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This new two-volume reference offers a practical approach to diseases of the muscle, neuro-muscular junction, and spinal cord. Volume I emphasises the pathophysiology of neuromuscular disease and its assessment using electrophysiological and radiological tools. Volume II focuses on the application of electrophysiological testing to the diagnosis and management of specific neuromuscular disorders. Edited by three respected experts, this essential resource also explores HIV, clinical trials, and neuromuscular disorders in critical care and the operating room. Integrates all the guidance needed to diagnose and manage the full range of neuromuscular diseases. Enhances the reader's ability to take neuromuscular histories, perform physical examinations, order and interpret laboratory tests, make effective management decisions and offer patients an accurate prognosis. Discusses new advances in electrophysiologic tests as well as genetic testing and the use of MRI to localize and diagnose neuromuscular conditions. Examines new management techniques such as the use of immunosuppressive drugs to treat Guillain-Barré syndrome, immune mediated peripheral neuropathy, myasthenia gravis and more. Features coverage of important topics such as HIV, clinical trials and neuromuscular disorders in critical care and the operating room. Details both adult and paediatric neuromuscular disorders. Reviews the basic science and genetics practicing physicians need to care effectively for their patients.
Neuromuscular Function and Disorders focuses on the various processes underlying disordered neuromuscular function. Topics covered include the nature of membrane defects in myotonia and familial periodic paralysis; the disorder of neuromuscular transmission responsible for myasthenia gravis and the various pseudo-myasthenic syndromes; and the disorders of Schwann cell function which cause demyelination. This book is comprised of 28 chapters divided into two sections and begins with a discussion on the normal anatomy and physiology of peripheral nerve and muscle. Included in the first section are descriptions of the ionic mechanisms responsible for the resting and action potentials of nerve and muscle; the sequential stages in neuromuscular transmission; excitation-contraction coupling; the sliding filament mechanism of myofibrillar shortening; and the morphological and functional properties of motor units. The neurophysiology of exercise and muscle fatigue is also considered, along with the nature of the trophic influences exerted by the motoneuron and muscle fiber upon each other. The second half of the book deals entirely with various diseases of peripheral nerve and muscle, together with diagnostic procedures and therapeutic management. A consistent theme in this section is the recognition of neural abnormalities in diseases hitherto considered as primary disorders of the muscle fiber. This monograph should be of value to neurologists, medical students, research workers, and students and research scientists in physiology, zoology, pharmacology, kinesiology, and physical education.
Comprehensive, thoroughly updated, and expanded, Neuromuscular Disorders in Clinical Practice, Second Edition encompasses all disorders of the peripheral nervous system, covering all aspects of neuromuscular diseases from diagnosis to treatment. Mirroring the first book, this two-volume edition is divided into two parts. Part one discusses the approach to neuromuscular disorders, covering principles and basics, neuromuscular investigations, and assessment and treatment of neurological disorders. Part two then addresses the complete range of specific neuromuscular diseases: neuronopathies, peripheral neuropathies, neuromuscular junction disorders, muscle ion channel disorders, myopathies, and miscellaneous neuromuscular disorders and syndromes. Neuromuscular Disorders in Clinical Practice, Second Edition is intended to serve as a comprehensive text for both novice and experienced practitioners. General neurologists as well as specialists in neuromuscular medicine and trainees in neuromuscular medicine, clinical neurophysiology and electromyography should find this book inclusive, comprehensive, practical and highly clinically focused. Additionally, specialists in physical medicine and rehabilitation, rheumatology, neurosurgery, and orthopedics will find the book of great value in their practice.
