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Purpose. To demonstrate, with the help of an example of cognitive treatment for patients with chronic low back pain, how a systematic description of the content and theoretical underpinnings of treatment can help to improve clinical practice. Methods. A conceptual analysis, two types of theories, and a programme-theory framework were instrumental in systematically specifying the content of the treatment and the underlying assumptions. Results. A detailed description of the cognitive treatment, including: (i) The intended outcomes; (ii) the related treatment components; (iii) the therapeutic process that is expected to mediate between outcomes and components, (iv) the conditions for optimal application; and (v) the guiding principles. Conclusions. The systematic description of the treatment revealed important issues for clinical practice, such as the patient and therapist characteristics that are needed for optimal provision of cognitive treatment. The discussions on the role of theory in rehabilitation practice are taken one step further in this clinical commentary: instead of simply describing the problems, we also demonstrated a means to tackle them. [ABSTRACT FROM AUTHOR]

Background: Fatigue is a major problem of cancer patients. Thirty percent of cancer survivors report serious fatigue three years after finishing treatment. There is evidence that physical exercise during cancer treatment reduces fatigue. This may also lead to an improvement of quality of life. Such findings may result in a decrease of healthcare related expenditures and societal costs due to sick leave. However, no studies are known that investigated these hypotheses. Therefore, the primary aim of our study is to assess the effect of exercise during cancer treatment on reducing complaints of fatigue and on reducing health service utilisation and sick leave.Methods/design: The Physical Activity during Cancer Treatment study is a multicentre randomised controlled trial in 150 breast and 150 colon cancer patients undergoing cancer treatment. Participants will be randomised to an exercise or a control group. In addition to the usual care, the exercise group will participate in an 18-week supervised group exercise programme. The control group will be asked to maintain their habitual physical activity pattern. Study endpoints will be assessed after 18 weeks (short term) and after 9 months (long term). Validated questionnaires will be used.Primary Outcome: fatigue (Multidimensional Fatigue Inventory and Fatigue Quality List) and cost-effectiveness, health service utilisation and sick leave. Secondary outcome: health related quality of life (European Organisation Research and Treatment of Cancer-Quality of Life questionnaire-C30, Short Form 36 healthy survey), impact on functioning and autonomy (Impact on functioning and autonomy questionnaire), anxiety and depression (Hospital Anxiety and Depression Scale), physical fitness (aerobic peak capacity, muscle strength), body composition and cognitive-behavioural aspects. To register health service utilisation and sick leave, participants will keep diaries including the EuroQuol-5D. Physical activity level will be measured using the Short Questionnaire to Assess Health-Enhancing Physical Activity and will be monitored with an exercise log and a pedometer.Discussion: This study investigates the (cost)-effectiveness of exercise during adjuvant treatment of patients with breast or colon cancer. If early physical exercise proves to be (cost) effective, establishing standardised physical exercise programmes during cancer treatment will be planned.Trial Registration: Current Controlled trials ISRCTN43801571, Dutch Trial Register NTR2138. [ABSTRACT FROM AUTHOR]

Background: In order to develop a feasible prehabilitation program before surgery of NSCLC, this study aimed to gain insight into beliefs, facilitators, and barriers of (1) healthcare professionals to refer patients to a prehabilitation program, (2) patients to participate in and adhere to a prehabilitation program, and (3) informal caregivers to support their loved ones., Methods: Semi-structured interviews were conducted with healthcare professionals, patients who underwent surgery for NSCLC, and their informal caregivers. The capability, opportunity, and motivation for behavior-model (COM-B) guided the development of the interview questions. Results were analyzed thematically., Results: The interviews were conducted with twelve healthcare professionals, seventeen patients, and sixteen informal caregivers. Four main themes were identified: (1) content of prehabilitation and referral, (2) organizational factors, (3) personal factors for participation, and (4) environmental factors. Healthcare professionals mentioned that multiple professionals should facilitate the referral of patients to prehabilitation within primary and secondary healthcare involved in prehabilitation, considering the short preoperative period. Patients did not know that a better preoperative physical fitness and nutritional status would make a difference in the risk of postoperative complications. Patients indicated that they want to receive information about the aim and possibilities of prehabilitation. Most patients preferred a group-based physical exercise training program organized in their living context in primary care. Informal caregivers could support their loved one when prehabilitation takes place by doing exercises together., Conclusion: A prehabilitation program should be started as soon as possible after the diagnosis of lung cancer. Receiving information about the purpose and effects of prehabilitation in a consult with a physician seems crucial to patients and informal caregivers to be involved in prehabilitation. Support of loved ones in the patient's own living context is essential for adherence to a prehabilitation program., (© 2023. The Author(s).)

Introduction: Pain neuroscience education is part of interdisciplinary pain management programs (IPMPs). To date, the role of health literacy on patients' understanding of pain neuroscience education has not sufficiently been examined., Objectives: Drawing on interviews with patients with diverse levels of health literacy, this article explores patient perspectives on pain neuroscience education., Methods: Purposively sampled patients from an IPMP were interviewed twice (waiting list and after 4 weeks). A directed qualitative content analysis was performed with the Integrated Conceptual Model of Health Literacy as an analytic framework., Results: Thirteen patients with chronic musculoskeletal pain were interviewed: 4 men and 9 women aged from 21 to 77 years with diverse educational and mostly low health literacy. One participant dropped out after baseline. Some participants gained access to health information actively; others relied on the expertise of their healthcare providers. Most participants did not seem to receive the information in the pain neuroscience education as intended, experienced difficulties with understanding the message, negatively appraised the information, and were not able to apply this in their daily lives. Health literacy levels likely played a role in this., Conclusions: Pain neuroscience education tailored to patients' health literacy levels, information needs, and learning strategies is needed., Competing Interests: The authors have no conflict of interest to declare.Sponsorships or competing interests that may be relevant to content are disclosed at the end of this article., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The International Association for the Study of Pain.)

Aim: To gain insight into parents' perspectives about their decision-making process concerning nusinersen treatment for their child, including perceived needs and concerns, and to explore factors that influence this process., Method: This was an exploratory qualitative interview study among parents of children with spinal muscular atrophy types 1 to 3. Data were analysed using inductive thematic analysis., Results: Nineteen parents of 16 children representing 13 families participated. A wide variety of perspectives was reported ranging from a biomedical approach, which focused on battling the disease, to a holistic approach, which aimed for a good quality of life for their child. The most important factors that helped parents to decide were honest and neutral communication with their physician and access to available information., Interpretation: It is important physicians understand that there are different perspectives influencing the decision-making process. Physicians should create an environment that allows parents to accept or reject treatment by communicating honestly and openly with them and by discussing both options extensively. Clear information about pros and cons, recent developments in research, and the experiences of other parents should be made available to enable parents to make an informed decision. What this paper adds Parents perceived different needs and concerns about nusinersen treatment, which emphasized individual differences. Parents' perspectives varied from battling the disease to preserving quality of life. Life expectancy, stopping deterioration, and improving quality of life were the perceived benefits of nusinersen treatment. Open communication about the pros and cons of treatment with clinicians facilitated decision-making. Clear and honest information facilitated the alignment of values and goals., (© 2021 The Authors. Developmental Medicine & Child Neurology published by John Wiley & Sons Ltd on behalf of Mac Keith Press.)

