Introduction: Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disease with no known cure, limited treatment options with minimal benefits, and significant unmet need for disease modifying therapies.
Aims: This study investigated memantine's impact on ALS progression, with an additional focus on the effects of memantine on cognitive and behavioral changes associated with the disease.
Methods: A randomized, double-blind, placebo-controlled clinical trial was conducted from December 2018 to September 2020.
Amyotroph Lateral Scler Frontotemporal Degener
September 2024
Objective: Health coaching may supplement the multidisciplinary ALS clinic model to facilitate patient-centered health behavior change. The aim of this study was to determine the effects of nurse health coaching (NHC) on the quality of life and self-efficacy of individuals living with ALS.
Methods: Twenty-nine participants were randomized at 1:1 to the standard of care and coaching arms.
Background: Self-perceived quality of life (QoL) is important in amyotrophic lateral sclerosis (ALS). Although caregiver burden and strain have been related to cognitive and behavioural impairment, there has been no comprehensive research looking at these impairments and how they may influence self-perceived QoL subdomains.
Aims: To explore how cognitive and behavioural impairment are related to different areas of self-perceived QoL using disease-specific measures.
Background: Chiari I malformation (CMI) is characterized by inferior descent of the cerebellar tonsils through the foramen magnum and is associated with headache and neck pain. Many morphometric research efforts have aimed to describe CMI anatomy in the midsagittal plane using classical measurement techniques such as linear dimensions and angles. These methods are less frequently applied to parasagittal features and may fall short in quantifying more intricate anatomy with fewer distinct homologous landmarks.
View Article and Find Full Text PDFPrior research has produced mixed findings regarding whether women feel more attractive during the fertile phase of the menstrual cycle. Here, we analyzed cycle phase and hormonal predictors of women's self-perceived attractiveness (SPA) assessed within a daily diary study. Forty-three women indicated their SPA, sexual desire, and interest in their own partners or other potential mates each day across 1-2 menstrual cycles; saliva samples collected on corresponding days were assayed for estradiol, progesterone, and testosterone; and photos of the women taken at weekly intervals were rated for attractiveness.
View Article and Find Full Text PDFIntroduction/aims: Sodium phenylbutyrate-taurursodiol (PB-TURSO) was recently approved for treating amyotrophic lateral sclerosis (ALS). Third-party payors' coverage policies are evolving, and adverse events are just being fully assessed. The goals of this study were to evaluate patients' experiences in obtaining and continuing PB-TURSO and assess adverse events and medication adherence.
View Article and Find Full Text PDFPurpose: The current study compared temporal and spectral acoustic contrast between vowel segments produced by speakers with dysarthria across three speech tasks-interactive, solo habitual, and solo clear.
Method: Nine speakers with dysarthria secondary to amyotrophic lateral sclerosis participated in the study. Each speaker was paired with a typical interlocutor over videoconferencing software.
The traditional ALS multidisciplinary clinical practice of quarterly respiratory assessment may leave some individuals in danger of developing untreated respiratory insufficiency between visits or beginning non-invasive ventilation (NIV) later than would be optimal. Remote, or home-based, pulmonary function testing (rPFT) allows patients with ALS to perform regular respiratory testing at more frequent intervals in the home. The aim of this study was to determine the clinical benefit of weekly rPFT compared to standard, quarterly in-clinic respiratory assessments: the number of individuals with earlier identification of NIV need, the magnitude of this advance notice, and the individual factors predicting benefit.
View Article and Find Full Text PDFBackground: Non-pharmaceutical interventions (NPIs) were crucial in the response to the COVID-19 pandemic, although uncertainties about their effectiveness remain. This work aimed to better understand the evidence generated during the pandemic on the effectiveness of NPIs implemented in the UK.
Methods: We conducted a rapid mapping review (search date: 1 March 2023) to identify primary studies reporting on the effectiveness of NPIs to reduce COVID-19 transmission.
