lateral amyotrophic sclerosis rehabilitation

PURPOSE OF REVIEW: Remote ischemic conditioning (RIC) involves transient blood flow restriction to one limb leading to systemic tissue-protective effects. RIC shares some potential underlying mechanisms with intermittent hypoxia (IH), in which brief bouts of systemic hypoxia trigger increases in growth factor expression and neural plasticity. RIC has shown promise in acute myocardial infarction and stroke but may be applicable toward chronic neuropathology as well. Consequently, this review discusses similarities and differences between RIC and IH and presents preliminary and ongoing research findings regarding RIC...

A 63-year-old male diagnosed with mixed-type cervical spondylotic amyotrophy (CSA) exhibited severe atrophy in the right biceps brachii, teres major, and intrinsic hand muscles, resulting in Level 3 muscle weakness. A MRI scan showed symmetrical high signal, also referred to as the 'snake eye sign'. Previously, he was erroneously diagnosed with amyotrophic lateral sclerosis (ALS). He had received anterior cervical surgery seven years prior. At present, his right upper limb muscles display minimal atrophy compared to the left, with muscle strength nearing Level 4, which is considered normal...

BACKGROUND: Antemortem diagnosis of degenerative myelopathy (DM) in dogs is presumptive and there are no accepted guidelines for the management of this condition. HYPOTHESIS/OBJECTIVES: Describe current practices of neurology clinicians and physical rehabilitation professionals in the diagnosis and management of DM. ANIMALS: None. METHODS: Online surveys examining diagnosis and management of DM were constructed and distributed via neurology and rehabilitation listservs...

OBJECTIVE: The self-administered version of the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) is used to monitor function and disease progression in individuals with Amyotrophic Lateral Sclerosis (ALS). However, the performance of the self-administered ALSFRS-R has not been assessed using Rasch Measurement Theory. Therefore, the purpose of this study was to examine the psychometric properties of the self-administered ALSFRS-R using Rasch analysis. METHODS: Rasch analysis was performed on self-administered ALSFRS-R data from individuals with ALS across Canada...

The Veterans Health Administration (VHA) has served as a leader in the implementation of telerehabilitation technologies and continues to expand utilization of non-traditional patient encounters to better serve a geographically and demographically diverse population. Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease impacting Veterans at a higher rate than the civilian population and associated with high levels of disability and limited access to subspecialized care. There is growing evidence supporting exercise-based interventions as an independent or adjunctive treatment to maintain or restore function for this patient population; many of these interventions can be delivered remotely by telehealth...

INTRODUCTION: Degenerative myelopathy (DM) is a neurodegenerative spinal cord disease with upper motor neurons, with progressive and chronic clinical signs, similar to amyotrophic lateral sclerosis (ALS). DM has a complex etiology mainly associated with SOD1 gene mutation and its toxic role, with no specific treatment. Daily intensive rehabilitation showed survival time near 8 months but most animals are euthanized 6-12 months after clinical signs onset. METHODS: This prospective controlled blinded cohort clinical study aims to evaluate the neural regeneration response ability of DM dogs subjected to an intensive neurorehabilitation protocol with mesenchymal stem cells (MSCs) transplantation...

BACKGROUND: Spinal and bulbar muscular atrophy (SBMA) is a progressive neuromuscular degenerative disease characterized by the degeneration of lower motor neurons in the spinal cord and brainstem and neurogenic atrophy of the skeletal muscle. Although the short-term effectiveness of gait treatment using a wearable cyborg hybrid assistive limb (HAL) has been demonstrated for the rehabilitation of patients with SBMA, the long-term effects of this treatment are unclear. Thus, this study aimed to investigate the long-term effects of the continued gait treatment with HAL in a patient with SBMA...

Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are fatal neurodegenerative disorders often co-occurring in the same patient, a feature that suggests a common origin of the two diseases. Consistently, pathological inclusions of the same proteins as well as mutations in the same genes can be identified in both ALS/FTD. Although many studies have described several disrupted pathways within neurons, glial cells are also regarded as crucial pathogenetic contributors in ALS/FTD. Here, we focus our attention on astrocytes, a heterogenous population of glial cells that perform several functions for optimal central nervous system homeostasis...

Brain-computer interfaces (BCIs) provide the central nervous system with channels of direct communication to the outside world, without having to go through the peripheral nervous system. Neurodegenerative diseases (NDs) are notoriously incurable and burdensome medical conditions that will result in progressive deterioration of the nervous system. The applications of BCIs in NDs have been studied for decades now through different approaches, resulting in a considerable amount of literature in all related areas...

