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International Review of Movement Disorders series, highlights new advances in the field, with this new volume presenting interesting chapters. Each chapter is written by an international board of authors. - Provides the authority and expertise of leading contributors from an international board of authors - Presents the latest release in the International Review of Movement Disorders series - Updated release includes the latest information on Myoclonus

Huntington's disease (HD) is one of the most common dominantly inherited neurodegenerative disorders, characterized by a clinical trial of movement disorder, cognitive deficits, and psychiatric symptoms. Huntington's Disease: Pathogenic Mechanisms and Implications for Therapeutics, reviews the most up-do-date content on HD pathogenic mechanisms and cutting-edge testing of therapeutic strategies for HD. Chapters explore areas such as, normal huntingtin biology in brain development and function, genetic modifiers of HD in patients, molecular pathogenic mechanism in HD, and mechanisms underlying selective neuronal vulnerability - Reviews the clinical course and genetics of HD - Reviews the biology of human huntingtin and HD-relevant cell types - Reviews the wide range of pathobiology associated with mutant huntingtin - Reviews genetic studies of HD and how these studies are informing the development of new therapeutic approaches - Reviews new tools and model systems for basic and translational research in HD, including new human-derived model systems, as well as systems biology and artificial intelligence–driven approaches - Provides an overview of new therapeutic approaches and current clinical programs in HD

Despite the critical importance of the cerebellum in brain function, the scientific community still lacks effective treatments for most cerebellar ataxias. This book provides a link between the pathogenesis and therapies of cerebellar ataxias while also providing a comprehensive assessment of the preclinical and clinical trials dedicated to cerebellar ataxias over the past 20 years of progress. This is the first book fully dedicated to the trials and therapies of these disorders. It is a truly authoritative and comprehensive reference, and comes at a time of major advances in genetic tools and neuroimaging assessments. The coverage begins by laying a foundation of the basic science of the cerebellum and ataxias, proceeds to discuss biomarkers and the tools of trials, offers guidelines on conducting trials, and then explores the full range of therapeutics and their trials, including gene therapy and cell transplantation. The authors are top experts on cerebellar research and the contributing authors have all made seminal contributions in the field.

This book logically and systematically reviews the major movement disorder emergencies. It instructs the reader on how to recognize and manage these problems. This updated new edition of Movement Disorder Emergencies consists of five comprehensive sections. It begins with chapters that relay the various practical approaches one can administer when treating a patient with a movement disorder emergency. Section two then delves into specific hyperacute movement disorder emergencies such as acute parkinsonism, serotonin syndrome, and malignant catatonia. Following this, section three examines various acute movement disorder emergencies, including tic emergencies and Sydenham's chorea. Sections four and five explore issues that can occur during treatment, such as pitfalls in diagnosis and risks in the clinic. Written by prominent neurologists from around the world, Movement Disorder Emergencies, 3rd edition is a practical reference that achieves excellence in diagnosis and management of movement disorder emergencies.

Cellular Migration and Formation of Neuronal Connections, Second Edition, the latest release in the Comprehensive Developmental Neuroscience series, presents the latest information on the genetic, molecular and cellular mechanisms of neural development. This book provides a much-needed update that underscores the latest research in this rapidly evolving field, with new section editors discussing the technological advances that are enabling the pursuit of new research on brain development. This volume focuses on the formation of axons and dendrites and cellular migration. Features leading experts in various subfields as section editors and article authors Presents articles that have been peer reviewed to ensure accuracy, thoroughness and scholarship Includes coverage of mechanisms which regulate the formation of axons and dendrites and cellular migration Covers neural activity, from cell-intrinsic maturation, to early correlated patterns of activity

The scope of the book includes all neurological problems in infancy. In the first half, it sets out in a succinct format the principles of working with families, of clinical assessment, of the use of investigations and treatments, and of evidence-based medicine. The second, symptom-based half of the book provides detailed guidance on the practice of child neurology in infancy. The authors are distinguished experts drawn from all over Europe. The contributors have approached their chapters using an accessible, didactic style and consistent organization of material. The emphasis throughout is on clinical methods, the use of appropriate investigations and treatments, and the avoidance of unnecessary or potentially harmful interventions. For quick reference, all chapters feature boxes highlighting key messages, common errors, and when to worry. The book is designed to be carried round as a handy reference.

