Spectrums Of Amyotrophic Lateral Sclerosis

SPECTRUMS OF AMYOTROPHIC LATERAL SCLEROSIS Discover state-of-the-art research findings on ALS from leading authors and editors in the field In Spectrums of Amyotrophic Lateral Sclerosis: Heterogeneity, Pathogenesis & Therapeutic Directions, distinguished researchers and editors Dr. Christopher A. Shaw and Jessica R. Morrice deliver a practical and powerful perspective on Amyotrophic Lateral Sclerosis (ALS) as a heterogeneous spectrum of disorders. This increasingly accepted point-of-view allows researchers and medical professionals to develop better targeted interventions and more precise therapies. In the book, readers will find chapters on a wide variety of critical issues facing ALS researchers and healthcare practitioners treating ALS sufferers, including animal models of ALS, neuronal support cells known to have a pivotal role in ALS, and current challenges in ALS clinical trials, among others. The authors describe pathologic features common to all cases of ALS and why animal models, though crucial, should be interpreted with caution. Finally, multiple genetic and environmental etiologies of the disease are discussed. Readers will also benefit from the inclusion of: A thorough introduction to ALS as a spectrum disease and the implications for models, therapeutic development and clinical trial design Explorations of the genetic basis of ALS, prospective sALS etiologies, and the involvement of microbiome in ALS Discussions of ALS-PDC and environmental risk factors, protein aggregation in ALS, defects in RNA metabolism in ALS, and the non-cell autonomous nature of ALS and the involvement of glial cells Examinations of animal models of ALS and perspectives on previously failed ALS therapeutics and current therapeutic strategies Perfect for clinical neurologists, healthcare providers and caretakers, clinicians, and researchers studying motor neuron disease, Spectrums of Amyotrophic Lateral Sclerosis: Heterogeneity, Pathogenesis & Therapeutic Directions is also an indispensable resource for the neurodegenerative research community, neurology residents, and graduate-level neuroscience students.

SPECTRUMS OF AMYOTROPHIC LATERAL SCLEROSIS Discover state-of-the-art research findings on ALS from leading authors and editors in the field In Spectrums of Amyotrophic Lateral Sclerosis: Heterogeneity, Pathogenesis & Therapeutic Directions, distinguished researchers and editors Dr. Christopher A. Shaw and Jessica R. Morrice deliver a practical and powerful perspective on Amyotrophic Lateral Sclerosis (ALS) as a heterogeneous spectrum of disorders. This increasingly accepted point-of-view allows researchers and medical professionals to develop better targeted interventions and more precise therapies. In the book, readers will find chapters on a wide variety of critical issues facing ALS researchers and healthcare practitioners treating ALS sufferers, including animal models of ALS, neuronal support cells known to have a pivotal role in ALS, and current challenges in ALS clinical trials, among others. The authors describe pathologic features common to all cases of ALS and why animal models, though crucial, should be interpreted with caution. Finally, multiple genetic and environmental etiologies of the disease are discussed. Readers will also benefit from the inclusion of: A thorough introduction to ALS as a spectrum disease and the implications for models, therapeutic development and clinical trial design Explorations of the genetic basis of ALS, prospective sALS etiologies, and the involvement of microbiome in ALS Discussions of ALS-PDC and environmental risk factors, protein aggregation in ALS, defects in RNA metabolism in ALS, and the non-cell autonomous nature of ALS and the involvement of glial cells Examinations of animal models of ALS and perspectives on previously failed ALS therapeutics and current therapeutic strategies Perfect for clinical neurologists, healthcare providers and caretakers, clinicians, and researchers studying motor neuron disease, Spectrums of Amyotrophic Lateral Sclerosis: Heterogeneity, Pathogenesis & Therapeutic Directions is also an indispensable resource for the neurodegenerative research community, neurology residents, and graduate-level neuroscience students.

A diagnosis of amyotrophic lateral sclerosis (also known as Lou Gehrig's disease or motor neuron disease) is a progressive neurodegenerative disorder that exerts a notorious life-shortening physical toll. Understandably, clinicians are keen to avoid a wrong diagnosis when there are such serious consequences, but any delay in diagnosis can result in unnecessary, and sometimes harmful, interventions, and prevents prompt implementation of much-needed physical and emotional support. Starting from the premise that ALS is not one disease but a syndrome, with a spectrum of upper and lower motor neuron involvement, this highly readable resource examines the causes of diagnostic delay and how to avoid them. With no diagnostic test to confirm the disease, no mandatory investigations and very few plausible 'ALS mimics', the authors take a pragmatic approach to what must always be a clinical diagnosis. With case presentations and teaching points to aid understanding, 'Fast Facts: ALS' will give clinicians the confidence to confirm or exclude a diagnosis of ALS, so that individuals facing this most challenging of conditions can receive rapid multidisciplinary support to maximize the quality of their remaining life. Contents: • Defining the syndrome • Epidemiology and pathophysiology • The first symptoms • Differential diagnosis • Investigations • Emerging diagnostic biomarkers

Amytrophic Lateral Sclerosis (ALS or motor neurone disease) is a progressive neurodegenerative disease that can cause profound suffering for both the patient and their family. Whilst new treatments for ALS are being developed, these are not curative and offer only the potential to slow its progression. Palliative care must therefore be integral to the clinical approach to the disease. Palliative Care in Amyotrophic Lateral Sclerosis: From diagnosis to bereavement reflects the wide scope of this care; it must cover not just the terminal phase, but support the patient and their family from the onset of the disease. Both the multidisciplinary palliative care team and the neurology team are essential in providing a high standard of care and allowing quality of life (both patient and carer) to be maintained. Clear guidelines are provided to address care throughout the disease process. Control of symptoms is covered alongside the psychosocial care of patients and their families. Case studies are used to emphasise the complexity of the care needs and involvement of the patient and family, culminating in discussion of bereavement. Different models of care are explored, and this new edition utilizes the increase in both the evidence-base and available literature on the subject. New topics discussed include complementary therapies, personal and family experiences of ALS, new genetics research, and updated guidelines for patient care, to ensure this new edition remains the essential guide to palliative care in ALS.