Objective: To evaluate the fidelity of delivery of a nurse-led intervention to enhance physical activity in patients at risk for cardiovascular diseases, the Activate intervention, by assessing: (1) self-reported fidelity of delivery; (2) observed fidelity of delivery; (3) quality of delivery of the Activate intervention and (4) nurses' beliefs about their capability, motivation, confidence and effectiveness towards delivering the Activate intervention, including behavioural change techniques., Design: An observational study., Setting: General practices in the Netherlands., Participants: Primary care nurses (n=20) from 16 general practices., Primary and Secondary Outcome Measures: Nurses' self-reported fidelity was evaluated using checklists (n=282), and the observed fidelity and quality of delivery were examined using audiorecordings of consultations of the delivery of the Activate intervention (n=42). Nurses' beliefs towards delivering the intervention were assessed using questionnaires (n=72)., Results: The self-reported fidelity was 88.1% and observed fidelity was 85.4%, representing high fidelity. The observed fidelity of applied behavioural change techniques was moderate (75.0%). The observed quality of delivery was sufficient and varied among nurses (mean 2.9; SD 4.4; range 0-4). Nurses' beliefs about their capability, motivation, confidence and effectiveness towards delivering the intervention increased over time., Conclusions: Nurses delivered most intervention components as intended with sufficient quality. Nurses believed they were capable, motivated and confident to deliver the intervention. They believed the intervention was effective to increase patients' physical activity level. Despite the high fidelity and moderate fidelity of applied behavioural change techniques, the varying quality of delivery within and across nurses might have diluted the effectiveness of the Activate intervention., Trial Registration Number: NCT02725203., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: To understand better the success of self-management interventions and to enable tailoring of such interventions at specific subgroups of patients, the nurse-led Activate intervention is developed targeting one component of self-management (physical activity) in a heterogeneous subgroup (patients at risk of cardiovascular disease) in Dutch primary care., Aim: The aim of this study was to evaluate the effectiveness of the Activate intervention and identifying which patient-related characteristics modify the effect., Methods: A two-armed cluster-randomised controlled trial was conducted comparing the intervention with care as usual. The intervention consisted of four nurse-led behaviour change consultations within a 3-month period. Data were collected at baseline, 3 months and 6 months. Primary outcome was the daily amount of moderate to vigorous physical activity at 6 months. Secondary outcomes included sedentary behaviour, self-efficacy for physical activity, patient activation for self-management and health status. Prespecified effect modifiers were age, body mass index, level of education, social support, depression, patient provider relationship and baseline physical activity., Results: Thirty-one general practices ( n  = 195 patients) were included (intervention group n  = 93; control group n  = 102). No significant between-group difference was found for physical activity (mean difference 2.49 minutes; 95% confidence interval -2.1; 7.1; P  = 0.28) and secondary outcomes. Patients with low perceived social support ( P  = 0.01) and patients with a low baseline activity level ( P  = 0.02) benefitted more from the intervention., Conclusion: The Activate intervention did not improve patients' physical activity and secondary outcomes in primary care patients at risk of cardiovascular disease. To understand the results, the intervention fidelity and active components for effective self-management require further investigation.Trial registration: ClinicalTrials.gov NCT02725203.

Objective: To describe the course of performance of activities (observed and self-reported) of people with chronic idiopathic axonal polyneuropathy (CIAP) over 4 years and to assess the associations with muscle strength, sensory function, and psychological personal factors (intention, perceived behavior control [PBC], and feelings of depression or anxiety)., Design: Prospective observational study with measurement at baseline, 6 months, 1 year, and 4 years., Setting: Outpatient neurology clinic., Participants: People with CIAP (N=92)., Main Outcome Measures: Walking was measured using the shuttle-walk test (SWT), a pedometer (mean step count/d), and the "physical functioning" subscale of the Short Form-36 questionnaire. Muscle strength and sensory function were measured using a MicroFET handheld dynamometer and the Sensory Modality Sum score. Personal factors were assessed with the Hospital Anxiety and Depression Scale, and intention and PBC were assessed with a protocolized questionnaire., Results: Multilevel model analysis showed a significant decrease over time in mean scores in performance of activities (SWT, step count), which was associated with older age and loss of muscle strength (SWT: β=73.392, step count: β=676.279, P<.001). Limitations in self-reported functioning (physical functioning) significantly increased and were associated with older age (β=-0.916, P=.001), increased comorbidity (β=-6.978, P=.024), loss of muscle strength (β=7.074, P<.001), low PBC (β=0.744, P<.001), and increased feelings of depression (β=1.481, P<.001)., Conclusions: Performance of activities of people with CIAP decreased over time (SWT, step count, physical functioning). Older age, loss of muscle strength, comorbidity, feelings of depression, and low perceived behavior control were associated with this decrease. However, there were considerable individual differences., (Copyright © 2020 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.)

Purpose: Patients with spinal muscular atrophy (SMA) suffer from slowly progressive weakness of axial, respiratory and proximal muscles, leading to restrictions in activity and participation. This study aims to investigate patients' level of psychological well-being, using the International Classification of Functioning model and self-determination theory as theoretical frameworks. Materials and methods: In this cross-sectional study, adults with SMA were invited to complete a questionnaire. Instruments to assess psychological well-being included the Satisfaction with Life Scale, the Rosenberg Self-Esteem Scale and the Positive and Negative Affect Scale. Hierarchical lineal regression analyses were performed to investigate the contribution of participation (International Classification of Functioning model) and satisfaction of the need for autonomy, competence and relatedness (self-determination theory) to well-being. Results: Ninety-two respondents (67%) returned the questionnaire. Levels of psychological well-being were comparable to that of healthy reference samples. Well-being was unrelated to sociodemographic variables or illness characteristics. By contrast, well-being was closely related to respondents' satisfaction with participation, and their sense of autonomy, competence and relatedness. Conclusions: This study illustrates the relevance of psychological needs for understanding well-being of individuals with SMA. Supporting patients in meeting their psychological needs should become an objective of person-centred care for this population.Implications for rehabilitationSpinal muscular atrophy is a rare inherited disease, characterized by slowly progressive muscle weakness.Psychological well-being, including satisfaction with life, self-esteem and emotional functioning of adults with spinal muscular atrophy appears very comparable with that of healthy reference samples.In line with the International Classification of Functioning framework, well-being in adults with spinal muscular atrophy may be improved by increasing their (satisfaction with) participation.Moreover, clinical assessment and management should focus on optimizing patients' satisfaction with their basic psychological needs (autonomy, competence, relatedness), as this is strongly related to indices of psychological well-being.