Nine years have elapsed since the second edition of this book was published. In this time the principal advances in neuromuscular diseases have been in the application of molecular genetics to understanding the aetiology and pathogenesis of this group of disorders. As a result many previously unrecognised disorders have been charac terised. Some clinical syndromes, such as the limb girdle dystrophies, have become better defined. In many such instances the new genetic information has led to major advances in knowledge of the biology of cell structures, for example, the membrane structural and channel proteins. The clinical syndromes themselves, and their patho logical and electrophysiological characteristics, however, remain as important as ever, since they constitute the clinical problem itself and, indeed, the database from which all other concepts emerge. Knowledge of the pathogenesis, genetics, and molecular biology of neuromuscular disorders is essential both in developing and applying new therapies and preventive measures, and in formulating genetic and prognostic advice. However, this informa tion does not necessarily always define clinically useful syndromes. Myotonia, for example, is an electrophysiological finding in some syndromes in which it is un detectable by clinical examination, although the phenomenon itself was originally defined as a clinical entity. The limb girdle muscular dystrophy syndromes can be defined by severity, distribution of weakness, age of onset, sex distribution and other characteristics and many of these can be better understood by study of the under lying defect in cell structural proteins.
This atlas offers a comprehensive overview of neuromuscular diseases. It discusses all aspects of neuromuscular disorders, including general tools, the cranial and spinal nerves, the nerve plexus, peripheral nerves, mono- and polyneuropathies, entrapment syndromes, the neuromuscular junction, motor neuron diseases, muscle disease, and autonomic involvement. Each chapter is structured into the following sections: anatomy, symptoms, signs, pathogenesis, diagnosis and differential diagnosis, therapy, and prognosis. The diagnostic tools used for neuromuscular disease are explained, and the therapeutic options for each disease are described. This updated third edition includes new chapters addressing a range of topics: from histology to molecular mechanisms, genetic aspects, the mechanisms of emerging new therapies, neuroimaging, neuromuscular disease, and new pathogenic mechanisms. The book aims to be a useful companion for neuromuscular disease. The homogenous structure, illustrations with figures, and representative images makes the atlas easy to read and helpful in understanding neuromuscular problems.
Neuromuscular disorders are diagnosed across the lifespan and create many challenges especially with infants, children and adolescents. This new edition of the definitive reference, edited by the established world renowned authorities on the science, diagnosis and treatment of neuromuscular disorders in childhood is a timely and needed resource for all clinicians and researchers studying neuromuscular disorders, especially in childhood. The Second Edition is completely revised to remain current with advances in the field and to insure this remains the standard reference for clinical neurologists and clinical research neurologists. The Second Edition retains comprehensive coverage while shortening the total chapter count to be an even more manageable and effective reference. Carefully revised new edition of the classic reference on neuromuscular disorders in infancy, childhood and adolescence. Definitive coverage of the basic science of neuromuscular disease and the latest diagnosis and treatment best practices. Includes coverage of clinical phenomenology, electrophysiology, histopathology, molecular genetics and protein chemistry
In this unique book, Dr. Bertorini guides you through more than 100 cases that demonstrate the diagnosis and management of a wide range of common and rare neuromuscular disorders. No other reference boasts such a large array of clinical studies devoted to all areas of this broad topic! Each case study reviews the etiologies, pathogenesis, differential diagnosis, and management of a particular disorder, helping you not only recognize its presentation, but also determine a diagnosis and the best treatment plans for your patients. You’ll also find expert guidance on the basic mechanisms of neuromuscular disorders, clinical examination, and diagnostic tests—including EMG, muscle biopsy, genetic testing, and more. More than 100 detailed case studies explore both common and rare neuromuscular disorders and the treatment protocols for each, equipping you with the knowledge you need to confidently manage any challenge. Each case includes a summary of important points or highlights of the study. Case studies are arranged either by complaint or by diagnosis so that you can successfully manage your patients with or without an initial diagnosis. Comprehensive coverage of EMGs and nerve conduction studies and other diagnostic tests, including muscle and nerve biopsies and genetic testing, helps you accurately diagnose nerve, muscle, and neuromuscular transmission disorders. Detailed discussions of treatment plans and commonly used drugs enhance your management of autoimmune disorders, painful neuropathy, dysautonomia, and other neuromuscular disorders. A reader-friendly format takes you step by step through the diagnosis and treatment of neuromuscular disorders, from the basic anatomy and physiology of the nerve and muscle through to clinical evaluation, diagnostic testing, and therapy. More than 350 high-quality illustrations, including full-color patient photographs, biopsies, and EMG tracings, make complex concepts easier to understand and apply.