an intervention decision, cognitive and education status, and level of executive dysfunction were associated with decision-making and acceptance or refusal of interventions. Patients' understanding of their illness, their early approach to considering interventions and carer-related factors were also associated with treatment decisions. In conclusion, our findings highlight the complexity of decision-making and provide a platform for designing further studies. Cognitive and psychosocial factors may assume a greater role in palliative care decisions for people with ALS than has been explicitly recognized. Future work must clarify how to ensure patients are not inadvertently being denied suitable interventions. [ABSTRACT FROM AUTHOR]

Several studies have reported changes in emotional memory and processing in people with ALS (pwALS). In this study, we sought to analyse differences in emotional processing and memory between pwALS and healthy controls and to investigate the relationship between emotional memory and self-reported depression. Nineteen pwALS and 19 healthy controls were assessed on measures of emotional processing, emotional memory, verbal memory and depression. Although pwALS and controls did not differ significantly on measures of emotional memory, a subgroup of patients performed poorly on an emotional recognition task. With regard to emotional processing, pwALS gave significantly stronger ratings of emotional valence to positive words than to negative words. Higher ratings of emotional words were associated with better recall in controls but not pwALS. Self-reported depression and emotional processing or memory variables were not associated in either group. In conclusion, the results from this small study suggest that a subgroup of pwALS may show weakened 'emotional enhancement', although in the current sample this may reflect general memory impairment rather than specific changes in emotional memory. Nonetheless, different patterns of processing of emotionally-salient material by pwALS may have care and management-related implications. [ABSTRACT FROM AUTHOR]

Replicable risk factors for ALS include increasing age, family history and being male. The male: female ratio has been reported as being between 1 and 3. We tested the hypothesis that the sex ratio changes with age in a population register covering the south-east of England. The sex ratio before and after the age of 51 years was compared using a Z-test for proportions. Kendall's tau was used to assess the relationship between age group and sex ratio using incidence and prevalence data. Publicly available data from Italian and Irish population registers were compared with results. There was a significant difference in the proportion of females with ALS between those in the younger group (30.11%) and those in the older group (43.66%) ( p = 0.013). The adjusted male: female ratio dropped from 2.5 in the younger group to 1.4 in the older group using prevalence data (Kendall's tau = −0.73, p = 0.039). Similar ratios were found in the Italian but not the Irish registry. We concluded that sex ratios in ALS may change with age. Over-representation of younger patients in clinic registers may explain the variation in sex ratios between studies. Menopause may also play a role. [ABSTRACT FROM AUTHOR]

We aimed to assess whether rural residence is associated with amyotrophic lateral sclerosis in the south-east of England using a population based register. Previous studies in different populations have produced contradictory findings. Residence defined by London borough or non-metropolitan district at time of diagnosis was recorded for each incident case in the South-East England ALS Register between 1995 and 2005. Each of the 26 boroughs or districts of the catchment area of the register was classified according to population density. Age- and sex-adjusted incidence of ALS was calculated for each region and the relationship with population density tested by linear regression, thereby controlling for the underlying population structure. We found that population density in region of residence at diagnosis explained 25% of the variance in ALS rates ( r = 0.5, p < 0.01). Thus, in this cohort in the south-east of England, people with ALS were more likely to be resident in areas of high population density at diagnosis. [ABSTRACT FROM AUTHOR]

Our objective was to determine change over time in quality of marital relationships of people with ALS (pwALS). Fifty pwALS and their spouse carers completed measures of marital intimacy, pwALS's functional status, their own psychological status, and beliefs about the pwALS's illness. Twenty-seven dyads completed two further assessments, at six-monthly intervals. Post-ALS diagnosis ratings of current marital relationship did not change over time for pwALS or their spouses. However, spouse carers' ratings of their current marital relationship were lower than their ratings prior to illness onset. After adjusting for the pre-illness marital relationship, pwALSs' current ratings of their marital relationship were initially predicted by their self-rated psychosocial functional impairment and satisfaction with their personal relationships, and subsequently by levels of self-esteem. After adjusting for ratings of the pre-illness marital relationship, carers' current marital intimacy scores at first and second interview were predicted by ratings of the patients' psychosocial functional impairment and by feelings of burden at the third interview. In conclusion, quality of the pre-illness marital relationship is a significant predictor of ongoing marital relationship in both pwALS and their spouse carers. Additionally, social and psychological rather than disease symptoms are important predictors of marital relationship quality after ALS diagnosis. [ABSTRACT FROM AUTHOR]

Amyotrophic lateral sclerosis (ALS) is increasingly recognized to be a multisystem disorder which includes both clinical and neuropathological features of a frontotemporal lobar degeneration (FTLD). In order to provide a common framework within which to discuss the characteristics of the cognitive and behavioural syndromes of ALS, and with which to conduct clinical and neuropathological research, an international research workshop on frontotemporal dementia (FTD) and ALS was held in London, Canada in June 2007. The recommendations arising from this research workshop address the requirement for a concise clinical diagnosis of the underlying motor neuron disease (Axis I), defining the cognitive and behavioural dysfunction (Axis II), describing additional non-motor manifestations (Axis III) and identifying the presence of disease modifiers (Axis IV). [ABSTRACT FROM AUTHOR]

Amyotrophic lateral sclerosis (ALS) is a multi-system disorder. Mild cognitive deficits are present in a subgroup of non-demented patients with ALS. Detailed neuropsychological assessments reveal deficits of word retrieval including impairments on tests of verbal fluency and confrontation naming. The PET GABAA receptor ligand [11C]-flumazenil is a marker of neuronal dysfunction in ALS. This study used [11C]-flumazenil PET to identify correlations between cortical regions and impairments in word retrieval. Twelve patients with ALS underwent [11C]-flumazenil PET and neuropsychological assessment, including tests of written letter fluency and confrontation naming. Poorer performance on verbal fluency correlated with decreased [11C]-flumazenil binding in a region including the right inferior frontal gyrus, superior temporal gyrus, and anterior insula. Poorer performance on a test of confrontation naming correlated with decreased binding in the left middle frontal gyrus (extending to Broca's area) and left cuneus. This study indicates that [11C]-flumazenil PET can be used to help localize cortical regions associated with cognitive deficits in ALS. [ABSTRACT FROM AUTHOR]

Previous studies have suggested a lower incidence of ALS in people of African origin. We used a population based register in an urban setting from inner city London postcodes where there is a large population of people of African ancestry to compare the frequency of ALS in people of European and African origin. Population statistics stratified by age, gender and ethnicity were obtained from the 2001 census. Incidence and prevalence were calculated in each ethnic group. Results showed that in a population of 683,194, of which 22% were of African ancestry, 88 individuals with ALS were identified over a seven-year period, including 14 people with African ancestry. The adjusted incidence in people of African ancestry was 1.35 per 100,000 person-years (95% CI 0.72-2.3) and in those of European ancestry 1.97 per 100,000 person-years (95% CI 1.55-2.48). In conclusion, in this small population based study we could not detect a difference in rates of ALS between people of African ancestry and those of European ancestry. [ABSTRACT FROM AUTHOR]