Objective: To evaluate whether a blended (face-to-face and online) psychosocial support program for caregivers of patients with Amyotrophic Lateral Sclerosis (ALS) and Progressive Muscular Atrophy (PMA), aimed at enhancing feeling of control over caregiving, reduces psychological distress. Methods: A randomized controlled trial using a wait-list control design was conducted. Caregiver-patient dyads were randomly assigned to either the support program ( n  = 74) or to a wait-list control group ( n  = 74). The support program, based on Acceptance and Commitment Therapy, consists of 1 face-to-face contact, 6 online guided modules and 1 telephone contact. Participants filled in questionnaires at baseline, 3 and 6 months. Caregivers' feeling of control over caregiving was assessed using two self-efficacy measures. Primary outcome was caregivers' psychological distress. Secondary outcomes included caregiver burden, caregiver quality of life and patients' quality of life and psychological distress. Intention-to-treat and per-protocol analyses were performed using linear mixed models. Results: The support program had no effect on the primary or secondary outcomes, despite a significant positive effect on the intervening variable self-efficacy with regard to control over thoughts. Almost half of the caregivers did not complete the intervention with the most frequently reported reason being lack of time. Caregivers who completed the intervention evaluated the support program positively. Conclusions: The support program did not reduce distress of partners of patients with ALS/PMA but may be beneficial by increasing feeling of control over the caregiving situation. The high level of intervention drop outs may have limited the ability to detect an intervention effect. Trial registration: Netherlands Trial Registry NTR5734, registered 28 March 2016.

Caregivers of Amyotrophic Lateral Sclerosis (ALS) and Progressive Muscular Atrophy (PMA) patients often experience psychological distress. Yet, it is unclear which factors explain the variance in psychological distress. This study seeks to evaluate how care demands and perceived control over caregiving influence psychological distress using moderation and mediation analysis. Data were collected as part of a RCT and 148 partners of patients with ALS or PMA were included. Psychological distress was assessed using the Hospital Anxiety and Depression Scale. Care demands were operationalized as physical functioning (Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised) and behavioural changes of the patient (Amyotrophic Lateral Sclerosis-Frontotemporal Dementia-Questionnaire). Perceived control over caregiving was assessed using items adapted from the Job Content Questionnaire. Results showed that more behavioural changes and lower perceived control over caregiving were associated with higher levels of psychological distress in caregivers. Patients' physical functioning was not significantly related to caregivers' psychological distress. No moderation or mediation effects were found of perceived control over caregiving on the relationship between demand and psychological distress. Monitoring, psychoeducation and caregiver support with regard to behavioural changes in patients, seem to be important for the wellbeing of caregivers. Caregivers' perceived control might be a target for future interventions.

Objective: The Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) is widely applied to assess disease severity and progression in patients with motor neuron disease (MND). The objective of the study is to assess the inter-rater and intra-rater reproducibility, i.e., the inter-rater and intra-rater reliability and agreement, of a self-administration version of the ALSFRS-R for use in apps, online platforms, clinical care and trials., Methods: The self-administration version of the ALSFRS-R was developed based on both patient and expert feedback. To assess the inter-rater reproducibility, 59 patients with MND filled out the ALSFRS-R online and were subsequently assessed on the ALSFRS-R by three raters. To assess the intra-rater reproducibility, patients were invited on two occasions to complete the ALSFRS-R online. Reliability was assessed with intraclass correlation coefficients, agreement was assessed with Bland-Altman plots and paired samples t -tests, and internal consistency was examined with Cronbach's coefficient alpha., Results: The self-administration version of the ALSFRS-R demonstrated excellent inter-rater and intra-rater reliability. The assessment of inter-rater agreement demonstrated small systematic differences between patients and raters and acceptable limits of agreement. The assessment of intra-rater agreement demonstrated no systematic changes between time points; limits of agreement were 4.3 points for the total score and ranged from 1.6 to 2.4 points for the domain scores. Coefficient alpha values were acceptable., Discussion: The self-administration version of the ALSFRS-R demonstrates high reproducibility and can be used in apps and online portals for both individual comparisons, facilitating the management of clinical care and group comparisons in clinical trials., Competing Interests: Competing interests: LAB, CDS, HHGT, SMAGV, RPAVE and JMAV-M have nothing to disclose. MAvE received grants from the Netherlands Organization for Health Research and Development (Veni scheme), The Thierry Latran Foundation, Joint Programme – Neurodegenerative Disease Research (JPND) and the Netherlands ALS Foundation (Stichting ALS Nederland). He received travel grants from Shire (previously Baxalta) and serves on the medical ethical review board of University Medical Centre Utrecht. LHvdB reports grants from ALS Foundation Netherlands, grants from The Netherlands Organization for Health Research and Development (Vici scheme), grants from The Netherlands Organization for Health Research and Development (SOPHIA, STRENGTH, ALS-CarE project), funded through the EU JPND, personal fees from Shire (previously Baxalta), personal fees from Biogen, personal fees from Cytokinetics, other from Prinses Beatrix SpierFonds, other from Latran Foundation, outside the submitted work., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: After deployment service members need to adapt to civilian life and return to participation in family, vocational and community life., Aims: To assess the level of activity and participation of service members with combat-related injury after their rehabilitation intervention and to measure the effect of injury severity, adaptive coping, number of deployments and traumatic stress., Methods: The physical functioning scale of the 36-Item Short Form Health Survey (SF-36 PF), the Assessment of Life Habits short version (LIFE-H), the Impact of Event Scale (IES-R) and the Cognitive Emotion Regulation Questionnaire (CERQ) were administered to service members who sustained combat-related injury. The Injury Severity Score (ISS) was calculated, and the number of deployments was noted. Correlations were calculated between the LIFE-H and ISS, IES-R, number of deployments and adaptive coping and between the SF-PF and ISS, IES-R, number of deployments and adaptive coping., Results: The response rate was 55% (32 service members). A moderate correlation was found between LIFE-H and IES (r = -0.49) and a very weak correlation was found between LIFE-H and injury severity (r = 0.31). No correlation was found between SF-36 PF and ISS, IES, number of deployments or CERQ and between LIFE-H and number of deployments or CERQ., Conclusions: A moderate correlation was found between level of participation and traumatic stress in service members with combat-related injury in a 5-year follow-up. Therefore, it is advisable to screen for traumatic stress symptoms and monitor these symptoms during the rehabilitation intervention., (© The Author(s) 2019. Published by Oxford University Press on behalf of the Society of Occupational Medicine. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Background: Partners are often the main caregivers in the care for patients with amyotrophic lateral sclerosis (ALS) and progressive muscular atrophy (PMA). Providing care during the progressive and fatal disease course of these patients is challenging and many caregivers experience feelings of distress. A blended psychosocial support program based on Acceptance and Commitment Therapy was developed to support partners of patients with ALS and PMA. The aim of this qualitative study is to gather insight into experiences with different components of the support program (program evaluation) and to discover what caregivers gained from following the program (mechanisms of impact)., Methods: Individual in-depth interviews, about caregivers' experiences with the support program were conducted with 23 caregivers of ALS/PMA patients enrolled in a randomized controlled trial designed to measure the effectiveness of the blended psychosocial support program. The program, performed under the guidance of a psychologist, consists of psychoeducation, psychological and mindfulness exercises, practical tips and information, and options for peer contact. Interviews were audio-recorded, transcribed verbatim and analyzed thematically., Results: The program evaluation showed that caregivers perceived each component of the program as beneficial but ambivalent reactions were expressed about the mindfulness exercises and peer contact functions. Caregivers expressed the need for a more personalized program with respect to the order and timing of the modules and wanted to continue the support program for a longer time. The main mechanism of impact of the program that caregivers reported was that they became more aware of their own situation. They further indicated that the program helped them to perceive control over the caregiving situation, to accept negative emotions and thoughts, to be there for their partner and feel acknowledged., Conclusions: The blended psychosocial support program for caregivers of patients with ALS/PMA is valued by caregivers for enhancing self-reflection on their challenging situation which stimulated them to make choices in line with their own needs and increased their feeling of control over caregiving. The different components of the program were overall appreciated by caregivers, but the mindfulness and peer support components should be further adapted to the needs of the caregivers., Trial Registration: Dutch Trialregister NTR5734 , registered 28 March 2016.