This systematic review and thematic analysis critically evaluated gene therapy trials in amyotrophic lateral sclerosis, haemoglobinopathies, immunodeficiencies, leukodystrophies, lysosomal storage disorders and retinal dystrophies and extrapolated the key clinical findings to individuals with Rett syndrome (RTT). The PRISMA guidelines were used to search six databases during the last decade, followed by a thematic analysis to identify the emerging themes. Thematic analysis across the different disorders revealed four themes: (I) Therapeutic time window of gene therapy; (II) Administration and dosing strategies for gene therapy; (III) Methods of gene therapeutics and (IV) Future areas of clinical interest...

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Introduction: Living with a neuromuscular disease often leads to a need for specialized rehabilitation due to the complexity and progression of the diseases. Aim: To investigate cross-sectoral collaboration on rehabilitation for patients with neuromuscular diseases among hospital professionals to inform future targeted rehabilitation services. Patients & methods: The design was qualitative using the interpretive description methodology and the theoretical lens of symbolic interactionism. Ethnographic fieldwork was conducted, and 50 hospital professionals included, 19 of whom were interviewed...

PURPOSE: Amyotrophic lateral sclerosis (ALS) is a progressive motor impairment leading to early death. Religiousness is one of the factors potentially alleviating the psychological burden of patients. However, its role might vary according to cultural context. Our study aimed to analyze religiosity, and its clinical, psychological and socio-demographic correlates in ALS patients and controls, comparing two European countries with different cultural backgrounds. METHODS: 268 Polish and German ALS patients, including 18 with locked-in syndrome (LIS) and 198 healthy controls (HC) were interviewed about religiousness, quality of life (Qol), depression, functional status and pain...

OBJECTIVE: To systematically evaluate post-exercise outcomes related to function and quality of life in people with ALS. METHODS: PRISMA guidelines were used for identifying and extracting articles. Levels of evidence and quality of articles were judged based on The Oxford Centre for Evidence-based Medicine Levels of Evidence and the QualSyst. Outcomes were analyzed with Comprehensive Meta-Analysis V2 software, random effects models, and Hedge's G. Effects were examined at 0-4 months, up to 6 months, and > 6 months...

BACKGROUND AND OBJECTIVE: The objective of this study was to evaluate the short-term physiologic effect and one-year functional effect of a 12-week inspiratory and expiratory respiratory strength training (RST) program in individuals with amyotrophic lateral sclerosis (ALS). METHODS: A double-blinded, randomized, sham-controlled trial was conducted in 45 individuals with early-stage ALS. Participants were randomized into 12 weeks of active RST (30% load, n = 23) or sham RST (0% load, n = 22)...

Despite promising results in the rehabilitation field, it remains unclear whether upper limb robotic wearables, e.g., for people with physical impairments resulting from neurodegenerative disease, can be made portable and suitable for everyday use. We present a lightweight, fully portable, textile-based, soft inflatable wearable robot for shoulder elevation assistance that provides dynamic active support to the upper limbs. The technology is mechanically transparent when unpowered, can quantitatively assess free movement of the user, and adds only 150 grams of weight to each upper limb...

OBJECTIVES: The aim of this study was to assess the cognitive abilities of people with spinal cord injury (SCI) using the Edinburgh Cognitive and Behavior Amyotrophic Lateral Sclerosis Screen (ECAS), a tool designed for testing cognition in individuals with limited hand motor function. The impact of cognitive dysfunction on quality of life was also assessed. METHODS: Forty-one patients with SCI were assessed using ECAS, the brief version of the World Health Organisation Quality of Life questionnaire (WHOQOL-BREF), and the Spinal Cord Independence Measure...

RATIONALE: Family caregivers of persons with amyotrophic lateral sclerosis and cognitive and/or behavioural impairments (PALS/CIs) experience various challenges and needs, including emotional and practical support from peers. Various forms of peer-support have shown different strengths and weaknesses; however, little is known about how family caregivers of PALS/CIs interact with and perceive virtual face-to-face peer-support. AIMS AND OBJECTIVES: The aim of this study was to understand how caregivers of PALS/CIs interact with and perceive virtual face-to-face peer-support in a palliative rehabilitation programme designed to promote targeted palliative rehabilitation initiatives for caregivers of PALS/CIs...

BACKGROUND: There is increasing interest in the role of EEG in neurorehabilitation. We primarily aimed to identify the knowledge base through highly influential studies. Our secondary aims were to imprint the relevant thematic hotspots, research trends, and social networks within the scientific community. METHODS: We performed an electronic search in Scopus, looking for studies reporting on rehabilitation in patients with neurological disabilities. We used the most influential papers to outline the knowledge base and carried out a word co-occurrence analysis to identify the research hotspots...

Since the 1990s, Veterans Health Administration (VHA) has maintained a registry of Veterans with Spinal Cord Injuries and Disorders (SCI/Ds) to guide clinical care, policy, and research. Historically, methods for collecting and recording data for the VHA SCI/D Registry (VSR) have required significant time, cost, and staffing to maintain, were susceptible to missing data, and caused delays in aggregation and reporting. Each subsequent data collection method was aimed at improving these issues over the last several decades...