Neuromuscular Disorders

Neuromuscular Disorders presents a multi-disciplinary approach to the management and therapeutic treatment of the full range of neuromuscular disorders and resulting complications. Dr. Tulio Bertorini and a contributing team of the world’s leading authorities in the field provide the latest tools and strategies for minimizing disability and maximizing quality of life. Effectively treat your patients using the latest management tools and targeted therapeutic strategies. Manage all neuromuscular disorders as well as resulting complications through comprehensive coverage of diagnosis and evaluations, treatments, and outcomes. Apply the multi-disciplinary approach of an expert in clinical neuromuscular care and a team of world-renown contributors. Easily refer to tools for diagnosis, treatment algorithms, and drug tables included throughout the text.

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

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.

Diagnose neuromuscular disorders more quickly and accurately with Electromyography and Neuromuscular Disorders: Clinical-Electrophysiologic Correlations, 3rd Edition! State-of-the-art guidance helps you correlate electromyographic and clinical findings and use the latest EMG techniques to their fullest potential. Consult this title on your favorite e-reader with intuitive search tools and adjustable font sizes. Elsevier eBooks provide instant portable access to your entire library, no matter what device you're using or where you're located. Successfully correlate electrodiagnostic findings with key clinical findings for more confident diagnoses. Clearly see how to apply what you’ve learned with abundant case studies throughout the book. Obtain relevant clinical guidance quickly and easily with an accessible, easy-to-read writing style that’s both comprehensive and easy to understand. Ensure correct EMG needle placement and avoid neurovascular injuries by referring to more than 65 detailed, cross-sectional anatomy drawings. Diagnose many newly defined genetic neuromuscular conditions based on their electrodiagnostic presentation. Stay up to date with must-know information on iatrogenic complications of electrodiagnostic studies. Visualize key concepts more easily with a brand-new full-color design, new artwork, and new photographs. Access Electromyography and Neuromuscular Disorders online, fully searchable, at www.expertconsult.com, along with more than 70 videos that allow you to see and hear the EMG waveforms discussed in the text, as well as a convenient "test yourself" module.

Part of the Oxford Textbooks in Clinical Neurology series, the Oxford Textbook of Neuromuscular Disorders covers the scientific basis, clinical diagnosis, and treatment of neuromuscular disorders with a particular focus on the most clinically relevant disorders. The book is organized into seven sections, starting with the general approach to the patient with neuromuscular disorders and then focusing on specific neuromuscular conditions affecting the peripheral nervous system from its origins at the spinal cord anterior horn on its outward course to their effector muscles and the inbound sensory pathways. Chapters on specific neuromuscular conditions are illustrated with typical case histories and their presenting features, allowing readers to put rarer conditions into their clinical context more easily. The concurrent online version allows access to the full content of the textbook, contains links from the references to primary research journal articles, allows full text searches, and provides access to figures and tables that can be downloaded to PowerPointRG. This textbook will serve as a useful reference for neurologists and specialist trainees in neurology, neuroscientists, neurophysiologists, and other healthcare professionals.

This book provides state of the art information on the pathogenesis, diagnosis, and treatment of a range of inflammatory, autoimmune, and idiopathic neuromuscular disorders. The opening section discusses the differential diagnosis of acquired myopathies based on clinical, electrophysiological, muscle biopsy, and serological criteria, with special focus on the role of electromyography and antibody testing. Each of the relevant clinical entities is then discussed in detail, the coverage including, for example, myasthenia gravis, polymyositis, immune-mediated neuropathies, multifocal motor neuropathies, amyotrophic lateral sclerosis, paraneoplastic neuropathies, and diabetic polyneuropathy. Clear guidance is provided on currently available treatments, with descriptions of the latest advances in physiotherapy, drug therapy, and respiratory care. Other features of this clinically oriented book are the inclusion of many illustrative case presentations and contributions by international authors from leading centers.