Purpose: Maintaining high adherence rates (session attendance and compliance) in exercise programs during breast cancer treatment can be challenging. We aimed to identify adherence rates and predictors to an exercise program during adjuvant breast cancer treatment., Methods: Ninety-two patients with localized breast cancer undergoing chemotherapy were randomly assigned to an 18-week supervised moderate-to-high intensity aerobic and resistance exercise program, including two 1-hour sessions/week. Additionally, participants were asked to be physically active for at least 30 minutes/day on at least three other days. We report median percentages for attendance, compliance with the prescribed duration and intensity of aerobic and muscle strength exercises, and the exercise advice given. Predictors included in univariate and multivariable linear regression models were demographical, tumor- and treatment-related factors, constructs of the theory of planned behavior, psychological and physical factors., Results: Patients attended 83% (interquartile range: 69-91%) of the supervised sessions. Compliance with the duration of aerobic exercise, high-intensity aerobic exercise (cycling at the ventilatory threshold), muscle strength exercises and the exercise advice were 88%(64-97%), 50%(22-82%), 84%(65-94%) and 61%(33%-79%), respectively. Education, radiotherapy, BMI and physical fatigue were important predictors of adherence to supervised exercise. Beliefs about planned behaviors were important predictors, especially for compliance with the exercise advice., Conclusions: Attendance to and compliance with an 18-week aerobic and strength exercise program were high. The lowest compliance was found for high-intensity supervised aerobic exercise. The identified predictors should be considered when designing or adapting exercise programs for patients with localized breast cancer to increase adherence., Trial Registration: Current Controlled Trials ISRCTN43801571 Dutch Trial Register NTR2138., Competing Interests: The authors have declared that no competing interests exist.

Objectives : To assess psychological distress levels over time in caregivers of patients in the early stage of amyotrophic lateral sclerosis (ALS) and to investigate the association between caregivers' coping styles and psychological distress over time. Methods : Fifty-four caregivers were followed in a RCT of patients in the early stage of ALS. Questionnaires were administered at baseline, 4, 7, and 10 months. Psychological distress was assessed using the Hospital Anxiety and Depression Scale (HADS) and task-, emotion-, and avoidance-oriented coping styles were identified with the short version of the Coping Inventory for Stressful Situations (CISS-21). Analyses were performed using linear mixed models. Results : Caregivers' psychological distress increased by 0.24 points on the HADS per month ( p  = 0.01). An emotion-oriented coping style was positively associated with psychological distress in caregivers ( b  = 0.90, p  < 0.01), but did not influence the development of psychological distress over time. The avoidance-oriented coping style and the task-oriented coping style were not significantly related to psychological distress. Conclusion : Feelings of distress increase in ALS caregivers during the course of the disease of the patient. Emotion-oriented coping is related to psychological distress; however, emotional coping and distress might represent overlapping constructs. Focusing on one coping style in the care for ALS caregivers may be too simplistic for the complex situations they face, as it ignores the fact that people may require different coping strategies in different situations .

Proximal spinal muscular atrophy (SMA) causes severe physical limitations but also has a major impact on the lives of parents. The aim of this study was to investigate participation and mental well-being (burden, emotional distress and satisfaction with participation) of parents of home-living patients with SMA. Caregiver burden was assessed with the Caregiver Strain Index, emotional distress with the Hospital Anxiety and Depression Scale and satisfaction with participation with the Utrecht Scale for Evaluation of Rehabilitation-Participation. Because the majority of parents were mothers of home-living SMA patients (76%), further analyses were restricted to mothers. Seventy-seven percent of mothers of patients with SMA had paid work. A substantial proportion of mothers (76%) perceived high caregiver burden. Burden, emotional distress and satisfaction with participation were comparable between mothers of children and mothers of adults with SMA. Caregivers' participation in leisure activities was significantly related to their perceived level of caregiver burden, emotional distress and satisfaction with participation. Mothers engaging in more social and leisure activities reported lower emotional distress and caregiver burden. Considering the high level of burden attention should be paid to mental well-being of primary caregivers of patients with SMA. Caregivers should be motivated to keep participating in social/leisure activities., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Objective: The aim of this study was to explore the support needs of Dutch informal caregivers of patients with amyotrophic lateral sclerosis (ALS)., Method: Individual semi-structured interviews were conducted with 21 caregivers of ALS patients. Audio-taped interviews were transcribed and data were analyzed thematically., Result: A total of four global support needs emerged: "more personal time", "assistance in applying for resources", "counseling", and "peer contact". Despite their needs, caregivers are reluctant to apply for and accept support. They saw their own needs as secondary to the needs of the patients., Significance of Results: ALS seems to lead to an intensive caregiving situation with multiple needs emerging in a short period. This study offers targets for the development of supportive interventions. A proactive approach seems essential, acknowledging the importance of the role of the caregivers in the care process at an early stage, informing them about the risk of burden, monitoring their wellbeing, and repeatedly offering support opportunities. Using e-health may help tailor interventions to the caregivers' support needs.

Objective: To explore the experiences of patients at risk for cardiovascular disease in primary care with the Activate intervention in relation to their success in increasing their physical activity., Methods: A convergent mixed methods study was conducted, parallel to a cluster-randomised controlled trial in primary care, using a questionnaire and semi-structured interviews. Questionnaires from 67 patients were analysed, and semi-structured interviews of 22 patients were thematically analysed. Experiences of patients who had objectively increased their physical activity (responders) were compared to those who had not (non-responders). Objective success was analysed in relation to self-perceived success., Results: The questionnaire and interview data corresponded, and no substantial differences among responders and non-responders emerged. Participating in the intervention increased patients' awareness of their physical activity and their physical activity level. Key components of the intervention were the subsequent support of nurses with whom patients' have a trustful relationship and the use of self-monitoring tools. Patients highly valued jointly setting goals, planning actions, receiving feedback and review on their goal attainment and jointly solving problems. Nurses' support, the use of self-monitoring tools, and involving others incentivised patients to increase their physical activity. Internal circumstances and external circumstances challenged patients' engagement in increasing and maintaining their physical activity., Conclusion: Patients experienced the Activate intervention as valuable to increase and maintain their physical activity, irrespective of their objective change in physical activity. The findings enable the understanding of the effectiveness of the intervention and implementation in primary care., Trial Registration: ClinicalTrials.gov NCT02725203., Competing Interests: The authors have declared that no competing interests exist.

