Amyotrophic Lateral Sclerosis Assessment Questionnaire Research Articles

The effect of social support on the emotional well-being of people with amyotrophic lateral sclerosis: Exploring the mediating role of spirituality.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that, so far, is considered always fatal. Treatments mainly consist in increasing survival and aim to improve the quality of life of people with ALS (pwALS). Social support and spirituality have been shown to play a key role in pwALS' quality of life. Our study explored it in depth by investigating the underlying mechanisms linking social support, spirituality, and emotional well-being. Thirty-six pwALS underwent a battery of tests evaluating emotional well-being (emotional well-being scale of the 40-item Amyotrophic Lateral Sclerosis Assessment Questionnaire), social support (6-item Social Support Questionnaire), and spiritual well-being (12-item Functional Assessment of Chronic Illness Therapy - Spiritual well-being). Our recruitment was web-based through the FILSLAN and the ARSLA websites as well as through Facebook® advertisements (ALS groups). Data were analyzed by Pearson correlation analysis and Process macro was used in an SPSS program to analyze the mediator variable effect. Availability of social support, spiritual well-being, and 2 of its dimensions, i.e., meaning and peace, were positively correlated with emotional well-being. The mediational analyses showed that spiritual well-being, meaning, and peace act as mediators in the association between availability of social support and good emotional well-being. Availability of social support and spirituality are essential for the emotional well-being of pwALS. Spirituality as a mediator between availability of social support and emotional well-being appears as real novel finding which could be explored further. Spiritual well-being, meaning, and peace appear as coping resources for pwALS. We provide practical guidance for professionals working with pwALS.

Health-related quality of life across disease stages in patients with amyotrophic lateral sclerosis: results from a real-world survey.

Amyotrophic lateral sclerosis (ALS) is characterized by a rapid disease course, with disease severity being associated with declining health-related quality of life (HRQoL) in persons living with ALS (pALS). The main objective of this study was to assess the impact of disease progression on HRQoL across King's, Milano-Torino Staging (MiToS), and physician-judgement clinical staging. Additionally, we evaluated the impact of the disease on the HRQoL of care partners (cALS). Data were sourced from the Adelphi ALS Disease Specific Programme (DSP)™, a cross-sectional survey of neurologists, pALS and cALS presenting in a real-world clinical setting between July 2020 and March 2021 in Europe and the United States. Neurologists (n = 142) provided data for 880 pALS. There were significant negative correlations between all three clinical staging systems and EuroQol (European Quality of Life) Five Dimension Five Level Scale (EQ-5D-5L) utility scores and visual analogue scale (VAS) ratings. Although not all differences were significant, 5-item Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-5) scores showed a stepwise increase in HRQoL impairment at each stage of the disease regardless of the staging system. At later stages, high levels of fatigue and substantial activity impairment were reported. As pALS disease states progressed, cALS also experienced a decline inHRQoL and increased burden. Across outcomes, pALS and cALS generally reported worse outcomes at later stages of the disease, highlighting an unmet need in this population for strategies to maximise QoL despite disease progression. Recognition and treatment of symptoms such as pain and fatigue may lead to improved outcomes for pALS and cALS.

Pain in patients with motor neuron disease: Variation of pain and association with disease severity, health-related quality of life and depression - A longitudinal study.

To describe levels of pain over time during disease progression in individual patients and for a total sample of patients with motor neuron disease (MND), respectively, and to examine associations between pain, disease severity, health-related quality of life (HRQOL), and depression. A prospective cohort study was conducted on 68 patients with MND, including data collected on five occasions over a period of 2 years. Pain was assessed using the Brief Pain Inventory - Short Form. Depression was assessed using the Amyotrophic Lateral Sclerosis (ALS)-Depression-Inventory (ADI-12). Disability progression was measured using the Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised Version (ALSFRS-R). HRQOL was assessed using the Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-5). Participants reported great individual variation over time. The median level of pain was 4 (min 0 and max 10). Higher levels of pain during the last 24h were associated with higher depression scores (ADI-12), poorer quality of life (ALSAQ-5), and lower reporting of fine and gross motor skills (ALSFRS-R). Baseline pain levels did not predict future values of depression and function. Individuals reporting average pain >3 experienced more hopelessness toward the future and reported higher depression scores compared with participants reporting average pain <3. Great within-individual variation of pain intensity was reported. Pain intensity was associated with depression, function and HRQOL cross-sectionally, but it did not have a strong prognostic value for future depression, function, or HRQOL. Patients with MND should be offered frequent assessment of pain and depressive symptoms in person-centered care, allowing for individualization of treatment.