Everything you need to know about neuromuscular disease-in one concise, full-color volume Neuromuscular Disorders is comprehensive in scope, yet concise enough to serve as a valuable point-of-care guide for your day-to-day clinical practice. Grounded by numerous reference sources and enhanced by the experience of two seasoned clinicians, this text provides a pragmatic approach to the evaluation and management of neuromuscular disease. Here, in a single source, you'll get an insightful look at both nerve and muscle conditions, ranging from motor neuron diseases to neuropathies, neuromuscular junction disorders, and myopathies. The book opens with the clinical and laboratory approach to patients with neuromuscular disease, covering electrodiagnostic testing, nerve conduction studies, and muscle and nerve histopathology. With an emphasis on effective diagnosis and treatment, the substance of the text examines the full spectrum of specific neuromuscular diseases, giving you the “big picture” of the discipline in a way that no other text can match. FEATURES: Convenient coverage of both nerve, muscle and neuromuscular transmission disorders-both primary and secondary neuromuscular diseases Specific disease sections encompass the range of conditions likely to be encountered in clinical practice, including amyotrophic lateral sclerosis, myasthenia gravis, radiculopathies and plexopathies, mononeuropathies, various polyneuropathies, toxic myopathies, and dozens more The very latest diagnosis and treatment recommendations and protocols based on the most recent clinical literature and research Well-illustrated with EMGs, histopathology, and clinical photos

This book provides a concise overview of the diagnosis and therapy of a wide variety of neuromuscular disorders, in tabulated form and with illustrative cases. Treating neuromuscular disorders calls for a sound, step-by-step clinical approach based on differential diagnosis and laboratory investigations. Yet to date, there has been no single, compact book that offers all the relevant information related to the management of these disorders. This book fills that gap, presenting the state of the art in the field and addressing practical problems together with their solutions. Each chapter covers disorder characteristics, clinical differentiating points, relevant investigations and their interpretation, available genetic testing, best management approaches and counselling. Illustrative cases provide valuable insights, while extensive tables and illustrations mean that information can be rapidly found.

This updated and expanded new edition of a successful book describes genetic diagnostic entities of neuromuscular disorders. Neuromuscular syndromes are presented clinically either as a case study or as an overview from the literature, accompanied by text presenting molecular defects, and differential diagnosis. This collection of neuromuscular disorders features the differential clinical phenotypes related to each genotype and are representative of the whole spectrum of a genetic muscle disorder, helping the clinician and neuromuscular physician to make a diagnosis. Key points for each genetic disease are identified to suggest treatment, when available, or the main clinical exams useful in follow-up of patients. Genetic Neuromuscular Disorders: A Case-Based Approach is aimed at neuromuscular physicians and neurology residents.

The first real cases video atlas of neuromuscular disorders that is supplemented with multiple-choice questions, and updates on the illustrated topics. It is easy to search and read. It is perfect for preparation to the neurology and neuromuscular boards and an excellent way to update the experts. By replacing the descriptive text with vivid illustrative videos, the reader will have more time to face the intellectual challenges of these cases instead of trying to build a mental picture of these cases first. Short and well-edited video clips from real clinic stories supplemented with challenging multiple choice questions, provides an excellent way to bridge the gap between overflow of information and short attention span. The chapters are arranged according the symptoms instead of diseases, yet, diseases are listed in the index if one wants to see all videos relevant to a specific disease. Close to 300 video cases* taken directly from a real neuromuscular clinic, illustrating a myriad of disorders and shedding light on their diagnosis, and treatment and giving updates about many of them provides an invaluable approach that should benefit any one who is interested in neuromuscular disorders which comprises more than 50% of presenting disease to general neurologists and even to general practitioners. Some rare diseases are also described, giving an opportunity for the new trainees to see them so that they can diagnosed them if they see them again which may not happen very often. *Due to size limitations, the videos are not included with any eBook version.

A highly-illustrated, case-based clinical guide for diagnosing and managing adult neuromuscular disease, starting from the case-history to mimic clinical practice.

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.

A Certain Kind of Wisdom In Plato’s Apology, the Greek philosopher Socrates is on trial to defend himself against the allegation of corrupting the youth of Athens. Socrates denies this charge and offers an alternate reason for why he is on trial. He explains, “[w]hat has caused my reputation is none other than a certain kind of wisdom. What kind of wisdom? Human wisdom, perhaps(1). ” He proceeds to tell the story of his friend Chaerophon, who once asked the Oracle at Delphi whether there was anyone wiser than Socrates. The Oracle answered that there was not. Socrates did not agree and thought that he would try to prove the Oracle wrong. And so he set about seeking out Athenians with a reputation for wisdom in various regards in order to test their claims to knowledge through questioning. He discovered many with false claims to knowledge and none with genuine wisdom and ultimately concluded that he was the wisest. He reached this conclusion not because of any special knowledge he possessed that others did not, but rather because he recognized his own lack of knowledge and strived to learn more, while others thought that they were kno- edgeable but were not. Socrates’ conclusion that there is wisdom in recognizing the limitations of accepted knowledge represents the motivation for this book.

