ALS Health care provider wellness.

Background: Interest in health care provider (HCP) wellness and burnout is increasing; however, minimal literature explores HCP wellness in the context of Amyotrophic Lateral Sclerosis (ALS) care.

Objectives: We sought to determine rates of burnout and resiliency, as well as challenges and rewards in the provision of ALS care.

Methods: A survey link was sent to physicians at all Canadian ALS centers for distribution to ALS HCPs in their network.

Perceptions and Experiences of Nutrition Interventions in Individuals with Amyotrophic Lateral Sclerosis (ALS) and their Caregivers.

Nutrition concerns are prevalent in individuals with Amyotrophic Lateral Sclerosis (ALS). Despite the prevalence of nutrition concerns, few data are available on perceptions and experiences of nutrition interventions in individuals with ALS and their caregivers; this study aimed to collect this information. An online survey was developed and hosted on Survey Monkey.

Neuromuscular Complications of SARS-CoV-2 and Other Viral Infections.

In this article we review complications to the peripheral nervous system that occur as a consequence of viral infections, with a special focus on complications of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2). We discuss neuromuscular complications in three broad categories; the direct consequences of viral infection, autoimmune neuromuscular disorders provoked by viral infections, and chronic neurodegenerative conditions which have been associated with viral infections. We also include discussion of neuromuscular disorders that are treated by immunomodulatory therapies, and how this affects patient susceptibility in the current context of the coronavirus disease 2019 (COVID-19) pandemic.

A Canadian Adult Spinal Muscular Atrophy Outcome Measures Toolkit: Results of a National Consensus using a Modified Delphi Method.

Background: Spinal Muscular Atrophy (SMA) is a rare disease that affects 1 in 11 000 live births. Recent developments in SMA treatments have included new disease-modifying therapies that require high quality data to inform decisions around initiation and continuation of therapy. In Canada, there are no nationally agreed upon outcome measures (OM) used in adult SMA.

Photovoice as a Participatory Research Tool in Amyotrophic Lateral Sclerosis.

Background: Photovoice is a qualitative research tool increasingly utilized in the healthcare field to understand the illness experience from the patient and caregiver perspective. This is the first study to evaluate photovoice in the context of amyotrophic lateral sclerosis (ALS).

Objective: A patient and caregiver centered research tool was utilized to gain a greater understanding of challenges faced when living with ALS.

A National Spinal Muscular Atrophy Registry for Real-World Evidence.

Background: Spinal muscular atrophy (SMA) is a devastating rare disease that affects individuals regardless of ethnicity, gender, and age. The first-approved disease-modifying therapy for SMA, nusinursen, was approved by Health Canada, as well as by American and European regulatory agencies following positive clinical trial outcomes. The trials were conducted in a narrow pediatric population defined by age, severity, and genotype.

Expression of Human Endogenous Retrovirus-K in Spinal and Bulbar Muscular Atrophy.

Spinal and Bulbar Muscular Atrophy (SBMA) is caused by the extension of the polyglutamine tract within the androgen receptor (AR) gene, and results in a multisystem presentation, including the degeneration of lower motor neurons. The androgen receptor (AR) is known to modulate the expression of endogenous retrovirus-K (ERVK), a pathogenic viral genomic symbiont. Since ERVK is associated with motor neuron disease, such as Amyotrophic Lateral Sclerosis (ALS), we sought to determine if patients with SBMA exhibit evidence of ERVK reactivation.

Provincial Differences in the Diagnosis and Care of Amyotrophic Lateral Sclerosis.

Background: Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron disease resulting in muscle weakness, dysarthria and dysphagia, and ultimately respiratory failure leading to death. Half of the ALS patients survive less than 3 years, and 80% of the patients survive less than 5 years. Riluzole is the only approved medication in Canada with randomized controlled clinical trial evidence to slow the progression of ALS, albeit only to a modest degree.

Patient perspectives on transitioning to amyotrophic lateral sclerosis multidisciplinary clinics.

Purpose: Multidisciplinary clinics (MDC) have become the standard of care for management of amyotrophic lateral sclerosis (ALS). No studies however, have captured patients' perspectives during a transition to ALS MDCs. Recently, an ALS MDC emerged from a single-physician clinic in Saskatoon, Canada, providing patients with a unique exposure to two different models of care.

Hospital utilization for patients with amyotrophic lateral sclerosis in saskatoon, Canada.

Objective: This retrospective study reviewed hospital and intensive care unit (ICU) admissions for patients with amyotrophic lateral sclerosis (ALS) in Saskatoon, Canada, between 2005 and 2017. The purpose was to understand hospital utilization and admission patterns for patients with ALS in the absence of coordinated multidisciplinary care.

Methods: Hospital/ICU admissions were detected at two hospitals in Saskatoon using the International Classification of Diseases (ICD-10) coding for ALS.

Prehospital Management of Acute Stroke in Rural versus Urban Responders.

Objective: Stroke guideline compliance of rural Canadian prehospital emergency medical services (EMS) care in acute stroke is unknown. In this quality assurance study, we sought to compare rural and urban care by prehospital EMS evaluation/management indicators from patients assessed at an urban Canadian stroke center.

Materials And Methods: One hundred adult patients were randomly selected from the stroke registry.

Breaking bad news in amyotrophic lateral sclerosis: the need for medical education.

The manner in which physicians deliver difficult diagnoses is an area of discontent for patients with amyotrophic lateral sclerosis (ALS). The American Academy of Neurology's Practice Parameter for care of the ALS Patient recommended teaching and evaluating strategies for disclosing the diagnosis (10). Our objective was to examine residents' ability in and perceptions of communicating the diagnosis of ALS.

Roaring in the ears: patulous Eustachian tube in bulbar amyotrophic lateral sclerosis.

We report a 64-year-old female with predominantly bulbar amyotrophic lateral sclerosis who experienced 'roaring' in her ears. She was diagnosed with patulous Eustachian tube, which is presumed to occur secondary to bulbar weakness. The roaring adversely affected the management of her ALS by impairing her ability to tolerate BiPaP.

A clinical tool for office assessment of lumbar spine stabilization endurance: prone and supine bridge maneuvers.

Objective: : To assess the validity and reliability of an office-based surrogate measure of lumbar spine-stabilization endurance capability; to establish norms and reliability in an asymptomatic group; and to compare their measures with those from a group of chronic mechanical low-back pain patients.

Design: : Eight healthy subjects participated in the tool-validation portion of the study that consisted of surface electromyographic (EMG) measurements of core muscle activation during prone and supine bridging. Subsequently, normative and test-retest reliability measures of prone and supine bridging duration were recorded from 43 subjects without back pain and were compared with those of 32 subjects with chronic mechanical low-back pain.