Quality-of-life assessment instruments used across ALS clinics.

Instruments have been developed to assess quality of life (QoL) among people with amyotrophic lateral sclerosis (ALS). It is unclear whether these are utilized regularly in the clinical setting to guide individual patient care. In this study we aimed to understand the current use of instruments and existing barriers to assessing QoL in clinical ALS care. An anonymous survey developed by Northeast ALS (NEALS) Consortium Palliative Committee members was distributed to all multidisciplinary NEALS members. Data were summarized via calculation of descriptive statistics. ALS Center characteristics were compared using chi-square and Fisher exact tests for categorical variables. Seventy-three (6.4%) of the 1132 NEALS members responded to the survey, representing 148 clinics, 49.3% of whom reported assessing QoL during clinic visits. The most used ALS-specific instruments were the ALS Assessment Questionnaire (19.4%) and Amyotrophic Lateral Sclerosis Specific Quality of Life scale (16.6%). Barriers reported were uncertainty regarding which instrument to use and length of visits. QoL assessment was not significantly correlated with length of clinic visit but with access to specialty palliative care. QoL assessments are performed by some, but not all, ALS centers during clinical visits. Although this study did have a low number of responding centers, the percentage, the proportion is similar to that seen in earlier studies, which limits the findings' generalizability. The value of QoL assessments' impact on outcomes should be further investigated and, if warranted, creative ways sought to increase the frequency of their use, including patient self-assessments before clinic and/or the use of teleheath to reduce the length of clinic visits.

Relationship between quantitative strength and functional outcomes in the phase 2 FORTITUDE-ALS trial

Objective To assess the relationship among measurements of strength, function, and quality of life in an amyotrophic lateral sclerosis (ALS) clinical trial. Methods In the FORTITUDE-ALS clinical trial (NCT03160898), 456 participants in the full-analysis set were treated with either reldesemtiv or placebo for 12 weeks; this post hoc analysis included all participants regardless of treatment assignments. Assessments included slow vital capacity (SVC), the ALS Functional Rating Scale-Revised (ALSFRS-R), and the 5-item ALS Assessment Questionnaire (ALSAQ-5). Muscle strength was measured quantitatively with hand-held dynamometry, and grip strength with a dedicated dynamometer. The relationship between strength and ALSFRS-R fine and gross motor domain scores, or responses to ALSAQ-5 questions on hand function and walking, was assessed with Spearman’s rank correlation. The relationship between mean upper- or lower-extremity muscle strength and specific ALSFRS-R domains was modeled using principal-components analysis. Results Upper-extremity muscle strength and hand grip were highly correlated with ALSFRS-R fine motor scores and the ALSAQ-5 hand function question. Similarly, lower-extremity strength correlated well with ALSFRS-R gross motor domain and the ALSAQ-5 walking question. For SVC, correlation was poor with the ALSFRS-R respiratory domain, but stronger with the total score, potentially reflecting the insensitivity of the respiratory questions in the scale. Upper- and lower-extremity strength were both strong predictors of ALSFRS-R domain scores. Conclusions In this analysis of data from an ALS clinical trial, muscle strength quantified by dynamometry was strongly correlated with functional capacity. These results suggest that muscle strength directly relates to specific functions of importance to people with ALS.

Measurement Properties of Self-Report Questionnaires for Amyotrophic Lateral Sclerosis: A Systematic Review and Meta-Analysis of Commonly Used Instruments.