This comprehensive book addresses the acute emergencies in neuromuscular disease from a novel perspective, focusing on clinical management and treatment of these disorders rather than the more traditional neuropathology, neurogenetics, and neurophysiology approaches. The book fills the gap in guidelines by providing an evidence-based guidance for the clinical adult or pediatric neurologist confronted by an acutely ill patient in a potentially life-threatening situation. The book is structured according to pathophysiological principles, but each chapter is strictly organised around case vignettes that emphasize clinical relevance allowing the non-specialist to access complex scientific backgrounds. The electronic supplementary material and multimedia are provided as an integral tool to facilitate accessibility and understanding of a notoriously difficult field of neurology. Finally, the role of patients, who are increasingly involved in many management decisions, is highlighted in a chapter on ethical issues discussing withholding or withdrawing life-sustaining therapy, patient end-of-life values, wishes and preferences, and on advanced directives. The publication is aimed equally at the non-specialist neurologist in frontline clinical practice confronted by a rare disorder needing immediate management decisions, at the clinical specialist referring for guidance in specific management aspects, and at the specialist researcher keen to maintain clinic relevance.

Core Topics in Neuroanesthesia and Neurointensive Care is an authoritative and practical clinical text that offers clear diagnostic and management guidance for a wide range of neuroanesthesia and neurocritical care problems. With coverage of every aspect of the discipline by outstanding world experts, this should be the first book to which practitioners turn for easily accessible and definitive advice. Initial sections cover relevant anatomy, physiology and pharmacology, intraoperative and critical care monitoring and neuroimaging. These are followed by detailed sections covering all aspects of neuroanesthesia and neurointensive care in both adult and pediatric patients. The final chapter discusses ethical and legal issues. Each chapter delivers a state-of-the art review of clinical practice, including outcome data when available. Enhanced throughout with numerous clinical photographs and line drawings, this practical and accessible text is key reading for trainee and consultant anesthetists and critical care specialists.

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 book offers the interventions that the researchers and clinicians of the UMDNH-NJMS Center for Ventilator Management Alternatives and Pulmonary Rehabilitation have found most effective as well as the interventions offered by other investigators so that the clinician can choose among all available options. It is designed to be a comprehensive guide for the day-to-day management of these conditions.

This book provides expert advice from leading authorities on how and when to use the numerous evaluation tests now available for diagnosis and management of neuromuscular disorders. Its practical approach will appeal to practicing neurologists as well as neurology residents and fellows. All methods of testing and assessment are covered and an invaluable section on protocol summarizes the material. A practical source of information on how and when to select a test written by experts in the field Includes all methods of neuromuscular disorder tests and assessments Assists the practitioners in narrowing down the number of tests necessary to diagnose a condition

This issue of Neurologic Clinics, guest edited by Dr. Aziz Shaibani, is devoted to Case Studies in Neuromuscular Disorders. This issue is one of four selected each year by the series Consulting Editor, Dr. Randolph W. Evan. Articles in this issue include: Myotonia, Muscular Dystrophy, Spinal Muscular Atrophy, Advances in Clinical Genetics, FSHD New Diagnosis and Therapies, Congenital Myasthenic Syndrome, Diabetic Amyotrophy, ALS: Management Problems, Diagnostic and Therapeutic Challenges in Myasthenia Gravis, Scapular Winging, Small Fiber Neuropathy, Myopathies, Distal Myopathies, Inflammatory Myopathies, Muscle Cramps, Misdiagnosis of IBM, and Immune Mediated Neuropathies.

This book functions as a practical reference for recognizing various patient presentations, signs, and symptoms that must be considered when treating neuromuscular disorders (NMD). It emphasizes the importance of recognizing these preexisting conditions as this can be crucial to treating patients properly with appropriate medications and procedures. Concise yet comprehensive, this 10-chapter guide analyses various neuromuscular weaknesses including respiratory and progressive muscle weakness. Chapters address the involvement of multiple organ systems in NMD, with specific attention to cardiomyopathy, cardiac arrhythmias, and dystrophinopathies. Discussions also address the challenges practitioners face when treating vulnerable demographics such as pregnant women and those with hyper metabolic conditions. Written by experts in the field, Neuromuscular Urgencies and Emergencies is an invaluable resource for neurologists, emergency medicine physicians, physician assistants, and the interventional neurologist. ​