Background: The Edinburgh cognitive and behavioral ALS screen (ECAS) was developed specifically to detect cognitive and behavioral changes in patients with amyotrophic lateral sclerosis (ALS). Differences with regard to normative data of different (language) versions of neuropsychological tests such as the ECAS exist., Objective: To derive norms for the Dutch version of the ECAS., Methods: Normative data were derived from a large sample of 690 control subjects and cognitive profiles were compared between a matched sample of 428 patients with ALS and 428 control subjects., Results: Age, level of education, and sex were significantly associated with performance on the ECAS in the normative sample. ECAS data were not normally distributed and therefore normative data were expressed as percentile ranks. The comparison of ECAS scores between patients and control subjects demonstrated that patients obtained significantly lower scores for language, executive function, verbal fluency, and memory, which is in line with the established cognitive profile of ALS., Conclusion: For an accurate interpretation of ECAS results, it is important to derive normative data in large samples with nonparametric methods. The present normative data provide healthcare professionals with an accurate estimate of how common or uncommon patients' ECAS scores are and provide a useful supplement to existing cut-off scores.

Objectives: Cognitive and behavioural changes within the spectrum of frontotemporal dementia (FTD) are observed frequently in patients with amyotrophic lateral sclerosis (ALS). Whether these changes also occur in other forms of motor neuron disease (MND) is not well studied. We therefore systemically screened a large cohort of patients with primary lateral sclerosis (PLS) and progressive muscular atrophy (PMA) for cognitive and behavioural changes, and subsequently compared our findings with a cohort of patients with ALS., Methods: Using a set of screening instruments (Edinburgh Cognitive and Behavioural ALS Screen, ALS and Frontotemporal Dementia Questionnaire, Frontal Assessment Battery, and Hospital Anxiety and Depression Scale), the presence of cognitive and behavioural changes as well as anxiety and depression in 277 patients with ALS, 75 patients with PLS and 143 patients with PMA was evaluated retrospectively., Results: We found a high frequency of cognitive and behavioural abnormalities with similar profiles in all three groups. Subjects with behavioural variant FTD were identified in all groups., Conclusions: The percentage of patients with PLS and PMA with cognitive dysfunction was similar to patients with ALS, emphasising the importance for cognitive screening as part of routine clinical care in all three patient groups. With a similar cognitive profile, in line with genetic and clinical overlap between the MNDs, the view of PLS as an MND exclusively affecting upper motor neurons and PMA exclusively affecting lower motor neurons cannot be held. Therefore, our findings are in contrast to the recently revised El Escorial criteria of 2015, where PLS and PMA are described as restricted phenotypes. Our study favours a view of PLS and PMA as multidomain diseases similar to ALS., Competing Interests: Competing interests: MAvE serves on the UK Motor Neurone Disease Association Biomedical Research Advisory Panel, has consulted for Biogen and has received travel grants from Shire (formerly Baxalta). LHvdB reports grants from Netherlands ALS Foundation, grants from Prinses Beatrix Spierfonds, grants from Netherlands Organization for Health Research and Development (Vici scheme), and grants from European Community’s Health Seventh Framework Programme (FP7/2007-2013) (grant agreement no 259867), during the conduct of the study; and personal fees from Baxter for scientific advisory board and travel grant, and personal fees from Scientific Advisory Board, Biogen Idec, outside the submitted work. The other authors declare that they have no conflict of interest., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Weakness caused by motor neuron degeneration in amyotrophic lateral sclerosis (ALS) may result in avoidance of physical activity, resulting in deconditioning and reduced health-related quality of life (HRQoL)., Objective: To study the effectiveness of aerobic exercise therapy (AET) on disease-specific and generic HRQoL in ambulatory patients with ALS., Methods: We conducted a multicenter, assessor-blinded, randomized controlled trial. Using a biphasic randomization model, ambulatory ALS patients were assigned (1:1) to AET+usual care (UC), or UC. AET consisted of a 16-week aerobic cycling exercise program. Primary outcome measures were the 40-item ALS assessment questionnaire (ALSAQ-40), and the mental component summary (MCS) and physical component summary (PCS) scores of the short-form survey (SF-36), using linear mixed effects models. Per-protocol (PP) analysis was performed for those patients who attended ≥75% of the training sessions; controls were matched (1:1) by propensity score matching., Results: Of 325 screened patients, 57 were randomized: 27 to AET+UC and 30 to UC. No significant mean slope differences between groups were observed for ALSAQ-40 (-1.07; 95% confidence interval [CI] -2.6 to 0.5, P=0.172) nor for SF-36 MCS (0.24; -0.7 to 1.1, P=0.576) or PCS (-0.51; -1.4 to 0.38, P=0.263). There were no adverse events related to the AET. PP-analyses showed significantly less deterioration in ALSAQ-40 (-1.88, -3.8 to 0.0, P=0.046) in AET+UC compared to UC., Conclusions: AET+UC was not superior to UC alone in preserving HRQoL in ambulatory ALS patient. However, the study was unfortunately underpowered, because only 10 patients completed the protocol. AET+UC may preserve disease-specific HRQoL in slow progressors., Clinical Trial Registration Number: Netherlands National Trial Register (NTR): 1616.

Background: Self-management support is widely accepted for the management of chronic conditions. Self-management often requires behaviour change in patients, in which primary care nurses play a pivotal role. To support patients in changing their behaviour, the structured behaviour change Activate intervention was developed. This intervention aims to enhance physical activity in patients at risk for cardiovascular disease in primary care as well as to enhance nurses' role in supporting these patients. This study aimed to evaluate nurses' perceptions towards the delivery and feasibility of the Activate intervention., Methods: A qualitative study nested within a cluster-randomised controlled trial using semistructured interviews was conducted and thematically analysed. Fourteen nurses who delivered the Activate intervention participated., Results: Three key themes emerged concerning nurses' perceptions of delivering the intervention: nurses' engagement towards delivering the intervention; acquiring knowledge and skills; and dealing with adherence to the consultation structure. Three key themes were identified concerning the feasibility of the intervention: expectations towards the use of the intervention in routine practice; perceptions towards the feasibility of the training programme; and enabling personal development., Conclusions: Delivering a behaviour change intervention is challenged by the complexity of changing nurses' consultation style, including acquiring corresponding knowledge and skills. The findings have increased the understanding of the effectiveness of the Activate trial and will guide the development and evaluation of future behaviour change interventions delivered by nurses in primary care., Trial Registration: ClinicalTrials.gov NCT02725203 .

