To (1) identify clinical situations that may contribute to the experience of moral distress (MD) among professionals working with motor neuron disease (MND), (2) measure the occurrence and intensity of MD, and (3) explore associations with professional quality of life, turnover intention, and associated risk and/or protective factors.

A cross-sectional online survey was distributed to healthcare professionals working in MND services across Europe. Data were analyzed using descriptive and inferential statistics.

In total, 230 responses from professionals across 17 European countries were analyzed from the international survey. And 67% of respondents indicated that MD resonated with their experience of working with MND. Those who considered leaving or changing their position due to the challenges associated with caring for this patient population were also more likely to report resonance with MD (χ2 = 7.772, p = 0.020). The intensity of MD was associated with reduced professional quality of life (burnout [β = 0.106, p < 0.05], and secondary traumatic stress [β = 2.881, p < 0.001]). A total of 24 clinical scenarios were identified as potential contributors to experiences of MD in this population. Across all professional groups, service-/organization-level factors were the most common and distressing barriers to providing effective MND care.

This study demonstrates that MD is experienced by healthcare professionals working with MND across Europe. MD was associated with reduced professional quality of life and increased intentions to leave or change positions, underscoring its potential implications for workforce retention and sustainability. The findings show that system/organization, patient/condition and family-level causes are the primary drivers of MD in this population. Future research should focus on evaluating the effectiveness of interventions designed to address these key drivers and mitigate the impact of MD among healthcare professionals working with MND.

This study investigates differing aspects of wishes to hasten death (WTHD) distinguished by the extent to which WTHD were linked to patients’ agency: desire for hastened death (DHD), defined as general wishes for death to come sooner, and hastening death intentions (HDI), defined as thoughts about ending one’s life. In particular, this study aims to examine the differences between DHD and HDI in patients with amyotrophic lateral sclerosis (pALS) and identify predictive factors for both.

A cross-sectional nested study was conducted within a multi-center longitudinal study involving pALS from 5 European countries. Data collected included DHD (Schedule of Attitudes toward Hastened Death), HDI (“could you currently imagine ending your life?”), sociodemographic and clinical characteristics, psychological distress, quality of life, and social and spiritual-existential aspects.

In our sample of 121 pALS, 12.4% (15/121) expressed DHD, and 28.1% (34/121) expressed HDI. Of the 38 patients reporting any WTHD, only 11 experienced both DHD and HDI simultaneously. 23 patients reported HDI without DHD, while 4 patients expressed DHD without HDI. Multivariable logistic regression identified loneliness (OR = 1.33, 95% CI 1.03–1.71, p = 0.028) and reduced meaning in life (OR = 0.89, 95% CI 0.84–0.95, p < 0.001) as independent predictors of DHD. For HDI, independent predictors were female gender (OR = 3.31, 95% CI 1.37–7.98, p = 0.008) and lower spirituality (OR = 0.92, 95% CI 0.88–0.95, p < 0.001).

One in 3 pALS expressed WTHD. Our separate analysis of DHD and HDI supports the existence of distinct manifestations of WTHD and varying underlying factors. While DHD and HDI were associated with different predictors, our results point to the crucial role of spiritual-existential factors in the experience of WTHD, identifying these aspects as target points for intervention. This study highlights the importance of a nuanced understanding and communication regarding WTHD.

Clinically relevant anxiety can be detected in patients with amyotrophic lateral sclerosis (ALS), but its prevalence and determinants have not yet been fully assessed.

This study aimed at assessing the prevalence and clinical underpinnings of anxiety in ALS.

Non-demented ALS patients (N = 433) and healthy controls (N = 313) were administered the State- and Trait-Anxiety Inventory – Form Y (STAI-Y1 for state-anxiety and STAI-Y2 for trait-anxiety) and the Beck Depression Inventory (BDI). Patients were further assessed for cognition (Edinburgh Cognitive and Behavioural ALS Screen), behaviour (Frontal Behavioural Inventory) and motor status (disease duration, ALS Functional Rating Scale-Revised and progression rate). The prevalence of clinically significant state- and trait-anxiety were estimated by applying age-stratified cut-offs to STAI-Y1/-Y2 t-scores. Linear and logistic regressions were run to test the determinants of STAI-Y1/-Y2 scores.