(1) Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease. There is no evidence on the analysis of the measurement instruments available to assess quality of life in these patients, following the consensus-based standards for the selection of health measurement instruments (COSMIN) checklist; (2) Methods: A systematic review was performed in PubMed, Embase, PEDro, Web of Science and Cochrane. The psychometric properties of the questionnaires were determined by using the COSMIN checklist. Two searches were carried out. This systematic review was registered in PROSPERO (CRD42021249005); (3) Results: There were four published articles that analysed the measurement properties in patients with ALS for the following scales: Amyotrophic Lateral Sclerosis Assessment Questionnaire 40, Amyotrophic Lateral Sclerosis-Specific Quality of Life Questionnaire, Short Form 36 Healthy Survey, Epworth Sleepiness Scale and Sickness Impact Profile. Another five scales also met the inclusion criteria: ALS-Depression-Inventory, State Trait Anxiety-Inventory, World Health Organization Quality of Life, Schedule for the Evaluation of Individual Quality of Life, Amyotrophic Lateral Sclerosis Assessment Questionnaire 5. Most Patient Reported Outcome Measures (PROMs) present a low-quality synthesis of evidence. It was observed an excellent pooled reliability of 0.92 (95% Confidence Interval: 0.83-0.96, I2 = 87.3%) for four dimensions for questionnaires ALSAQ-40. (4) Conclusions: There is little evidence on generic instruments. Future studies are necessary to develop new tools.

Safety and Efficacy of Edaravone in Patients with Amyotrophic Lateral Sclerosis: A Systematic Review and Meta-analysis.

The efficacy and safety of edaravone for the treatment of amyotrophic lateral sclerosis (ALS) remain unclear. The aim of this meta-analysis was toprovide evidence-based medical guidance and advice for the clinical application of edaravone in the treatment of ALS. PubMed, Embase, Chinese Biomedical Literature Database (CBM), Cochrane Library and Web of Science were searched through 09 March 2022 for randomized controlled trials (RCTs) on the safety and efficacy of edaravone versus placebo during follow-up of patients with ALS. A summary of the outcome measures with GRADE was performed. This study was registered on PROSPERO (ID: CRD 42022319997). Five RCTs with a total of 566 participants were included, and there was a significant difference (mean difference [MD] 1.33, 95% confidence interval [CI] 0.33-2.34; p=0.009) in the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) score between the treatment and placebo groups. The edaravone group had an increased grip strength (MD 0.26, 95% CI 0.03-0.49; p=0.03) and modified Norris Scale score (MD 2.81, 95% CI 1.18-4.43; p=0.0007). However, there were no significant differences between groups for the change in forced vital capacity (FVC)% (MD 0.55, 95% CI -3.15 to 4.24; p=0.77), pinch strength (MD 0.05, 95% CI -0.05 to 0.16; p=0.33) or Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40) score (MD -4.76, 95% CI -9.56 to 0.03; p=0.05). The incidence of adverse events (AEs) (risk ratio [RR] 0.09, 95% CI 0.93-1.05; p=0.65), serious adverse events (SAEs) (RR 0.72, 95% CI 0.52-1.00; p=0.05) and the number of deaths (risk difference [RD] 0.00, 95% CI -0.02 to 0.03; p=0.83) were not statistically different from the placebo group. The quality of evidence was low only for SAEs, and the remaining outcome measures were of moderate quality. Compared with placebo, edaravone may provide potential clinical benefits in the treatment of ALS and may not increase the number of AEs and deaths. However, due to the low-quality evidence of the included studies and the small sample size, more high-quality and high-standard research evidence is needed to confirm these results. This study was registered on PROSPERO (ID: CRD 42022319997).

POWERbreathe® Inspiratory Muscle Training in Amyotrophic Lateral Sclerosis.

