Clinical And Molecular Aspects Of Motor Neuron Disease

In this e-book, motor neuron disease (MND) shall refer to amyotrophic lateral sclerosis (ALS), the most common neurodegenerative disorder affecting both the upper and lower motor neurons. With the discovery of C9ORF72 expansions in approximately 10% of all MND cases, in certain populations, we stand at the brink of a new era of MND research and hopefully treatment facilitated by the ability to associate a relatively large group of patients with a similar disease mechanism. This review will summarise both current clinical management of MND and our present understanding of the molecular pathogenesis of MND. Study of C9ORF72-MND has the potential to rapidly advance both of these aspects in the coming years. In the first section, we will discuss the clinical features of MND and describe how patients with this devastating condition present, are investigated, and managed in the 21st century. Although, currently, management is limited by an incomplete understanding of disease pathophysiology, there is much which can be done to assist and support patients with MND. In the following sections, we will discuss molecular mechanisms implicated in MND, highlighting observations which unify different theories. Particular attention will be given to placing proposed mechanisms within the clinical course of MND. Furthermore, novel therapeutic targets will be discussed. Table of Contents: Clinical Aspects of MND / Molecular Aspects of MND / References / Author Biographies / Titles of Related Interest / Series of Related Interest

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

Dedicated to our readers, we include novel information (not reported in IntechOpen’s books before) about new contributions of aberrant astrocytes to MND damage and death in the SOD1G93A rat experimental model of ALS; novel genetic studies on ALS; an update of the structural and functional consequences of the spinal muscular atrophy-linked mutations of the survival motor neuron protein; stem cell therapy for MND; and the novel treatment for SMA and ALS in the introductory chapter. This book contains selected peer-reviewed chapters written by international researchers. In this publication, the readers will find a compilation of state-of-the-art reviews about etiology, therapies, investigations, the molecular basis of disease progression and clinical manifestations, and the genetic familial ALS, as well as novel therapeutic modalities. We look forward with confidence and pride to the remarkable role that this book will play for a new vision and mission.

'Motor Neuron Disease in Adults' reviews new information from 1998 as it applies to all aspects of motor neuron disease. Articles included use evidence-based methods to ensure that the new information is solid and advances the topic. The book can be used by anyone who provides any type of care to ALS patients.

In this e-book, motor neuron disease (MND) shall refer to amyotrophic lateral sclerosis (ALS), the most common neurodegenerative disorder affecting both the upper and lower motor neurons. With the discovery of C9ORF72 expansions in approximately 10% of all MND cases, in certain populations, we stand at the brink of a new era of MND research and hopefully treatment facilitated by the ability to associate a relatively large group of patients with a similar disease mechanism. This review will summarise both current clinical management of MND and our present understanding of the molecular pathogenesis of MND. Study of C9ORF72-MND has the potential to rapidly advance both of these aspects in the coming years.

Here is the first book to provide a comprehensive overview of the clinical, pathological, and research aspects of motor neuron disease (MND). The text contains all essential features of the anatomy, physiology, pharmacology and toxicology of the motor system, a full description of MND and its variants, as well as historical developments and a review of the current concepts and controversies. This book comes at a time of increasing interest in neurodegenerative disorders and MND in particular. It will prove a key reference book with an integrated overview of the field, and will be indispensable to practicing neurologists, researchers, and all those with an interest in MND.

This title reflects the exponential growth in the knowledge and information on this subject and defines the extensive clinical translation of cardiovascular genetics and genomics in clinical practice. This concise, clinically oriented text is targeted at a broad range of clinicians who manage patients and families with a wide range of heterogeneous inherited cardiovascular conditions. Cardiovascular Genetics and Genomics: Principles and Clinical Practice includes a concise and clear account on selected topics written by a team of leading experts on clinical cardiovascular genetics. Each chapter include key information to assist the clinician and case histories have been incorporated to reflect contemporary practice in clinical cardiovascular genetics and genomics. Therefore this will be of key importance to all professionals working in the discipline, from clinicians and trainees in cardiology, cardiac surgery, electrophysiology, immunology through geneticists, nursing staff and those involved in precision medicine.

The Molecular and Clinical Pathology of Neurodegenerative Disease brings together in one volume our current understanding of the molecular basis of neurodegeneration in humans, targeted at neuroscientists and graduate students in neuroscience, and the biomedical and biological sciences. Bringing together up-to-date molecular biology data with clinical evidence, this book sheds a light on common molecular mechanisms that underlie many different neurodegenerative diseases and addresses the molecular pathologies in each. The combined research and clinical background of the authors provides a unique perspective in relating clinical experiences with the molecular understanding needed to examine these diseases and is a must-read for anyone who wants to learn more about neurodegeneration. Provides an up-to-date summary of neurodegeneration at a molecular, cellular, and tissue level for the most common human disorders Describes the clinical background and underlying molecular processes for Alzheimer’s disease, Parkinson’s, Prion, Motor Neuron, Huntington’s, and Multiple Sclerosis Highlights the state-of-the-art treatment options for each disorder Details examples of relevant cutting edge experimental systems, including genome editing and human pluripotent stem cell-derived neuronal models