A preliminary comparison between ECAS and ALS-CBS in classifying cognitive–behavioural phenotypes in a cohort of non-demented amyotrophic lateral sclerosis patients

2021 ◽  
Author(s):  
Lucia Catherine Greco ◽  
Andrea Lizio ◽  
Jacopo Casiraghi ◽  
Valeria Ada Sansone ◽  
Lucio Tremolizzo ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Preliminary Comparison ◽  
Cognitive Behavioural ◽  
Behavioural Phenotypes ◽  
Lateral Sclerosis

#3090 Different measures of behavioural involvement in amyotrophic lateral sclerosis yield varying rates of behavioural change

2021 ◽  
Vol 92 (8) ◽  
pp. A12.1-A12
Author(s):  
Lyndsay Didcote ◽  
Silia Vitoratou ◽  
Ammar Al-Chalabi ◽  
Laura H Goldstein
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Behavioural Change ◽  
Screening Tools ◽  
Complete Agreement ◽  
Spearman Correlation ◽  
Healthcare Provision ◽  
Cognitive Behavioural ◽  
Moderate Range ◽  
Lateral Sclerosis

Objectives/AimsAmyotrophic Lateral Sclerosis (ALS), also known as Motor Neuron Disease (MND), is a progressive and life-limiting neurodegenerative disease which can involve behavioural change. There are five commonly reported disease-specific screening tools of behavioural change but it is not clear how they differ in applicability. This study therefore set out to investigate the extent to which these measures similarly identify impairment by examining i) intercorrelations between scores on the measures completed about the same people with ALS, and ii) the percentage of people with ALS characterised as impaired on each measure.MethodsThe behavioural component of the ALS-Cognitive Behavioural Screen (ALS-CBS-b), behavioural component of the Edinburgh Cognitive and Behavioural ALS Screen (ECAS-b), ALS-Frontotemporal Dementia Questionnaire (ALS-FTD-Q), Beaumont Behavioural Inventory (BBI), and MND Behavioural Instrument (MiND-B) were all completed by 35 carers of people with ALS. Total scores for each measure underwent Spearman correlation analysis. Classifications of impairment (for behavioural impairment or ALS-FTD) were determined using published cut-offs and agreement between measures was determined by calculating Cohens kappa coefficients.ResultsThe behavioural measures were significantly intercorrelated (p<0.05 in all cases) but with differing strengths of association. The association between the ALS-CBS-b and ALS-FTD-Q was weak to moderate (r=0.41) while other associations between the ALS-FTD-Q, MiND-B, BBI, and ALS-CBS-b were moderate to strong (0.53-0.75). While the ECAS-b was moderately associated with the BBI (r=0.53), the ECAS-b was only weakly to moderately associated with the ALS-CBS-b, MiND-B and ALS-FTD-Q (0.36-0.48). The association between ALS-CBS-b and ALS-FTD-Q scores (r=0.41) was similarly in the weak to moderate range. The ECAS-b and ALS-CBS-b had the weakest intercorrelation (r=0.36). Percentages of the sample classified with behavioural involvement by the measures ranged between 20.0% (ALS-FTD-Q) and 74.3% (MiND-B). Percentages of the sample classified with ALS-FTD by the measures ranged between 2.9% (ECAS-b) and 20% (ALS-CBS-b). Agreement of classification between measures (Cohens kappa) was mostly fair to moderate (0.21-0.57) although generally better for classifications of ALS-FTD than for milder behavioural involvement. Between the ECAS-b and BBI there was complete agreement on the classification of ALS-FTD (k = 1.0).ConclusionsExisting measures of behavioural change in people with ALS may yield very differing conclusions and cannot be assumed to be interchangeable. Variability in the detection of impairment between measures may result from differing item content, behaviours sampled or cut-off scores for impairment. This inconsistency between measures may lead to inappropriate healthcare provision and discrepancies in research conclusions.FundingMND Association

Psychological research in amyotrophic lateral sclerosis: Past, present, and future

2018 ◽  
Author(s):  
Tamlyn J. Watermeyer ◽  
Laura H. Goldstein
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Well Being ◽  
Psychological Research ◽  
Behavioural Therapy ◽  
Future Research ◽  
Psychological Treatments ◽  
Dignity Therapy ◽  
Cognitive Behavioural ◽  
Lateral Sclerosis

This chapter, of particular interest to those interested in psychological treatments for people with amyotrophic lateral sclerosis (ALS), outlines earlier work that sought to identify correlates of reduced well-being and quality of life in people with ALS and delineates possible targets for intervention. In this context, the chapter then evaluates several studies that have investigated psychological interventions for optimizing well-being in people with ALS and their caregivers. The chapter reviews current efforts to address the paucity of interventional research in this patient group, focusing on five therapies that have so far been evaluated for treatment efficacy. These therapies include hypnosis, mindfulness, cognitive behavioural therapy, expressive disclosure therapy, and dignity therapy. The main findings from these studies and their clinical implications for people with ALS and their families are discussed. Recommendations for future research are considered, together with a discussion of the implementation of such interventions in therapeutic or multidisciplinary settings.