Inspiratory muscle training may benefit respiratory function, cardiocirculatory parameters, quality of life and functionality in neuromuscular diseases. This pilot study aimed to demonstrate the POWERbreathe® inspiratory muscle training effects on maximum inspiratory pressure (PImax), heart rate (HR) and HR variability, as well as the quality of life impairment and functionality in patients with Amyotrophic Lateral Sclerosis (ALS). A pilot single-blinded, non-randomized controlled clinical trial was carried out. A total of 20T ALS patients were enrolled and divided into experimental (n = 10) and control (n = 10) groups. The experimental group received POWERbreathe® inspiratory muscle training in conjunction with usual care, and the control group received only usual care for 8 weeks. PImax (measured by POWERbreathe® KH1), HR and HR variability (evaluated by Polar H7), quality of life impairment [measured by the Amyotrophic Lateral Sclerosis Assessment Questionnaire-40 items (ALSAQ-40)] and functionality [assessed by the ALS Functional Rating Scale Revised (ALSFRS-R)] were collected at baseline and after 8 weeks of intervention. We detected statistically significant differences (p &lt; 0.05) with an effect size ranging from medium to large (Cohen's d = 0.72-1.37); relative to the control group, the experimental group had an increased PImax (mean difference = 10.80 cm H2O; 95% CI = 3.42-18.17) and ALSFRS-R score (mean difference = 5.30 points; 95% CI = -0.03-10.63) and reduced HR (mean difference = -8.80 beats-per-minute; 95% CI = -20.27-2.67) and R-R interval (mean difference = 78.30 ms; 95% CI = 2.89-153.70). POWERbreathe® inspiratory muscle training, in addition to usual care, may improve inspiratory strength and heart rate in patients with ALS. These results encourage larger and longer trials investigating potential clinically relevant benefits of inspiratory muscle training to these patients over the disease course.

Pain-Related Coping Behavior in ALS: The Interplay between Maladaptive Coping, the Patient's Affective State and Pain.

Background: Pain is a common symptom in patients with amyotrophic lateral sclerosis (ALS). Coping plays a central role in adjustment to pain. Objective: This study evaluates the use of different pain coping strategies in patients with ALS and investigates the interplay of maladaptive coping, and the patient’s affective state and pain. Methods: One hundred and fifty ALS patients from three German outpatient clinics completed the Brief Pain Inventory (BPI), the ALS-Functional Rating Scale-Extension (ALSFRS-EX), the ALS Depression Inventory (ADI-12), the subscale “emotional functioning” of the ALS Assessment Questionnaire (ALSAQ-40) and the Coping Strategies Questionnaire (CSQ). Based upon the results of correlational analyses, multiple regression analyses were performed to identify predictors of pain severity and to explore factors contributing to maladaptive coping. Results: Pain was prevalent in 56% (n = 84) of the patients. Patients applied different adaptive coping strategies as well as the maladaptive strategy “catastrophizing”. Regression analysis indicated that the CSQ-subscale “catastrophizing” significantly predicted pain intensity, explaining 34.0% of the variance (p < 0.001). Pain-related catastrophizing was associated with higher pain-related functional impairments and worse emotional functioning. The ADI-12 sum score as an indicator for depressive symptoms contributed significantly to the maladaptive coping strategy “catastrophizing” (p < 0.001) and explained 40.8% of the variance. Conclusion: Patients with ALS apply different strategies to cope with pain. Catastrophizing is an important determinant of higher pain intensity ratings and is associated with higher pain interferences and decreased emotional well-being. Pain-related catastrophizing is promoted by depressive symptoms. Catastrophizing and depressive symptoms thus represent important targets of individualized pain-management strategies.

Study protocol on the safety and feasibility of a normocaloric ketogenic diet in people with amyotrophic lateral sclerosis

ObjectivesThis study evaluates the safety and feasibility of a normocaloric ketogenic diet (KD) in people with amyotrophic lateral sclerosis (ALS) for reducing hyperexcitability levels and modulating neuroinflammation. MethodsThis is a prospective, open-label pilot study involving men and women diagnosed with ALS, ages 18 to 75 y. The primary outcome is the safety and reproducibility of the KD in people with ALS. We will monitor secondary clinical outcomes with the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale score, forced vital capacity, the Amyotrophic Lateral Sclerosis Assessment Questionnaire, blood parameters, and gut microbiota analyses. All participants will follow the KD for 8 wk. During the diet, the clinical status of all participants will be monitored every 15 d through neurologic and nutritional visits and biochemical markers. The research ethics committee approved the study. ResultsSafety will be assessed by measuring the number and severity of adverse events, including death, and any changes in blood chemistry, vital signs, and clinical exam results. Tolerability will be assessed to complete the proposed 8 wk of treatment while maintaining adequate nutritional status without inducing malnutrition. ConclusionsAdequate caloric intake is essential in ALS, because insufficient intake induces loss of body mass. We hope that the proposed study will provide a positive result in terms of the safety and feasibility of a KD in people ALS, with the purpose of developing a patient-centered diet program to limit disease progression and possibly improve survival.