Epilepsy is one of most frequent neurological disorders affecting about 50 million people worldwide and 50% of them have at least another medical problem in comorbidity; sometimes this is a the cause of the epilepsy itself or it is due to shared neurobiological links between epilepsy and other medical conditions; other times it is a long-term consequence of the antiepileptic drug treatment. The Comorbidities of Epilepsy offers an up-to-date, comprehensive overview of all comorbidities of epilepsy (somatic, neurological and behavioral), by international authorities in the field of clinical epileptology, with an emphasis on epidemiology, pathophysiology, diagnosis and management. This book includes also a critical appraisal of the methodological aspects and limitations of current research on this field. Pharmacological issues in the management of comorbidities are discussed, providing information on drug dosages, side effects and interactions, in order to enable the reader to manage these patients safely. The Comorbidities of Epilepsy is aimed at all health professionals dealing with people with epilepsy including neurologists, epileptologists, psychiatrists, clinical psychologists, epilepsy specialist nurses and clinical researchers. - Provides a comprehensive overview of somatic, neurological and behavioral co-morbidities of epilepsy - Discusses up-to-date management of comorbidities of epilepsy - Written by a group of international experts in the field

Cardioskeletal Myopathies in Children and Young Adults focuses on plaques that kill people in their 40's-50's and the way they start to form in young adulthood. The Annals of Family Medicine report that approximately half of young adults have at least one cardiovascular disease risk factor (Mar 2010), and an increase in cardiovascular mortality rates in young adults was substantiated in a study at Northwestern Medicine (Nov 2011). Given the increasing recognition of genetic triggers behind all types of cardiovascular disease, and the growing population of young adults with primary or acquired myocardial disease, the need has arisen for a reference that offers a comprehensive approach to the understanding of basic, translational, and clinical aspects of specific muscle diseases while making the link between young adult and adult health. - Reveals the link between cardiac muscle disease and skeletal muscle disease - Explains how genetics and environmental factors effect muscle function of diverse origins - Designates current and novel therapeutic strategies that target both cardiac and skeletal muscle systems

This highly accessible work, now thoroughly revised, has shown thousands of students and clinicians how to assess and treat children's emotional and behavioral difficulties from a developmental perspective. The authors provide a sound understanding of typical development (ages 2–12) and the risk and protective factors for psychopathology. Chapters on common psychological disorders and family stressors describe the nature of each problem, review evidence-based treatments, and offer step-by-step guidelines for intervention, illustrated with helpful case examples. A comprehensive framework for assessing children and planning treatment is used throughout. Purchasers get access to a Web page where they can download and print the book's 24 reproducible forms and handouts in a convenient 8 1/2'x 11'size. New to This Edition: •Chapters on developmental disabilities and trauma. •Significantly revised to reflect advances in assessment, treatment, and developmental psychopathology research. •Additional material on pharmacological treatments in each disorder-specific chapter. •Sibling rivalry chapter expanded to include difficulties with peers. •Updated for DSM-5.

Huntington Disease summarizes the most recent findings related to the disease, providing both cutting edge coverage for clinical/research specialists looking to expand their knowledge base of Huntington disease information, as well as solid groundwork for advanced students from various backgrounds (neurology, psychiatry, neuropsychology, genetics). The volume includes all major areas of Huntington disease clinical care and research, whereas many other HD texts focus solely on neurological symptoms. This book also addresses behavioral and cognitive symptoms, brain imaging, and family dynamics and therapeutic alliances in working with individuals affected by HD. Clinical trials are covered extensively, including design considerations for therapeutic studies. The devastating nature of Huntington's disease is well appreciated throughout the neuroscience, neurology, and psychiatric communities, and a great amount of basic and clinical research is currently taking place. However, much of that occurs in isolated research silos, and it is critical that an interdisciplinary resource be developed to provide in depth information to enhance communication and collaboration. This volume in the Handbook of Clinical Neurology series is that resource. - Includes coverage of both basic science and clinical aspects of the disease, as well as treatment, experimental therapeutics, and biomarkers - Provides an essential resource for the non-neurologist, including necessary background for understanding the disease before making a more detailed study proposal - Provides an interdisciplinary approach that can be applied in everyday clinic and research efforts - Features chapters edited by leaders in the field around the globe—the broadest expert coverage available