Molecular and Cellular Therapies for Motor Neuron Diseases discusses the basics of the diseases, also covering advances in research and clinical trials. The book provides a resource for students that will help them learn the basics in a detailed manner that is required for scientists and clinicians. Users will find a comprehensive overview of the background of Amyotrophic Lateral Sclerosis (ALS/Lou Gehrig’s Disease) and Spinal Muscular Atrophy (SMA), along with the current understanding of their genetics and mechanisms. In addition, the book details gene and cell therapies that have been developed and their translation to clinical trials. Provides an overview of gene and cell therapies for amyotrophic lateral sclerosis (ALS) and other motor neuron diseases Edited by a leading Neurosurgeon and two research scientists to promote synthesis between basic neuroscience and clinical relevance Presents a great resource for researchers and practitioners in neuroscience, neurology, and gene and cell therapy

Amyotrophic Lateral Sclerosis: A Patient Care Guide for Clinicians is intended as a practical reference for clinicians caring for ALS patients, and will bring together the collective wisdom of those at the forefront of patient-oriented research and practice. This will be an official project of the ALS Research Group (founded by Dr. Mitsumoto and currently headed by Dr. Bedlack), and provides both an evidence-based and experience-based guide to multidisciplinary ALS care. The book will begin with a brief review of current concepts of ALS including diagnostic criteria, genetic and sporadic subty.

ALS, also known as Lou Gehrig's Disease, is the most common of the motor neuron diseases that cause muscle atrophy. ALS is a chronic, progressively debilitating disease characterised by progressive muscle atrophy starting in the limbs and spreading to the rest of the body, often accompanied by overactive reflexes. It usually manifests itself after the age of 40. The exact cause of ALS is unknown and there is no cure at this time. ALS may be fatal in one year or continue for 10 or more years. This new book includes leading edge research from around the world and covers the aetiology, pathogenesis, symptoms, diagnosis, and treatment of amyotrophic lateral sclerosis (ALS).

Though considerable amount of research, both pre-clinical and clinical, has been conducted during recent years, Amyotrophic Lateral Sclerosis (ALS) remains one of the mysterious diseases of the 21st century. Great efforts have been made to develop pathophysiological models and to clarify the underlying pathology, and with novel instruments in genetics and transgenic techniques, the aim for finding a durable cure comes into scope. On the other hand, most pharmacological trials failed to show a benefit for ALS patients. In this book, the reader will find a compilation of state-of-the-art reviews about the etiology, epidemiology, and pathophysiology of ALS, the molecular basis of disease progression and clinical manifestations, the genetics familial ALS, as well as novel diagnostic criteria in the field of electrophysiology. An overview over all relevant pharmacological trials in ALS patients is also included, while the book concludes with a discussion on current advances and future trends in ALS research.

ALS is not a curable disease, but it is a treatable one. Treatments are now available that can make a major difference in prolonging life and enhancing the quality of life for people with the disease, and there are treatments for many of the symptoms of ALS that can help ease its burden. Multidisciplinary teams in specialized ALS centers are providing top quality care and comprehensive rehabilitation for persons with ALS. In spite of the progressive nature of this disease and its clear tendency to shorten life, the momentum of research in this disease is expanding dramatically and numerous clinical trials are testing promising new therapies. Our understanding of the basic causes of ALS is expanding gradually. The substantial resources of patient advocacy groups such as the Amyotrophic Lateral Sclerosis Association and Muscular Dystrophy Association provide tremendous help and support for people with ALS and their families. Although the diagnosis of ALS can initially be devastating, the vast majority of people discovering new courage from within to battle this disease and live life with vigor and enthusiasm. The information in this book will prove useful to people with ALS and their families both in managing the disease and living within its limitations. ''

"Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are clinically distinct neurodegenerative diseases that are connected by genetic and pathological overlap. ALS patients present with muscle weakness and spasticity associated with degeneration of motor neurons in the motor cortex, brainstem, and spinal cord that ultimately leads to death. In contrast, patients with FTLD display cognitive dysfunction associated with degeneration of neurons in the frontal and temporal lobes of the brain. Despite being clinically distinct, 15% of individuals presenting FTLD also have ALS, whereas 30% of individuals with ALS will develop FTLD. This implies that these two neurodegenerative diseases are part of a shared clinical spectrum. In recent years, several mechanisms have been proposed as contributory factors in the pathogenesis of neuron damage in ALS and FTLD, including excitoxicity, mitochondrial and energy metabolism failure, oxidative stress damage, altered glial cells, inflammation, cytoskeletal abnormalities, alterations in RNA metabolism, and altered TDP-43 metabolism, among others. However, it is poor known about the etiology of these disorders and their possible treatment. The objective of the investigations presented in this doctoral thesis is focused in the identification of new molecular alterations underlying motor and cognitive changes in post-mortem human spinal cord and brain samples of ALS patients and brain samples of FTLD-TDP patients compared with controls, combining microarray, mRNA, protein and enzyme assays studies. The obtained results have identified new molecular alterations in ALS and FTLD of different biological functions and cellular pathways including changes in mitochondrial energy metabolism, neuroinflammation, neuronal structure, neurotransmission, axonal transport mechanisms and oligodendrocyte function; allowing in turn, the screening and identification of new candidate molecules as biomarkers for these disorders." -- TDX.