Safety and efficacy of edaravone in well-defined Iranian patients with amyotrophic lateral sclerosis: A parallel-group single-blind trial

Background: This parallel-group single-blind trial evaluates the safety and efficacy of Edaravone, as a free radical scavenger, in a highly selective subgroup of Iranian patients with amyotrophic lateral sclerosis (ALS). Methods: The study was registered in ClinicalTrials.gov (registration number: NCT03272802) and Iranian Registry of Clinical Trials (registration number: IRCT20190324043105N). Patients were included into the study, who were diagnosed as probable or definite ALS (according to revised El Escorial criteria), mildly to moderately affected by the disease [according to Amyotrophic Lateral Sclerosis Health State Scale (ALS/HSS)], scored ≥ 2 points on all items of the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R), and had forced vital capacity (FVC) of at least 80%. 20 patients (10 cases, 10 controls) were observed for 12 cycles (each cycle lasted four weeks). Cases received Edaravone for the first 14 days in the first cycle and for the first 10 days in the next cycles. In addition, all patients received Riluzole. The 40-item Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40), ALSFRS-R, and Manual Muscle Testing (MMT) scores were measured every 3 cycles to evaluate the physical and functional status of the patients. Besides, injection reactions, adverse events (AEs), and serious adverse events (SAEs) were measured during the study. Results: ALSAQ-40, ALSFRS-R, and MMT scores were not significantly different between cases and controls in 5 different time points. During the study, no injection reactions were observed. AEs and SAEs were not significantly different between cases and controls. Conclusion: Our data did not demonstrate efficacy of Edaravone in ALS treatment, but showed its safety for use in patients with ALS. Further studies are necessary to investigate Edaravone efficacy in patients with ALS before prescribing this new drug outside Japan.

A Nation-Wide, Multi-Center Study on the Quality of Life of ALS Patients in Germany.

Improving quality of life (QoL) is central to amyotrophic lateral sclerosis (ALS) treatment. This Germany-wide, multicenter cross-sectional study analyses the impact of different symptom-specific treatments and ALS variants on QoL. Health-related QoL (HRQoL) in 325 ALS patients was assessed using the Amyotrophic Lateral Sclerosis Assessment Questionnaire 5 (ALSAQ-5) and EuroQol Five Dimension Five Level Scale (EQ-5D-5L), together with disease severity (captured by the revised ALS Functional Rating Scale (ALSFRS-R)) and the current care and therapies used by our cohort. At inclusion, the mean ALSAQ-5 total score was 56.93 (max. 100, best = 0) with a better QoL associated with a less severe disease status (β = −1.96 per increase of one point in the ALSFRS-R score, p < 0.001). “Limb-onset” ALS (lALS) was associated with a better QoL than “bulbar-onset” ALS (bALS) (mean ALSAQ-5 total score 55.46 versus 60.99, p = 0.040). Moreover, with the ALSFRS-R as a covariate, using a mobility aid (β = −7.60, p = 0.001), being tracheostomized (β = −14.80, p = 0.004) and using non-invasive ventilation (β = −5.71, p = 0.030) were associated with an improved QoL, compared to those at the same disease stage who did not use these aids. In contrast, antidepressant intake (β = 5.95, p = 0.007), and increasing age (β = 0.18, p = 0.023) were predictors of worse QoL. Our results showed that the ALSAQ-5 was better-suited for ALS patients than the EQ-5D-5L. Further, the early and symptom-specific clinical management and supply of assistive devices can significantly improve the individual HRQoL of ALS patients. Appropriate QoL questionnaires are needed to monitor the impact of treatment to provide the best possible and individualized care.