The 2nd Edition of Metabolic Diseases provides readers with a completely updated description of the Foundations of Clinical Management, Genetics, and Pathology. A distinguished group of 31 expert authors has contributed 25 chapters as a tribute to Enid Gilbert-Barness and the late Lewis Barness--- both pioneers in this topic. Enid's unique perspectives on the pathology of genetic disorders and Lew's unsurpassed knowledge of metabolism integrated with nutrition have inspired the contributors to write interdisciplinary descriptions of generally rare, and always challenging, hereditary metabolic disorders. Discussions of these interesting genetic disorders are organized in the perspective of molecular abnormalities leading to morphologic disturbances with distinct pathology and clinical manifestations. The book emphasizes recent advances such as development of improved diagnostic methods and discovery of new, more effective therapies for many of the diseases. It includes optimal strategies for diagnosis and information on access to specialized laboratories for specific testing. The target audience is a wide variety of clinicians, including pediatricians, neonatologists, obstetricians, maternal-fetal specialists, internists, pathologists, geneticists, and laboratorians engaged in prenatal and/or neonatal screening. In addition, all scientists and health science professionals interested in metabolic diseases will find the comprehensive, integrated chapters informative on the latest discoveries. It is our hope that the 2nd Edition will open new avenues and vistas for our readers and that they will share with us the interest, excitement and passion of the research into all these challenging disorders.

The second edition of Neurobiology of Disease includes nearly 200 articles surveying all major disorders of the nervous system in both adults and children, focusing on relevant diagnosis and treatments from the perspective of cutting edge clinical and basic neurobiological research. Akin to an encyclopedia of every neurologic disorder, this comprehensive work is ideal for graduate and medical school students, residents, and candidates preparing for their board certification examinations. Each chapter is illustrated with detailed figures, supplemented with descriptive and diagnostic tables, and thoroughly referenced for further investigations. The book's editors, Michael V. Johnston, Harold P. Adams Jr., and Ali Fatemi bring their unique expertise in clinical and research neurology to the overall scope of this work. To further enhance the scope and quality of this new edition, the following Section Editors provided oversight of their respective sections: · Movement Disorders-Joel Perlmutter, Washington University · Dementias-David Knopman, Mayo Clinic · Motorneuron Diseases-Merit Cudkowicz, Massachusetts General Hospital · Paroxysmal Disorders-Solomon Moshe, Albert Einstein College of Medicine · Pediatric Neurology and Developmental Disorders-Tanjala Gipson and Deepa Menon, Kennedy Krieger Institute and Johns Hopkins University · Neuroimmunological Diseases-Carlos Pardo-Villamizar, Johns Hopkins University · Cerebrovascular Diseases-Harold P. Adams Jr., University of Iowa · Peripheral and Autonomic Nervous System Disorders and Pain-Nicholas Maragakis, Johns Hopkins University · Neoplastic and Paraneoplastic Diseases-Lisa DeAngelis, Memorial Sloan-Kettering Cancer Center · Infectious Diseases of the Nervous System-Karen L. Roos, Indiana University · Sleep Disturbances-Mark Dyken, University of Iowa · Substance Abuse and Toxicology Disorders-Barry E. Kosofsky, Weill-Cornell University Medical Center · Neurologic Manifestations of Medical Disorders-John C. Probasco, Johns Hopkins University