STAI-Y1 and -Y2 scores above cut-off were detected in 18.2 and 13.9% of patients, respectively – with proportions being higher in cases versus controls (ps < 0.001). BDI, but neither cognitive/behavioural nor motor variables, was identified as a significant predictor of STAI-Y1/-Y2 scores (ps < 0.003). The cognitive–affective subscale of BDI was the sole predictor of scores above cut-off on both STAI-Y1 and STAI-Y2 (ps < 0.001).

Clinically significant levels of state- and trait-anxiety occur in ∼18 and ∼14% of non-demented ALS patients, respectively, mostly driven by cognitive and affective facets of depression, and are independent of motor and cognitive/behavioural features.

Narrative-based approaches have been utilized in medicine to better understand the illness experiences of individuals living with chronic conditions. In particular, people with amyotrophic lateral sclerosis (pALS) may benefit from use of narrative-based approaches, given the potential impact of progressive decline on identity of self. This review explores the use of narrative-based approaches in studies involving pALS to provide further insight to the experiences and psychosocial needs of this population.

A search was conducted utilizing EMBASE, CINAHL, PsycInfo, and Google Scholar with several terms related to amyotrophic lateral sclerosis (ALS) and narrative-based approaches. Studies were included if they were written in English, incorporated methods that promoted the production of narratives, and reported data that could be clearly isolated to pALS.

The search revealed a total of 154 articles for title and abstract screening. Fifty-two articles were selected for full-text review. Thirty-two articles met the criteria for data extraction. Four descriptive categories emerged upon examination of the narrative-based approaches implemented across the studies: psychosocial intervention, illness experience, intervention targeting specific needs, and secondary analysis of data. Some of the common themes identified across studies included: loss of physical and communicative function, adaptation to life changes, shifts in identity, and tension with the healthcare system.

Despite the communication challenges that often coincide with disease progression, narrative-based approaches can be utilized in pALS. These approaches should be implemented to gain insight on the disease experiences of pALS, providing opportunity for patient-centered interventions to address the psychosocial needs of this population.

Amyotrophic lateral sclerosis (ALS) is a rare, progressive, and fatal disease that impacts the lives of affected individuals and their caregivers. Informal caregivers play a crucial role in supporting people with ALS (pwALS), yet they face major challenges. This study aims to analyze caregiver burden and health status among informal caregivers of pwALS in Portugal.

A cross-sectional survey-based study was conducted with adult informal caregivers of pwALS in Portugal, recruited through the Portuguese ALS patient association and healthcare professionals. Data included sociodemographics, caregiving activities, caregiver health (SF-36), patient functional status (ALSFRS-R), and caregiver burden (ZBI).

The study included 113 caregivers. Most were female (61.9%) and the partner (65.5%) or offspring (23.9%) of the pwALS. A quarter of caregivers received no social benefits. Mean ZBI was 32 ± 14.8, with most reporting mild to moderate burden. On the SF-36, general health was 51.1 ± 19.8, with mental health (55 [40; 70]) and vitality (43.8 [31.3; 56.3]) particularly impaired. ZBI scores correlated positively with caregiving hours (r = 0.274, p = 0.003) and negatively with ALSFRS-R (r = −0.411, p < 0.001). High burden caregivers exhibited poorer sleep quality (p =  0.026).

Caregivers experienced mild to moderate burden, with impaired mental health and vitality, but preserved physical functioning. A higher burden was linked with lower quality of life, poorer sleep, and greater patient disability. These findings underline the need for targeted education and training to support caregivers of pwALS.

Early detection of respiratory decline is crucial in amyotrophic lateral sclerosis (ALS). We tested if nocturnal polysomnography (PSG) predicts dyspnea onset in mild ALS patients with preserved daytime function.