Evaluation of quality of life and mood disorders in caregivers of patients with amyotrophic lateral sclerosis: A single-center cross-sectional study

Background: Caregivers of patients with amyotrophic lateral sclerosis (ALS) may suffer from anxiety, depression, and reduced quality of life (QoL). Our goal was to evaluate the QoL and mood disorders in caregivers and their correlation with the patients' demographic, physical, and mental conditions. Methods: We analyzed data from 39 patients with ALS and their caregivers. Patients completed questionnaires of anxiety assessed by Generalised Anxiety Disorder Assessment (GAD-7), depression using the Beck Depression Inventory-II (BDI-II), and QoL via 40-item Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40). Physical impairment was also measured in the patients using the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R). Caregivers were also assessed by BDI-II, GAD-7, and 36-item Short-Form Health Survey questionnaire (SF-36). Results: The prevalence of depression and anxiety in the patients was 82.1% and 71.8%, respectively. Caregivers also had higher rates of anxiety and depression and lower levels of QoL in comparison with the general population (anxiety: 66.7%, depression: 43.6%). Depression and anxiety were considerably associated with worsened QoL in the caregivers. None of the demographic, physical, or mental characteristics of patients with ALS were related to either mood status or QoL of the caregiver population. Conclusion: Caregivers experience higher rates of anxiety and depression and lower QoL in comparison with the general population. The severity of mood disorders is inversely associated with the physical and mental domains of caregivers' QoL. Nonetheless, QoL in the caregivers is not affected by the physical or mental disability of the patients.

Funcionalidade e qualidade de vida de pessoas com esclerose lateral amiotrófica e percepção da sobrecarga e apoio social de cuidadores informais

Objetivo: Esta pesquisa tem como objetivo mensurar a funcionalidade e qualidade de vida de pacientes com Esclerose Lateral Amiotrófica (ELA) e fornecer evidências sobre a possível sobrecarga de cuidados gerados aos seus cuidadores informais. Método: Trata-se de um estudo descritivo exploratório do tipo quantitativo, com delineamento transversal. A amostra do estudo foi composta por 24 participantes, sendo 12 pacientes com ELA e 12 cuidadores informais, recrutados em um Centro Especializado de Reabilitação. Foram administrados aos indivíduos com ELA os instrumentos: Questionário sobre Informações Demográficas; Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R); Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40) e aos cuidadores: Questionário de Avaliação da Sobrecarga do Cuidador Informal (QASCI); Escala de Apoio Social do Medical Outcomes Study (MOS). Resultados: Foram encontradas fortes correlações entre ALSFRS-R e ALSAQ-40 (r = - 0.709 e p &lt; 0.010), entre o domínio Atividades de Vida Diária e a ALSFRS-R (r = - 0.877 e p &lt; .001), entre os domínios da QASCI e MOS, Mecanismo de Eficácia e de Controle e Apoio material (r = - 0.598 e p &lt; 0.040), Satisfação com o Papel e com o Familiar e Apoio Afetivo (r = - 0.604 e p &lt; 0.037), e Suporte Familiar e Interação Social Positiva (r = - 0.683 e p &lt; 0.014). Conclusão: A funcionalidade e a qualidade de vida do paciente com ELA influenciam na provisão de cuidados, o suporte social percebido foi uma variável moderadora da sobrecarga de estresse dos cuidadores.    

Dyspnea as a Fatigue-Promoting Factor in ALS and the Role of Objective Indicators of Respiratory Impairment

ContextThere is no evidence-based treatment for fatigue in amyotrophic lateral sclerosis (ALS), and identification of treatable causes determines management strategies. Although dyspnea is a key symptom of ALS and effectively treatable, it has not been sufficiently investigated whether dyspnea may be a fatigue-promoting factor. ObjectivesTo determine the level of fatigue in dyspneic ALS patients and whether fatigue is promoted by dyspnea. We further evaluated the correlation of fatigue with respiratory function tests. MethodsAbout 101 dyspneic patients and 20 matched controls completed the ALS Functional Rating Scale—Extension and the Fatigue Severity Scale. Dyspneic patients additionally completed the Dyspnea-ALS Scale and the ALS Assessment Questionnaire and underwent respiratory function tests (forced vital capacity, sniff nasal inspiratory pressure, mean inspiratory and expiratory pressure with respective relaxation rates, and blood gases). Multiple regression and correlation analyses were conducted. ResultsDyspneic patients had significantly higher fatigue scores than nondyspneic patients, and their fatigue significantly affected quality of life. Dyspnea alone explained up to 24% of the variance in fatigue. No associations were observed between fatigue and respiratory function tests. Patients with noninvasive ventilation reported significantly more dyspnea and fatigue. ConclusionFatigue is a frequent and bothersome symptom in dyspneic ALS patients. Dyspnea-related distress is, in contrast to objective indicators of respiratory impairment, a determining factor of experienced fatigue. There is an urgent need for further symptom relief beyond noninvasive ventilation. Adequate treatment of dyspnea has the potential for synergies in symptom management arising from the association between fatigue and dyspnea.