Frontiers in Clinical Drug Research – Central Nervous System presents the latest researches and clinical studies on the central nervous system (CNS). It covers a range of topics such as the development and pathophysiology of the brain and spinal cord, physiological sites of drug action in the CNS and clinical findings on drugs used to treat CNS defects due to injury or impaired development. In addition to clinical research on humans, the book also highlights other avenues of CNS medicine and research such as pain medicine, stem cell research, pharmacology, toxicology and translational models in animals. The second volume of the series features chapters on the following topics: - Nucleic acids as drugs for neurodegenerative diseases - Cellular Cysteine Network (CYSTEINET): - Non-motor Symptoms in Parkinson's Disease and drug therapies - Multi-modal pharmacological treatments for major depressive disorder

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

This comprehensive edited volume synthesizes the current state of research and practice in psychological, medical, and motor disorders as they affect individuals with intellectual disabilities (ID). It examines how these disorders exist across this population, sometimes confound diagnosis, and often affect individuals'quality of life. In addition, this book explores which treatments are effective for patients and points to future challenges.Comorbid conditions featured include:Challenging behaviors.ADHD, autism, and other conditions present during early childhood.Anxiety, depression, and schizophrenia.Balance and gait problems.Cerebral palsy.Medical conditions common to persons with ID, such as epilepsy, obesity, and chronic pain.Comorbid Conditions in Individuals with Intellectual Disabilities is an essential resource for researchers, clinicians/professionals, and graduate students in clinical child, school, and developmental psychology, child and adolescent psychiatry, and social work as well as rehabilitation medicine/therapy, behavioral therapy, pediatrics, and educational psychology.

This fourth edition of Huntington's Disease presents a comprehensive summary of the current knowledge of this disease, including the major scientific and clinical advances that have occurred since publication of the third edition in 2002. Completely updated and expanded, chapters in this volume are organized in five sections: · Clinical aspects of Huntington's disease, including updated chapters on historical perspectives, neurological, neuropsychiatric, and neuropsychological aspects, and new chapters on juvenile Huntington's and the premanifest and early stages · The genetics of Huntington's disease, including new information on its epidemiology discussions of new testing guidelines · Neurobiology, including recent insights into correlations between pathology and symptoms and a new chapter on neuronal circuitry · The molecular biology of Huntington's disease, including new chapters on the normal function of huntingtin, the molecular pathogenesis of Huntington's disease and the peripheral pathology of the disorder, and an extensively updated chapter on its structural biology · An updated description of the comprehensive care for Huntington's disease, featureing a new chapter on preclinical therapeutics and a completely rewritten chapter on the state of the art of experimental therapeutics and clinical trials.

Un guide adapté à la pratique courante pour établir un diagnostic positif de chaque type de malaise, même le plus rare. Il est courant d'avoir à prendre en charge un patient ayant subi un malaise avec ou sans perte de connaissance, situation où il est difficile de poser un diagnostic tant les causes possibles sont nombreuses. Cet ouvrage répertorie toutes les causes possibles des malaises et pertes de connaissances transitoires de l'adulte, en les classant par grande typologie : traumatismes crâniens, pertes de connaissance toxiques, cérébro-vasculaires, cardio-vasculaires, métaboliques, psychogènes, épileptiques, sans oublier les pertes de connaissance liées à une hypertension intracrânienne, aux migraines, aux accès de sommeil, aux drop-attacks et aux expériences de mort imminente. Nulle possibilité n'est oubliée. Chaque possibilité causale est présentée de façon méthodique : rappels étiologiques et physiologiques, anamnèse détaillée du malaise, examen physique, pose du diagnostic, pièges à éviter. Il s'agit de donner au lecteur une méthodologie opératoire appuyée sur la clinique, et particulièrement sur l'interrogatoire, afin de poser un diagnostic positif de chaque type de malaise - même le plus rare - pour qu'aucun ne soit rejeté dans la catégorie floue du diagnostic d'élimination. Devant la riche diversité des symptômes associés aux malaises, nous souhaitons que cet ouvrage serve les praticiens et les guide sans faillir.