Sexuality and intimacy in ALS: systematic literature review and future perspectives

2018 ◽  
Vol 90 (6) ◽  
pp. 712-719
Author(s):  
Barbara Poletti ◽  
Laura Carelli ◽  
Federica Solca ◽  
Rita Pezzati ◽  
Andrea Faini ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Literature Review ◽  
Systematic Literature Review ◽  
English Literature ◽  
Well Being ◽  
Intimate Relationship ◽  
Level Of Evidence ◽  
Sexual Activities ◽  
Cognitive Behavioural ◽  
Lateral Sclerosis

Several features of amyotrophic lateral sclerosis (ALS) impact on sexuality and intimate relationship; however, the issue has received poor attention so far. We performed a systematic literature review in order to provide an up-to-date account of sexuality in ALS. References were identified by searches of PubMed, Web of Science, Scopus and PsycINFO (1970–2017, English literature). The following were the key terms: ‘sexual’ OR ‘sexuality’ OR ‘intimacy’ OR ‘marital’ AND ‘ALS’ OR ‘Amyotrophic Lateral Sclerosis’ OR ‘Motor Neuron Disease’ OR ‘MND’. Titles and abstracts were screened for relevance and a full-text analysis was performed on the selected articles. Studies were included if they referred to sexual well-being/activities/functions or intimate relationship between patients and their partners and management of such topic by clinicians. Eligibility assessment was performed independently by two reviewers. A thematic and level of evidence classification of studies was performed. Studies’ design, objectives, measurements and outcomes were summarised. Thirty articles were included and four topics were identified: intimacy in the dyads; sexual activities in patients and with their partners; sexual function disturbances; and sexuality and cognitive-behavioural alterations. The quality of the studies varies, with globally poor level of evidence. Some sexuality issues have been only sparsely addressed, such as gender-related differences, same-sex relationships and sexual activities other than intercourse. Sexuality in ALS is still not adequately considered by clinicians and researchers. We present preliminary recommendations for improving sexuality and intimacy at any ALS multidisciplinary clinics.

scholarly journals The Cognitive and Behavioural Profile of Amyotrophic Lateral Sclerosis: Application of the Consensus Criteria

2013 ◽  
Vol 27 (2) ◽  
pp. 143-153 ◽  
Author(s):  
Monica Consonni ◽  
Sandro Iannaccone ◽  
Chiara Cerami ◽  
Paola Frasson ◽  
Marco Lacerenza ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Short Term Memory ◽  
Executive Dysfunction ◽  
Cognitively Impaired ◽  
Action Naming ◽  
Cognitive Behavioural ◽  
Behavioural Profile ◽  
Als Patients ◽  
The Impact ◽  
Lateral Sclerosis

Objective:The study aims to assess the spectrum of cognitive and behavioural disorders in patients affected by Amyotrophic Lateral Sclerosis (ALS) according to the recent consensus criteria [9]. The study also intends to assess the impact of physical disability on cognitive and behavioural abnormalities.Methods:Detailed neurological, neuropsychological and neurobehavioral evaluations were administered to 23 ALS patients, 11 Lower Motor Neuron Disease (LMND) patients and 39 healthy controls. Strong et al.’s criteria [9] were applied to diagnose the presence of cognitive/behavioural impairment. Clinical and neuropsychological scores were used for group comparisons and correlation analyses.Results:In comparison with LMND and controls, a subgroup of ALS patients (∼30%) manifested executive dysfunction, which was severe enough to classify them as cognitively impaired. Action naming difficulties and short-term memory deficits were also observed. Aspontaneity, disorganization and mental rigidity reached clinical relevance in 20% of ALS patients. A small percentage of ALS patients (13%) also had comorbid dementia. The cognitive or behavioural status was not related to the clinical features of ALS.Conclusion:The use of consensus criteria for cognitive and behavioural impairment and the comparison with the LMND group proved useful in defining the spectrum of non-motor manifestations of ALS.

Automated Acoustic Analysis of Oral Diadochokinesis to Assess Bulbar Motor Involvement in Amyotrophic Lateral Sclerosis

2020 ◽  
Vol 63 (1) ◽  
pp. 59-73 ◽  
Author(s):  
Panying Rong
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Acoustic Analysis ◽  
Speaking Rate ◽  
Disease Stage ◽  
Healthy Controls ◽  
Tongue Movement ◽  
Major Barrier ◽  
Syllable Repetition ◽  
Oral Diadochokinesis ◽  
Lateral Sclerosis

Purpose The purpose of this article was to validate a novel acoustic analysis of oral diadochokinesis (DDK) in assessing bulbar motor involvement in amyotrophic lateral sclerosis (ALS). Method An automated acoustic DDK analysis was developed, which filtered out the voice features and extracted the envelope of the acoustic waveform reflecting the temporal pattern of syllable repetitions during an oral DDK task (i.e., repetitions of /tɑ/ at the maximum rate on 1 breath). Cycle-to-cycle temporal variability (cTV) of envelope fluctuations and syllable repetition rate (sylRate) were derived from the envelope and validated against 2 kinematic measures, which are tongue movement jitter (movJitter) and alternating tongue movement rate (AMR) during the DDK task, in 16 individuals with bulbar ALS and 18 healthy controls. After the validation, cTV, sylRate, movJitter, and AMR, along with an established clinical speech measure, that is, speaking rate (SR), were compared in their ability to (a) differentiate individuals with ALS from healthy controls and (b) detect early-stage bulbar declines in ALS. Results cTV and sylRate were significantly correlated with movJitter and AMR, respectively, across individuals with ALS and healthy controls, confirming the validity of the acoustic DDK analysis in extracting the temporal DDK pattern. Among all the acoustic and kinematic DDK measures, cTV showed the highest diagnostic accuracy (i.e., 0.87) with 80% sensitivity and 94% specificity in differentiating individuals with ALS from healthy controls, which outperformed the SR measure. Moreover, cTV showed a large increase during the early disease stage, which preceded the decline of SR. Conclusions This study provided preliminary validation of a novel automated acoustic DDK analysis in extracting a useful measure, namely, cTV, for early detection of bulbar ALS. This analysis overcame a major barrier in the existing acoustic DDK analysis, which is continuous voicing between syllables that interferes with syllable structures. This approach has potential clinical applications as a novel bulbar assessment.

Sign in / Sign up

Export Citation Format

Share Document