Associations between illness cognitions and health-related quality of life in the first year after diagnosis of amyotrophic lateral sclerosis

ObjectiveTo describe illness cognitions among patients with amyotrophic lateral sclerosis (ALS), to study cross-sectional associations between illness cognitions and health-related quality of life (HRQoL) and to study the predictive value of illness cognitions measured shortly after the diagnosis for HRQoL at follow-up. MethodsProspective longitudinal design. We administered Self-report questionnaires at study onset (n = 72) and follow-up (n = 48). Median follow-up period was 10.0 months. At baseline median ALS Functional Rating Scale-Revised was 43, median time since onset of symptoms was 13.6 months, 79% of patients presented with spinal onset. Illness cognitions Helplessness, Acceptance and Disease Benefits were measured with the Illness Cognitions Questionnaire (ICQ) and HRQoL with the ALS Assessment Questionnaire (ALSAQ-40). Correlational and regression analyses were used. ResultsPatients experienced more Helplessness at follow-up. We found no significant changes in Acceptance or Disease Benefits at follow-up. In cross-sectional analyses, Helplessness was independently related to worse HRQoL at baseline (β = 0.44; p = .001) and Acceptance and Disease Benefits were independently related to worse HRQoL at follow-up (β = −0.17, p = .045) and (β = −0.186, p = .03 respectively). Longitudinal analyses showed that, adjusted for disease severity at baseline, Helplessness at baseline was a predictor of worse HRQoL at follow-up (β = 0.43; p = .006). None of the illness cognitions were a significant predictor of HRQoL with adjustment for baseline HRQoL. ConclusionHelplessness was independently associated with HRQoL in the cross-sectional and longitudinal analyses. These results can help us identify patients shortly after diagnosis who might benefit from psychological interventions.

Functionality and quality of life in patients with amyotrophic lateral sclerosis: a cross-sectional study

PurposeTo identify factors constraining functionality and life quality in amyotrophic lateral sclerosis (ALS).MethodsCross-sectional study in 6 ALS patients aged 51.16 ± 8.70 years, with disease duration of 3.16 ± 2.63 years. Amyotrophic Lateral Sclerosis Severity Scale (ALSSS) and Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R/BR) served to evaluate functionality; Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40) analysed life quality. Descriptive statistics was applied to clinical characteristics. The correlation of clinical profile and disease duration with the degree of functionality and life quality was verified by Spearman’s correlation, with values considered significant if &lt;i&gt;p&lt;/i&gt; &lt; 0.05 and &lt;i&gt;r&lt;/i&gt; &gt; 0.8.ResultsThere was a positive correlation of age with ALSFRS (&lt;i&gt;r&lt;/i&gt; = 0.852), disease duration with ALSAQ-40/physical mobility (&lt;i&gt;r&lt;/i&gt; = 0.805), ALSFRS-R with ALSSS (&lt;i&gt;r&lt;/i&gt; = 0.972), physical mobility with food (&lt;i&gt;r&lt;/i&gt; = 0.881), and communication with emotional status (&lt;i&gt;r&lt;/i&gt; = 0.947). Negative correlations were observed of age with mobility (&lt;i&gt;r&lt;/i&gt; = –0.805) and emotional status (&lt;i&gt;r&lt;/i&gt; = –0.876), disease duration with ALSFRS-R (&lt;i&gt;r&lt;/i&gt; = –0.858) and ALSSS (&lt;i&gt;r&lt;/i&gt; = –0.930), ALSFRS-R with physical mobility (&lt;i&gt;r&lt;/i&gt; = –0.978) and food (&lt;i&gt;r&lt;/i&gt; = –0.855), ALSSS with physical mobility (&lt;i&gt;r&lt;/i&gt; = –0.910) and daily life activity (&lt;i&gt;r&lt;/i&gt; = –0.874).ConclusionsLonger disease was associated with lower functionality and poor life quality. Better functionality indicated lower severity and higher life quality in physical mobility and food. Higher severity was bound with poor life quality in physical mobility and daily life activity. We also observed correlations of physical mobility with food, and communication with emotional status.