MRI can play an important role in identifying and localizing epileptogenic foci. This book aims to provide the clinical and imaging information required in order to decide whether an MRI scan is appropriate and whether it is likely to be sufficient to detect a lesion. The first part of the book presents background information on epilepsy patients and explains how to perform an MRI examination. Detailed attention is paid to functional MRI and post-processing, and the examination of subcategories of patients is also discussed. The second part of the book then documents the MRI findings obtained in the full range of epileptogenic lesions with the aid of high-quality images. Throughout, emphasis is placed on guiding the reader in the correct interpretation of the imaging findings. Both radiologists and referring physicians will find this book to be an indispensable guide to the optimal use of MRI in epilepsy.

Parasomnias are phenomena that occur exclusively during sleep or are exacerbated during sleep/wake transition. These disorders are known to contribute towards impaired quality of life, disturbed and non-restorative sleep, risk for injuries to self and others and often associated with other medical, neurological and psychiatric disorders. Advances in sleep medicine have revealed a high prevalence of parasomnias across all ages. With the growing interest for diagnosing and management of parasomnias in sleep medicine, a practical guide to parasomnias is greatly needed.Parasomnias provides a comprehensive review of epidemiology, pathophysiology, clinical characteristics, diagnostic evaluation and treatment of parasomnias across the patient's life span. Written by experts, each chapter integrates the latest research and clinical data. In addition, several chapters address medico-legal and forensic aspects of parasomnias.Clinicians and researchers with an interest in sleep medicine will find Parasomnias to not only be an important contribution to the literature, but an indispensible guide to identifying, understanding and treating this disorder.

Im vorliegenden Band wird die vielschichtige klinische Problematik von Herzmuskel- und Perikarderkrankungen sowie der Herz- und Perikardtumoren ausführlich dargestellt. Die Klassifizierung der Myokarderkrankungen folgt der derzeitigen Einteilung in (primäre) Kardiomyopathien und (sekundäre) spezifische Herzmuskelerkrankungen. Besondere Schwerpunkte liegen in der Darstellung der Immunpathogenese myokardialer Erkrankungen und der Anwendung neuer diagnostischer Verfahren wie Myokardbiopsie und bildgebender Verfahren. Breiter Raum ist in der Therapie neben den klassischen Methoden der Anwendung von Vasodilatantien, Betablockern, Kalziumantagonisten und Immunsuppressiva gewidmet. Erstmalig werden Myokardalterationen bei genetischen Erkrankungen und neurologischen Systemkrankheiten zusammenfassend dargestellt. Von besonderer Aktualität sind toxische Einwirkungen auf das Myokard sowie Strahlenschäden. Internisten und allen kardiologisch interessierten Ärzten vermittelt dieser Band umfassende Basisinformationen über die diagnostischen und therapeutischen Fortschritte unter Einschluß der jeweils aktuellen Probleme in Pathogenese und Therapie.

Celem autorów publikacji było dostarczenie czytelnikowi krótkich omówieni różnych rodzajów zespołów padaczkowych i zilustrowanie każdego z nich rzeczywistymi przypadkami klinicznymi z towarzyszącymi zapisami wideo-EEG. Chociaż podręcznik napisano przede wszystkim z myślą o epileptologach i rezydentach neurologii, może przydać się także technikom EEG, pielęgniarkom, neurochirurgom, psychologom, tym bardziej że współautorzy rozdziałów reprezentują szeroki zakres specjalności medycznych, wchodzących w skład typowego zespołu zajmującego się chorymi na padaczkę. Książka zaopatrzona kodem dostępu do strony expertconsult.com W komplecie dwie płyty DVD