Background: Informal caregivers of patients with Amyotrophic Lateral Sclerosis (ALS) or Progressive Muscular Atrophy (PMA) face stressful demands due to severe impairments and prospect of early death of the patients they care for. Caregivers often experience feelings of psychological distress and caregiver burden, but supportive interventions are lacking. The objective of this study is to investigate the effectiveness of a psychosocial support program aimed at enhancing feelings of control over caregiving tasks and reducing psychological distress. This support program is based on an existing program for adult partners of people with cancer and is adapted to meet the needs of ALS caregivers., Methods: This study is a randomized controlled trial using a wait-list control design. One hundred and forty caregiver-patient dyads, recruited from a nationwide database and through the website of the Dutch ALS Center, will be either randomized to a support program or a wait-list control group. The blended intervention is based on Acceptance and Commitment Therapy and consists of 1 face-to-face contact, 6 online guided modules and 1 telephone contact. The intervention can be worked through in 8 weeks. The effectiveness and the participants' satisfaction with the intervention will be evaluated using a mixed method design. Caregivers and patients will be asked to fill in questionnaires on 4 occasions during the study: baseline, 3 months, 6 months and 9 months. The main study outcome is the psychological distress of the caregiver assessed with the Hospital Anxiety and Depression Scale. Secondary outcomes are caregiver burden, caregiver quality of life, quality of life of the patient and psychological distress of the patient. Group differences in primary and secondary outcomes at 6 months will be compared with linear mixed model analysis. In a subgroup of caregivers we will explore experiences with the support program through semi-structured interviews. Usage of the online modules will be logged., Discussion: The study will provide insights into the effectiveness of a blended psychosocial support program on psychological distress of caregivers of patients with ALS or PMA, as well as into indirect relations with patients' wellbeing., Trial Registration: Netherlands Trial Registry NTR5734 , registered 28 March 2016.

Objectives: To examine how and to what extent self-management support, including behaviour change support, is provided by primary care nurses in routine consultations with chronically ill patients., Design: Observational study design., Methods: Routine consultations of primary care nurses in the Netherlands with chronically ill patients were audio-taped and analysed. The analysis identified health topics addressed according to health care standards, self-management topics addressed using a validated set of topics, and behaviour change techniques (BCTs) using the Behaviour Change Techniques Taxonomy v1., Results: Seventy-eight routine consultations of 17 primary care nurses with chronically ill patients were included in the analysis. Nurses addressed both health topics and self-management topics in brief, fragmented, and often inconsistent manners. Dietary intake and physical activity were the most frequently addressed topics. Nurses applied 21 BCTs to target behaviour change, but the use of these techniques was mainly inconsistent and implicit. The most consistently used BCTs were review behaviour goal(s) (56.4%) and feedback on behaviour (51.3%)., Conclusions: Nurses addressed both health topics and self-management topics in their routine consultations. The duration, frequency, and number of addressed topics differed throughout the consultations. Nurses tended to prioritize the monitoring and optimization of patients' medical treatment and provided limited self-management support. Nurses seldom deepened their focus on behaviour change and infrequently used effective techniques to support this change. Adoption of self-management in primary care, including behaviour change, might be enhanced if nurses consistently and explicitly use effective BCTs in their consultations. Statement of contribution What is already known on this subject? Primary care nurses play a pivotal role in self-management support for patients with a chronic condition. Adequate self-management support requires nurses to activate patients and enhance behaviour change. Little is known regarding to what extent nurses provide self-management support in routine primary care. What does this study add? Self-management support is brief, fragmented, and inconsistently provided by nurses. Nurses tend to prioritize medical treatment optimization and seldom focus on behavioural change. Nurses' use of effective behaviour change techniques is low and should be enhanced., (© 2017 The British Psychological Society.)

Background: Informal caregivers of patients with amyotrophic lateral sclerosis experience increased levels of caregiver burden as the disease progresses. Insight in the factors related to caregiver burden is needed in order to develop supportive interventions., Aim: To evaluate the evidence on patient and caregiver factors associated with caregiver burden in amyotrophic lateral sclerosis informal caregivers., Design: A systematic review., Data Sources: Four electronic databases were searched up to 2017. Studies that investigated quantitative relations between patient or caregiver factors and caregiver burden were included. The overall quality of evidence for factors was assessed using the Grading of Recommendations Assessment, Development and Evaluation approach., Results: A total of 25 articles were included. High quality of evidence was found for the relation between caregiver burden and the factor "behavioral impairments." Moderate quality of evidence was found for the relations between caregiver burden and the factors "feelings of depression" of the caregiver and "physical functioning" of the patient. The remaining rated caregiver factors-"feelings of anxiety," "distress," "social support," "family functioning," and "age"-and patient factors-"bulbar function," "motor function," "respiratory function," "disease duration," "disinhibition," "executive functioning," "cognitive functioning," "feelings of depression," and "age"-showed low to very low quality of evidence for their association with caregiver burden., Conclusion: Higher caregiver burden is associated with greater behavioral and physical impairment of the patient and with more depressive feelings of the caregiver. This knowledge enables the identification of caregivers at risk for caregiver burden and guides the development of interventions to diminish caregiver burden.

Objective: Primary lateral sclerosis (PLS) is a rare form of motor neuron disease characterised by UMN degeneration leading to slowly progressive spasticity. Whether it is a separate disease or a subtype of ALS has been debated. In ALS comorbid frontotemporal dementia (FTD) is frequently seen (±15%). However, cognitive and behavioural changes are generally not considered to be a part of PLS., Methods: To report the clinical findings and frequency of PLS patients that developed FTD in a referral-based cohort and provide an overview of the literature., Results: In our cohort six out of 181 (3.3%) PLS patients developed FTD. In the literature a few cases of PLS with FTD have been reported and only a limited number of small studies have investigated cognition in PLS. However, when these studies are summarised a pattern emerges with FTD diagnoses in ±2% and frontotemporal impairment in 22% of patients., Conclusions: These findings suggest that PLS is part of the FTD-MND continuum and would favour viewing it as a subtype of ALS. It is, however, not a restricted (isolated UMN involvement) phenotype.

Introduction: The aim of this study was to assess the prevalence of participation restrictions in ambulatory patients with amyotrophic lateral sclerosis (ALS) and to identify physical and psychological contributory factors., Methods: In this cross-sectional study, self-reported participation restrictions of 72 ambulatory ALS patients were assessed using the social health status dimension (SIPSOC) of the Sickness Impact Profile (SIP-68). Associations between SIPSOC and physical functioning, psychological factors, and demographic factors were analyzed using hierarchical regression analyses., Results: Ninety-two percent of the patients reported participation restrictions; 54.9% could be explained by physical functioning; psychological factors accounted for 8.1% of the variance. Lung capacity, functional mobility, fatigue, and helplessness were independently associated with participation restrictions., Conclusions: Ambulatory ALS patients have participation restrictions, which may be influenced if early ALS care is directed toward lung capacity, functional mobility, fatigue, and feelings of helplessness. Muscle Nerve 56: 912-918, 2017., (© 2017 Wiley Periodicals, Inc.)