In this study, 41 mild ALS patients (ALS Functional Rating Scale-Revised [ALSFRS-R] ≥ 37, sitting forced vital capacity [FVC] ≥80% predicted, no dyspnea) and 41 matched controls underwent baseline assessment, including ALSFRS-R scoring, pulmonary function tests, and overnight PSG. ALS patients were followed for 12 months. Baseline apnea–hypopnea index (AHI) and oxygen saturation (mean SpO2, minimum SpO2) were analyzed as continuous predictors and using exploratory thresholds (AHI ≥ 5 events/h, min SpO2 ≤ 88%, mean SpO2 ≤ 95%) for dyspnea onset (Dyspnea-ALS-15 [DALS-15] > 0).

Compared to controls, ALS patients had significantly higher AHI (p = 0.004) and lower minimum SpO2 (p = 0.018). The ALSFRS-R orthopnea subscore showed a significant positive correlation with mean and minimum SpO2 (P < 0.05). Cox regression identified baseline AHI (HR 1.08 per event/h; 95% CI 1.01–1.15, p = 0.028) and minimum SpO2 (HR 0.94 per %; 95% CI 0.88–0.99, p = 0.033) as independent predictors of dyspnea onset within 12 months. Thresholds AHI ≥ 5 (HR 2.28, p = 0.031) and min SpO2 ≤ 88% (HR 2.42, p = 0.027) also predicted increased risk. Patients meeting ≥1 threshold (n = 25/37) showed trends toward greater FVC and ALSFRS-R decline.

In patients with mild ALS and normal daytime function, specific nocturnal PSG parameters (AHI, minimum SpO2) predicted the risk of dyspnea within 12 months. This longitudinal study provides novel evidence that PSG could identify early respiratory vulnerability in the incipient stage, earlier than conventional FVC-based monitoring, supporting its potential utility in refining early intervention strategies. Validation in larger cohorts is warranted.

Early diagnosis of amyotrophic lateral sclerosis (ALS) is essential for treatment initiation and symptom management, yet it remains challenging due to nonspecific symptoms and the lack of reliable diagnostic biomarkers. Although conventional MRI sequences such as T2* weighted and fluid-attenuated inversion recovery (FLAIR) have shown potential in identifying upper motor neuron abnormalities, their diagnostic utility in ALS is not well established. This study aimed to evaluate the sensitivity and specificity of brain T2* weighted and FLAIR MRI sequences in diagnosing ALS using prospectively collected data and to assess associations with disease severity.

Data were analyzed from 20 patients with ALS and 20 healthy controls enrolled at the Edmonton site of the Canadian ALS Neuroimaging Consortium 1 (CALSNIC-1) study. Single-slice 2D axial susceptibility-weighted echo planar imaging (SWEPI) and FLAIR images were independently rated by a blinded neurologist and radiologist for signs of corticospinal tract and motor cortex abnormalities. Sensitivity and specificity were calculated, and linear regression was used to examine associations with ALS Functional Rating Scale-Revised (ALSFRS-R) scores.

T2* weighted and FLAIR MRI sequences showed high specificity (0.95 and 0.85, respectively) but low sensitivity (both 0.25) for ALS diagnosis. No significant correlation was found between imaging abnormalities and ALSFRS-R scores. Inter-rater reliability was poor (κ = 0.25 for SWEPI; κ = 0.14 for FLAIR).

While T2* weighted and FLAIR MRI sequences may have some specificity for ALS, our study suggests they are not sufficiently sensitive to be used as reliable diagnostic tools for ALS.

Installation of a percutaneous gastrostomy tube is often needed for patients with amyotrophic lateral sclerosis (ALS) who develop severe dysphagia. However, there is uncertainty regarding the optimal timing for this procedure, especially with regard to the decline in respiratory function. Several guidelines suggest that gastrostomy should be placed before the forced vital capacity (FVC) drops below 50%, since the procedural risks are heightened. However, multiple studies argue that this procedure could be safe in patients with an FVC of less than 50%.