Validity and Reliability of the Turkish Version of the Amyotrophic Lateral Sclerosis Assessment Questionnaire.

The aim of this study was to adapt the Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ), developed for the evaluation of quality of life in amyotrophic lateral sclerosis (ALS) patients, into the Turkish society. This methodological study was conducted in 92 ALS patients registered in 2 chapters (Istanbul and Izmir) of Turkish ALS-Motor Neuron Diseases Association. The expert opinion and forward-backward translation methods were applied for the linguistic validity of the ALSAQ (long and short versions: ALSAQ-40 and ALSAQ-5, respectively). In the analysis of internal consistency, the Cronbach's α reliability coefficient was .96 for the total scale, whereas it was between .88 and .98 for the 5 dimensions. The dimensions of the long form were correlated with the items of the short form. The Turkish versions of the ALSAQ short and long forms are valid and reliable tools to be used in the assessment of the quality of life in ALS patients.

Poor emotional well-being is associated with rapid progression in amyotrophic lateral sclerosis.

The study aimed to determine the impact of emotional well-being on disease aggressiveness and survival in amyotrophic lateral sclerosis (ALS). In 224 patients with ALS (without significant cognitive deficits) the revised ALS Functional Rating Scale (physical function), the ALS Assessment Questionnaire (ALSAQ-40) for health-related Quality of Life and survival data were collected. Data analysis comprised logistic regression, Kaplan-Meier curves analyses and Cox regression model. Most patients reported to be worried about how the disease will affect them in the future and 67% reported to feel depressed. Patients with good emotional well-being were characterized by better physical function (higher ALSFRS-R) and lower disease aggressiveness. The association between high emotional well-being and lower disease aggressiveness was confirmed in the univariate analysis and also after adjustment for known predictors of disease progression. In the Kaplan-Meier survival curve analysis the overall 1-year, 2-year and 3-year mortality did not significantly differ between patients with poor and good emotional well-being. Our study demonstrates an association between emotional well-being and disease progression. Knowing that subjective well-being is neither a necessary nor a sufficient cause of health, longitudinal studies are necessary to explore when well-being does and does not influence disease progress and survival in ALS.

Cognitive deficits have only limited influence on health-related quality of life in amyotrophic lateral sclerosis

Objective To explore the association between cognitive deficits and health-related quality of life in amyotrophic lateral sclerosis (ALS). Methods The revised ALS Functional Rating Scale (ALSFRS-R for physical impairment), the ALS Assessment Questionnaire (ALSAQ-40 for health-related quality of life) and the Edinburgh Cognitive and Behavioral ALS Screen (ECAS for cognition) were assessed in 125 patients with ALS. Correlations between ALSAQ-40 domains and ECAS functions were tested using Spearman correlation. Linear regression was used to evaluate the relationship between dysphagia, depression, hopelessness, pain (all derived from corresponding items from the ALSFRS-R or ALSAQ-40), ALSFRS-R, ECAS and the ALSAQ-40. Results Verbal fluency, language and executive function were disturbed in 69 (55%), 54 (43%) and 41 (33%) patients, respectively. In the ALS non-specific domains the memory and visuospatial function were impaired in 44 (35%) and 12 (10%) patients. In the non-demented group the five ECAS functions did not correlate with the ALSAQ-40 subdomains. The ALSFRS-R score, hopelessness, pain, and depression explained 65% of the ALSAQ-40 SI variance; the ECAS total score did not significantly predict ALSAQ-40 summary index. The ECAS visuospatial, executive function and fluency significantly predicted emotional well-being (adjusted R 2 = 0.08). When the model was controlled for depression, hopelessness and pain none of the ECAS functions (visuospatial, executive function and fluency) were significant predictors of emotional well-being. Conclusion Deficits in visuospatial function, executive function and fluency constrain the ability to manage activities of daily living and this might cause decline in well-being.