Amyotrophic lateral sclerosis-specific quality of life-short form (ALSSQOL-SF): A brief, reliable, and valid version of the ALSSQOL-R.

Introduction: The Amyotrophic Lateral Sclerosis (ALS)-Specific Quality of Life instrument and its revised version (ALSSQOL and ALSSQOL-R) have strong psychometric properties, and have demonstrated research and clinical utility. In this study we aimed to develop a short form (ALSSQOL-SF) suitable for limited clinic time and patient stamina.

Methods: The ALSSQOL-SF was created using Item Response Theory and confirmatory factor analysis on 389 patients.

Fixed dynamometry is more sensitive than vital capacity or ALS rating scale.

Introduction: Improved outcome measures are essential to efficiently screen the growing number of potential amyotrophic lateral sclerosis (ALS) therapies.

Methods: This longitudinal study of 100 (70 male) participants with ALS compared Accurate Test of Limb Isometric Strength (ATLIS), using a fixed, wireless load cell, with ALS Functional Rating Scale-Revised (ALSFRS-R) and vital capacity (VC).

Results: Participants enrolled at 5 U.

National Study of Muscle Cramps in ALS in the USA.

The objective of this study was to describe muscle cramps in an US sample of amyotrophic lateral sclerosis (ALS) patients. Utilizing an anonymous web based questionnaire we queried ALS patients regarding the severity, frequency, time-course, treatment of muscle cramps and their relationship to pain. The survey had 282 respondents with 92% reporting that they had cramps.

The role of mental health and self-efficacy in the pain experience of patients with amyotrophic lateral sclerosis.

Complex interactions between pain, depression, and anxiety impact quality of life in patients with ALS. Psychological approaches to pain control may be useful. This study explored the role of self-efficacy in mitigating pain.

Patient-reported problematic symptoms in an ALS treatment trial.

This study was undertaken to determine which symptoms are perceived to be most problematic for patients with ALS and how their severity changes over time. A retrospective study was performed of data from a randomized, double-blind, placebo-controlled trial of ceftriaxone in ALS. Participants completed the ALS Specific Quality of Life Instrument (ALSSQoL) at baseline and at intervals up to 96 weeks.

Pain in amyotrophic lateral sclerosis: Patient and physician perspectives and practices.

Our objective was to better understand the experience and impact of pain on ALS patients in the U.S., and to survey ALS physicians on their pain assessment and management practices.

Compliance with recommendations made in a multidisciplinary ALS clinic.

Multidisciplinary ALS clinics provide recommendations at each visit, but these do little good unless recalled and followed. This study was conducted to determine recall of, and compliance with, these recommendations, and to study factors affecting compliance. Patients were contacted by telephone six weeks after their ALS clinic visit and asked about recommendations made by the multidisciplinary team.

A Qualitative Study of Multidisciplinary ALS Clinic Use in the United States.

Objectives: The multidisciplinary clinic (MDC) has become the standard of care for individuals with amyotrophic lateral sclerosis (ALS) in the United States, yet many patients choose not to receive care at MDCs. We undertook a qualitative study of individuals with ALS to explore patients' perceptions of this form of service delivery.

Methods: Participants completed an online survey that posed open-ended questions about their attitudes and behaviors surrounding MCDs.

Multidisciplinary ALS clinics in the USA: A comparison of those who attend and those who do not.

Optimization of quality of life (QoL) is perceived by many as the primary goal for patients with amyotrophic lateral sclerosis (ALS), often via multidisciplinary clinics (MDCs). The aim of this study was to examine the differences in QoL, physical function, and social problem-solving skills for individuals with ALS attending MDCs compared to non-attenders. An online survey was completed by 295 people with ALS in the United States.

Serum ferritin is elevated in amyotrophic lateral sclerosis patients.

Our objective was to measure serum ferritin levels, which reflect iron metabolism, in ALS patients versus healthy and disease controls, and determine whether serum ferritin levels correlate with survival. We retrospectively analyzed data from 138 ALS patients, 152 healthy controls, and 82 disease controls. Gender, age, site of onset, and dates of symptom onset and death were recorded.

Acceptance of brain-computer interfaces in amyotrophic lateral sclerosis.

Brain-computer interfaces (BCI) have the potential to permit patients with amyotrophic lateral sclerosis (ALS) to communicate even when locked in. Although as many as half of patients with ALS develop cognitive or behavioral dysfunction, the impact of these factors on acceptance of and ability to use a BCI has not been studied. We surveyed patients with ALS and their caregivers about BCIs used as assistive communication tools.

Biomarker-based predictive models for prognosis in amyotrophic lateral sclerosis.

Importance: Although median survival in amyotrophic lateral sclerosis (ALS) is 2 to 4 years, survival ranges from months to decades, creating prognostic uncertainty. Strategies to predict prognosis would benefit clinical management and outcomes assessments of clinical trials.

Objective: To identify biomarkers in plasma and cerebrospinal fluid (CSF) of patients with ALS that can predict prognosis.

H63D HFE polymorphisms are associated with increased disease duration and decreased muscle superoxide dismutase-1 expression in amyotrophic lateral sclerosis patients.

Introduction: H63D HFE polymorphisms increase the risk of neurodegenerative disorders and, specifically, may increase amyotrophic lateral sclerosis (ALS) risk. Investigating the physiological alterations induced by H63D polymorphisms in ALS patients may elucidate mechanisms by which this genotype alters disease.

Methods: Clinical measures and muscle biopsies were available from patients previously diagnosed with ALS who underwent HFE genotyping.

Non-invasive ventilation and gastrostomy may not impact overall quality of life in patients with ALS.

Non-invasive positive pressure ventilation (NIPPV) may improve health-related quality of life (HRQoL) in patients with ALS. The effect of percutaneous endoscopic gastrostomy (PEG) on HRQoL is not known. Instruments measuring QoL more broadly have not been used to assess effects of these interventions.

Psychological health in patients with ALS is maintained as physical function declines.

Although quality of life (QoL) in patients with ALS has been shown to be independent of physical function and to be maintained over time, the status of psychological health over the disease course has not been studied using an ALS-specific instrument. It is also uncertain how three common interventions - antidepressants, percutaneous endoscopic gastrostomy (PEG), and non-invasive ventilatory support (NIPPV) - influence psychological health. We performed a retrospective review of the Negative Emotion subscale (NES) score, a measure of psychological health within the ALS-Specific QoL Instrument.

Plasma biomarkers associated with ALS and their relationship to iron homeostasis.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease with complicated pathogenesis with variable presentation and disease progression. There is a critical need for a panel of biomarkers to provide clinicians and researchers with additional information. In this study, multiplex immunoassays were used to screen a number of cytokines, growth factors, and iron-related proteins.