In this retrospective study, we reviewed the medical records of all patients with ALS who had a gastrostomy at our center between 2010 and 2023. Our primary objective was to identify the 30-day mortality rate and the incidence of complications after this procedure. Also, we investigated whether predictive factors of adverse outcomes could be identified, particularly to evaluate if there was an association with pulmonary function.

We included 54 patients. The 30-day mortality rate was 9.3%, and the incidence of major complications was 16.7%. There was no statistical difference in complications between percutaneous endoscopic and radiologically inserted gastrostomy procedures. Predictive factors for complications were pre-existing pulmonary disease, pre-procedural CO2 levels and, although not statistically significant, diabetes. There was no association between FVC and the occurrence of adverse outcomes, although only 70% of patients had a measure of pulmonary function.

In our study, there was no correlation between FVC and the occurrence of adverse events from the gastrostomy procedure. This suggests that the traditional cutoff of 50% FVC level should be re-examined and explored further in future studies.

Currently, there are no head-to-head studies to compare the efficacy of riluzole, edaravone, sodium phenylbutyrate and taurursodiol (SPT) and tofersen. This study aims to compare all possible interventions for amyotrophic lateral sclerosis (ALS) using network meta-analysis (NMA) methods.

A conventional meta-analysis was done if at least two studies with the same intervention, control and outcomes were present. Since all studies included were randomized clinical trials, a NMA comparing five interventions was done, especially when similarity and consistency were assured. Both riluzole and edaravone had three clinical trials included, while SPT and tofersen each had one.

A total of 1601 ALS patients were included in this review, 1185 in the intervention group and 416 in the control group. Compared to placebo, ALS patients taking riluzole had 36% higher probability of surviving (OR: 1.36, I2 = 4%, p = 0.03, FEML) while those in the edaravone group had 1.44 point lower ALSFRS-R score (SMD: 1.44, p = 0.19, I2 = 98%, REML) at study end. Comparing all interventions in terms of mortality, all no interventions were significantly different to placebo. Moreover, compared to one another, no statistically significant differences were noted.

Despite the benefit of riluzole in terms of survival in conventional meta-analysis, non-significant findings and the lack of comparison of ALSFRS-R to placebo, edaravone, SPT and tofersen in NMA may preclude any strong recommendation for its use. Moreover, the difference in outcome measures limits important comparison between interventions, and while global consistency in NMA was satisfied, the heterogeneity of patient population limits the interpretability of our results.

To explore the spiritual needs and resources of People with Amyotrophic Lateral Sclerosis (PALS) at different stages of its trajectory and to characterize the experiences of the current state of the disease.

A convergent mixed-methods study was conducted using qualitative and quantitative approaches. Participants were assessed using the clinical and sociodemographic data, ALSFRS-R (function assessment), and the GES Questionnaire to evaluate spiritual needs and resources. Data were collected through in-person or online interviews, transcribed and coded. The qualitative analysis was based on the content analysis method. Statistical analysis was performed using SPSS software. Both datasets were integrated during data analysis.

Twenty-four patients were interviewed, with a duration of the illness ranging from 1 year to 12 years. Participants were at different stages of functional dependence. Analyzing the open questions of the GES questionnaire, six categories were established related to the inner world of PALS: Concern, Nuisance, Help, Support, Safety, and Valorization. Contrary to what was hypothesized, no correlations were found between functionality and the spiritual dimensions. Spiritual needs and resources tend to vary with age, with younger ages presenting a more fragile spiritual dimension overall. Also, the intrapersonal and interpersonal dimension seems to play a central role in the lives of PALS. A negative correlation was identified between the feeling of connection to a supreme/transcendent reality and the level of educational qualifications.

Spirituality often provides crucial emotional support, meaning, and resilience during challenging times. Despite its importance, it is often overlooked in clinical settings. The study emphasizes the need for personalized, holistic care, which should include spiritual care support, regardless of the functional state, highlighting the importance of addressing both intrapersonal and interpersonal domains, resources and needs from early phases. Allowing to create a structured care plan that meets patients’ individual spiritual needs, that can contribute to a better QoL and reduce suffering.