Purpose: This study assessed the validity of the shuttle walk test (SWT) to evaluate walking ability in patients with polyneuropathy., Methods: Forty-one patients with chronic idiopathic axonal polyneuropathy (CIAP) and 49 patients with multifocal motor neuropathy (MMN) performed both the 10-meter walk test (10MWT) and the SWT. Face validity was assessed by evaluating whether patients considered both tests to reflect their walking ability (Likert scale: 1 = not at all, 10 = very well). Concurrent validity was determined by Spearman rank-correlation analyses performed on the outcomes of both tests., Results: Mean (SD) scores for how well the 10MWT and SWT reflected daily walking ability were 6.8 (1.3) and 7.4 (1.6) (p = 0.117) in patients with CIAP and 6.9 (1.2) and 7.9 (1.0) (p = 0.001) in patients with MMN, respectively. Correlation scores between both tests ranged from -0.70 to -0.82, except for 18 patients with MMN with a "normal" walking speed at the 10MWT (-0.21)., Conclusion: The SWT seems a valid instrument for assessing walking ability in individuals with CIAP and MMN. Moreover, the SWT seems to be useful for investigating the symptoms elicited by walking long distances and may be more sensitive to changes when compared to the 10MWT. Implications for Rehabilitation Patients with polyneuropathy mainly experience problems when walking long distances. The 10-meter walk test does not possess sufficient psychometrics to diagnose walking abilities in these circumstances. The shuttle walk test is a valid instrument for assessing walking ability in individuals with polyneuropathy and might be the preferred instrument of choice when compared to the 10-meter walk test.

The amyotrophic lateral sclerosis functional rating scale-revised (ALSFRS-R) is a widely used primary outcome measure in amyotrophic lateral sclerosis (ALS) clinical practice and clinical trials. ALSFRS-R items cannot, however, validly be summed to obtain a total score, but constitute domain scores reflecting a profile of disease severity. Currently, there are different measurement models for estimating domain scores. The objective of the present study is, therefore, to derive the measurement model that best fits the data for a valid and uniform estimation of ALSFRS-R domain scores. Data from 1556 patients with ALS were obtained from a population-based register in The Netherlands. A random split of the sample provided a calibration and validation set. Measurement models of the ALSFRS-R were investigated using both exploratory factor analyses and confirmatory factor analyses. The measurement model with a four-factor structure (i.e., bulbar, fine motor, gross motor, and respiratory function), with correlated factors and cross-loading items on dressing and hygiene and turning in bed and adjusting bed clothes on both motor function scales, provided the best fit to the data in both sets. Correlation between factors ranged from weak to modest, confirming that the ALSFRS-R constitutes a profile of four clinically relevant domain scores rather than a total score that expresses disease severity. The internal consistency of the four domain scores was satisfactory. Our revision of the measurement model may allow for a more adequate estimation of disease severity and disease progression in epidemiological studies and clinical trials.

Background: Self-management interventions are considered effective in patients with chronic disease, but trials have shown inconsistent results, and it is unknown which patients benefit most. Adequate self-management requires behaviour change in both patients and health care providers. Therefore, the Activate intervention was developed with a focus on behaviour change in both patients and nurses. The intervention aims for change in a single self-management behaviour, namely physical activity, in primary care patients at risk for cardiovascular disease. The aim of this study is to evaluate the effectiveness of the Activate intervention., Methods/design: A two-arm cluster randomised controlled trial will be conducted to compare the Activate intervention with care as usual at 31 general practices in the Netherlands. Approximately 279 patients at risk for cardiovascular disease will participate. The Activate intervention is developed using the Behaviour Change Wheel and consists of 4 nurse-led consultations in a 3-month period, integrating 17 behaviour change techniques. The Behaviour Change Wheel was also applied to analyse what behaviour change is needed in nurses to deliver the intervention adequately. This resulted in 1-day training and coaching sessions (including 21 behaviour change techniques). The primary outcome is physical activity, measured as the number of minutes of moderate to vigorous physical activity using an accelerometer. Potential effect modifiers are age, body mass index, level of education, social support, depression, patient-provider relationship and baseline number of minutes of physical activity. Data will be collected at baseline and at 3 months and 6 months of follow-up. A process evaluation will be conducted to evaluate the training of nurses, treatment fidelity, and to identify barriers to and facilitators of implementation as well as to assess participants' satisfaction., Discussion: To increase physical activity in patients and to support nurses in delivering the intervention, behaviour change techniques are applied to change behaviours of the patients and nurses. Evaluation of the effectiveness of the intervention, exploration of which patients benefit most, and evaluation of our theory-based training for primary care nurses will enhance understanding of what works and for whom, which is essential for further implementation of self-management in clinical practice., Trial Registration: ClinicalTrials.gov identifier: NCT02725203 . Registered on 25 March 2016.

Background: Ambulatory patients with Amyotrophic Lateral Sclerosis (ALS) show a decreased aerobic capacity which may hamper the ability to perform activities of daily living. A standardized measure, however, for assessing aerobic capacity in patients with ALS during the disease course, is lacking., Objective: To examine the feasibility of the Åstrand-Ryhming (ÅR) test protocol longitudinally in ambulatory patients with amyotrophic lateral sclerosis (ALS)., Methods: Seven ambulatory male patients with spinal ALS onset were assessed at baseline and at 4, 7 and 10 months' follow-up. Feasibility of the ÅR test protocol was analysed using percentage of: a) completed ÅR tests; b) achieved steady states; and c) predefined heart rates., Results: Test completion decreased from 7/7 at baseline to 10/21 at follow-up due to ALS-related symptoms as fatigue, muscle weakness and cramps. Steady states and predefined heart rates were achieved in 12/17 and 17/17 of the completed tests, respectively. Overall, the feasibility of the ÅR test protocol declines from 5/7 at baseline to 7/21 at follow-up., Conclusions: The results suggest that changes in aerobic capacity in ambulatory patients with ALS could not be successfully monitored due to a diminished feasibility of the ÅR test protocol.

Objective: To systematically identify and appraise evidence on associations between psychological factors (moods, beliefs, personality) and Health-related QoL (HRQoL) and/or global QoL in patients with Amyotrophic Lateral Sclerosis (ALS)., Methods: A systematic review was conducted in several online databases (PsycINFO, EMBASE, PubMed and CINAHL) up to October 2015. Articles were included if they reported associations between psychological factors (moods, beliefs and personality) and HRQoL and/or global QoL in an ALS population. The search was limited to empirical studies, published in English, which provided quantitative data. The methodological quality of the included articles was assessed., Results: In total, 22 studies were included. Mood was investigated in 14 studies, beliefs in 11 studies and personality in one study. Fifteen different psychological factors were extracted and assessed using 24 different measures. Twelve different QoL measures were used in the selected studies, subdivided into seven different HRQoL measures and five different global QoL measures. Higher levels of anxiety and depression appeared to be related to a poorer HRQoL, whereas a higher level of religiosity seemed to be associated with better global QoL. No conclusive associations were found for confusion-bewilderment (mood), spirituality, mindfulness, coping styles, hopelessness, perception of burden, cognitive appraisal (beliefs), neuroticism, extraversion, openness, agreeableness and conscientiousness (personality), due to insufficient or inconsistent evidence. Religiosity and spirituality appeared to become more positively associated over time., Conclusions: Our results suggest that higher levels of anxiety and depression are related to a poorer HRQoL, whereas higher levels of religiosity appeared to be related to better global QoL. Associations might change during the disease course. This review supports the importance of psychological factors with regard to ALS care. Further research is needed to supplement the available evidence and to investigate how psychological factors can be modified to improve QoL., Review Registration Number: PROSPERO 2015:CRD42015027303.