Purpose: In this study, we examined the utility of vowel intelligibility testing for assessing the impact of dysarthria on speech characteristics in people with amyotrophic lateral sclerosis (ALS). We tested the sensitivity and specificity of overall vowel identification, as well as that of vowel-specific identification, to dysarthria presence and severity. We additionally examined the relationship between vowel intelligibility and sentence intelligibility.
View Article and Find Full Text PDFPurpose: Motor neuron disease (MND) is a neurodegenerative disease, progressively impacting function and self-perceived quality of life (QoL). Up to 50% of people with MND can present with cognitive and behavioural impairment, with an associated increase in caregiver burden or strain. However, there has been no systematic exploration of the relationship between QoL and cognitive or behavioural impairment in MND.
View Article and Find Full Text PDFAmyotroph Lateral Scler Frontotemporal Degener
May 2024
Objective: To predict ALS progression with varying observation and prediction window lengths, using machine learning (ML).
Methods: We used demographic, clinical, and laboratory parameters from 5030 patients in the Pooled Resource Open-Access ALS Clinical Trials (PRO-ACT) database to model ALS disease progression as fast (at least 1.5 points decline in ALS Functional Rating Scale-Revised (ALSFRS-R) per month) or non-fast, using Extreme Gradient Boosting (XGBoost) and Bayesian Long Short Term Memory (BLSTM).
Introduction/aims: 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.
View Article and Find Full Text PDFAmyotroph Lateral Scler Frontotemporal Degener
February 2024
Background, Objectives: Decrease in the revised ALS Functional Rating Scale (ALSFRS-R) score is currently the most widely used measure of disease progression. However, it does not sufficiently encompass the heterogeneity of ALS. We describe a measure of variability in ALSFRS-R scores and demonstrate its utility in disease characterization.
View Article and Find Full Text PDFAmyotroph Lateral Scler Frontotemporal Degener
August 2023
Introduction: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that alters gait and increases the risk of falls. The current model of care involves in-person multidisciplinary clinic visits to, in part, assess alterations in gait, evaluate safety, and make recommendations for management. Clinic visits, however, are relatively infrequent, and multidisciplinary evaluations can be physically demanding for patients.
View Article and Find Full Text PDFObjectives: The goals of this study were to assess the feasibility of maintaining multidisciplinary remote care, patient preferences, and outcomes of this transition because of COVID-19.
Methods: From March 18, 2020 to June 3, 2020, 127 patients with amyotrophic lateral sclerosis (ALS) who were scheduled to be seen in our ALS clinic were contacted and scheduled according their preference for a telemedicine visit, telephone visit, or postponement until the next available in-person visit. Age, time from disease onset, ALS Functional Rating Scale-Revised, patient choices, and outcomes were recorded.
Introduction: Sleep valuation is the relative worth individuals place on sleep. Our prior study using a Sleep Valuation Item Bank (SVIB) showed that sleep valuation relates to age, gender, and health status. In this study, the psychometric properties of the SVIB and its latent factor structure were explored.
View Article and Find Full Text PDFBackgroud: The United States is facing a dramatic shortage of clinician-scientists within the domains of medicine and dentistry.
Point Of View: In this perspective article, we stressed the problem involving the continuous shortage of specialized professionals capable of addressing complex basic sciences questions, while maintaining clinical relevance. Here we present a different perspective regarding the early engagement of young students to clinical sciences by teaming up with high schools across the United States, and to energize the debate on our current shortage of clinician-scientists.
Peripheral nerve injury and the nerves' subsequent repair and regeneration continues to be marked clinically by poor functional recovery. The analysis of nerve morphology is an aspect which may provide an impact on successful clinical outcomes through better prediction of donor and recipient matching. In this study, we evaluated the morphological aspects of the human obturator nerve for a better understanding of its potential in nerve transplantation.
View Article and Find Full Text PDFAmyotroph Lateral Scler Frontotemporal Degener
August 2023