ALS is a disease characterized by the progressive loss of upper and lower motor neurons leading to weakness and spasticity. Diagnosis of ALS is based on exclusion. ALS and frontotemporal dementia (FTD) constitute the extremes of the spectrum of one disease. Many patients show signs of both ALS and FTD. ALS is a heterogeneous disease in which multiple genetic factors contribute. More than 20 genes are known to play a role in ALS pathogenesis. In approximately 5-10% of cases the disease is familial with autosomal dominant inheritance. There is no curative treatment for ALS. The treatment of ALS patients is symptomatic and is focused on achieving a high level of quality of life. New insights into the genetic fundamentals of ALS offer hope for new therapies. Gene-targeted treatment strategies using antisense oligonucleotides are a promising development.

Valid questionnaires for measuring functional limitations in patients with Whiplash Associated Disorders (WAD) are lacking, since existing measures are not suitable for addressing the specific limitations of these patients and because of cross contamination between theoretical constructs. The objective of this study was to evaluate dimensionality, test-retest reliability, measurement error, construct validity, and responsiveness of a new condition-specific questionnaire for WAD as well as to estimate the minimally important change score. Patients with WAD grade I or II were recruited from physical therapy practices and rehabilitation centers. Dimensionality was examined by internal consistency analysis (Cronbach's alpha) and factor analysis. Test-retest reliability was estimated by intraclass correlations and measurement error was calculated by the minimal detectable change (MDC) scores. Construct validity was investigated by testing predefined hypotheses on correlations of the WAL scores with generic health measures and by using the known group method. Responsiveness was expressed as the minimal clinically important change (MCIC) score. 73 patients (53 women) were included. Cronbach's alpha was high (0.95) and unidimensionality was plausible because factor analysis showed 40.3% variance explained by one dominant factor, which was more than 4.5 times larger than the second largest factor. Test-retest reliability was excellent (0.92, 95% CI 0.87-0.95). Construct validity was supported by 14 out of 15 confirmed hypotheses and the WAL showed statistically significant differences between known groups. MDC was 16 points while the MCIC was 18 points. In conclusion, the present study suggests that the WAL has adequate measurement properties, but additional research is needed., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Our objective was to compare the effects of cognitive behavioural therapy (CBT) and usual care (UC) on quality of life (QoL) in psychologically distressed patients with ALS and their caregivers. We conducted a multicentre randomized controlled trial (RCT). In 16 weeks, patient-carer pairs received five to 10 CBT sessions plus usual care (UC) or UC alone. Outcome measures were SF-36 Mental Component Summary (MCS), ALSAQ-40 Emotional Functioning (EF), Hospital Anxiety and Depression Scale (HADS) and Caregiver Strain Index (CSI). Assessments took place at baseline, four, seven and 10 months. The steering committee decided to stop the trial prematurely and analyse the data due to: 1) slow recruitment (15 patients over 42 months); and 2) the low demand for joint patient-carer CBT sessions. Caregivers, however, expressed an unanticipated demand for individual psychological support. Patients' ALSAQ-40-EF and caregivers' SF-36-MCS were significantly better in CBT than UC (p < 0.05). CSI was significantly lower in the CBT than the UC (p < 0.05). In conclusion, CBT might be beneficial to patients and caregivers. The stringent eligibility criteria limited participation rate and consequently the generalizability of results. Future studies should further examine the impact of CBT interventions for patients with ALS and their caregivers.

Objective: Mechanical neck pain is a common human phenomenon. In most cases, no patho-anatomical lesion can be identified with the limitations of current radiological imaging. Therefore it is preferable to assess patients with neck pain with a focus on functioning and health, within a biopsychosocial model. The Neck Bournemouth Questionnaire aims to assess biopsychosocial aspects of neck pain. However, the content validity of the Neck Bournemouth Questionnaire Dutch language version (NBQ-NL) in relation to the International Classification of Functioning, Disability and Health (ICF) is unknown. The purpose of the study was to examine the content validity of the NBQ-NL in relation to the health components of the ICF., Method: Content validity was assessed in relation to the ICF, by means of Discriminant Content Validity (DCV)., Results: The results indicate that the NBQ-NL measures four components of the five ICF (impairment, activity, participation and personal factors)., Conclusion: The NBQ is a multidimensional questionnaire, representing four of the five components of the ICF., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Purpose: The International Classification of Human Functioning Disability and Health (ICF) provides insight into functional health status in patients with whiplash-associated disorders (WAD). In the assessment of functional limitations in patients with WAD, there are several condition-specific questionnaires available. Estimation of the true relationship between the separate constructs of the ICF is only possible if the items of the salient questionnaires measure exactly the constructs of interest, while not simultaneously measuring other constructs of the model. This study aimed to develop a condition specific and clinically relevant and usable instrument for patients with WAD that measures activity limitations and participation restrictions, as defined by the ICF framework., Methods: Item generation consisted of (1) a semi-structured interview which was conducted among 69 WAD patients; (2) a Delphi study involving 13 health professionals experienced in the assessment of patients with WAD; (3) a literature search for items from self-assessment questionnaires for neck pain., Results: A 35-item condition-specific self-assessment questionnaire for patients with WAD was developed. This new questionnaire measures purely activity limitations and participation restrictions according to the ICF and is based on patients' opinions and expert opinions., Conclusion: The whiplash activity and participation list tends to measure clinically relevant activity limitations and participation restrictions in WAD patients.

Background: Amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative disorder affecting motor neurons in the spinal cord, brainstem and motor cortex, leading to muscle weakness. Muscle weakness may result in the avoidance of physical activity, which exacerbates disuse weakness and cardiovascular deconditioning. The impact of the grave prognosis may result in depressive symptoms and hopelessness. Since there is no cure for ALS, optimal treatment is based on symptom management and preservation of quality of life (QoL), provided in a multidisciplinary setting. Two distinctly different therapeutic interventions may be effective to improve or preserve daily functioning and QoL at the highest achievable level: aerobic exercise therapy (AET) to maintain or enhance functional capacity and cognitive behavioural therapy (CBT) to improve coping style and cognitions in patients with ALS. However, evidence to support either approach is still insufficient, and the underlying mechanisms of the approaches remain poorly understood. The primary aim of the FACTS-2-ALS trial is to study the effects of AET and CBT, in addition to usual care, compared to usual care alone, on functioning and QoL in patients with ALS., Methods/design: A multicentre, single-blinded, randomized controlled trial with a postponed information model will be conducted. A sample of 120 patients with ALS (1 month post diagnosis) will be recruited from 3 university hospitals and 1 rehabilitation centre. Patients will be randomized to one of three groups i.e. (1) AET + usual care, (2) CBT + usual care, (3) Usual care. AET consists of a 16-week aerobic exercise programme, on 3 days a week. CBT consists of individual psychological support of patients in 5 to 10 sessions over a 16-week period. QoL, functioning and secondary outcome measures will be assessed at baseline, immediately post intervention and at 3- and 6-months follow-up., Discussion: The FACTS-2-ALS study is the first theory-based randomized controlled trial to evaluate the effects, and the maintenance of effects, of AET and CBT on functioning and QoL in patients with ALS. The results of this study are expected to generate new evidence for the effect of multidisciplinary care of persons with ALS., Trial Registration: Dutch Trial Register NTR1616.