The field of movement disorders is one of the key subspecialty areas in clinical neurology, and understanding of the relevant conditions can often be difficult. The scope of this area requires a wide knowledge base, and clinicians might, in the course of a single clinic, need to recall the differential of Huntington's-like disorders, the gene implicated in dopa-responsive dystonia, and compare a case of suspected neuroacanthocytosis with a'classical'case. Part of the accessible Oxford Textbooks in Clinical Neurology series, this volume covers the basic science and clinical concepts underlying the movement disorders, as well as the diagnosis and treatment of individual hypokinetic and hyperkinetic movement disorders. Specifically written to aid understanding and treatment of a wide range of movement disorders, this textbook includes a useful section covering miscellaneous causes of disordered movement, which are routinely encountered by neurologists. It is also supplemented with illustrative video clips that can be accessed through the concurrent online edition. Although firmly rooted in evidence-based management approaches, the authors included their own top tips and experience on the management of difficult cases where no current guidance exists, engaging the reader and providing a better feel for handling real-world clinical problems. The Oxford Textbook of Movement Disorders is an indispensable reference for neurologists and senior trainees in neurology, as well as any physicians advising people with movement disorders.

We are most pleased to present Volume 3 of Developmental-Behavioral Disorders: Selected Topics, designed to serve as a companion for standard reference textbooks that address cogent issues in developmental pedi­ atrics. Periodic publications such as Selected Topics and theme-related articles, as well as continuing education programs, attempt to supple­ ment in a timely fashion the rapidly changing knowledge base in devel­ opmental-behavioral pediatrics. These media are important as forums for enhancing the quality of clinical practice, teaching skills, and re­ search activities. The need is critical for periodically disseminating and updating information about issues in developmental medicine, in as much as this field of study continues to expand at a meteoric pace. During the past several decades, developmental medicine has been recognized as a defined subspecialty in pediatrics. The spectrum of problems encompassed by this discipline is relatively broad and at times clinically overwhelming. The ultimate goal of preventing delays, disor­ ders, and/or dysfunctions from becoming chronic handicapping condi­ tions has, by volume per se, created clinical dilemmas for pediatric health care providers. There are numerous facets of providing efficient and effective care, which in the field of developmental-behavioral pediatrics are often exaggerated impediments to the delivery of services by pri­ mary health care specialists, e. g., time, clinical skills, need for inter­ disciplinary management, medical-legal responsibilities, financial reim­ bursements. These issues, as well as clinical problems, are still very much part of the information base to be disseminated to concerned pro­ fessionals.

For decades, Emery and Rimoin's Principles and Practice of Medical Genetics has provided the ultimate source for practicing clinicians to learn how the study of genetics can be integrated into practice. With almost 5,000 pages of detailed coverage, this fully online sixth edition of the classic reference adds the latest information on prenatal diagnosis, genetic screening, genetic counseling, and treatment strategies to complete its coverage of the growing field for medical students, residents and physicians involved in the care of patients with genetic conditions. Clinically oriented information is supported by expanded sections on basic principles of genetics, research approaches, and analytics to embrace the evolving population of students, researchers, and practitioners who are integrating their work to provide advanced diagnosis, prevention and treatment of human disease. With advances in high-throughput technologies propelling the closer integration of lab and clinical work, this edition bridges the gap between high-level molecular genetics and clinical application. - Features 174 review-length contributions that encompass traditional and new areas of the field - including in cancer genetics, genomic technologies, and molecular assays - Provides many thousands of pertinent literature references guiding the reader in identifying related topics - Fully illustrated with hundreds of color images, supporting identification, concept illustration and method processing

At a significant point in the life of an individual or an organization, it is normal that one reminisces and savors the qualities of the past. In doing so, one may learn of facts and factors that explain the present and even project into the future. So it is with the Second World Congress of Pediatric Cardiology. We can at this time avail ourselves of a panoramic view, not only of the present but also an exposure of the past, and a glimpse of what lies ahead. When Dr. Jane Somerville and Professor Fergus Macartney proposed developing a World Congress of Pediatric Cardiology, the factors of vision and imagina­ tion were driving forces. These, with the aid both of hard work and dedication to purpose, led to the being of the First Congress. The major success of that meeting was not without premonitory concerns as to the width of multinational acceptance of a concept that Pediatric Cardiol­ ogy had come of age and a firm foundation for academic interchange existed. The Congress showed that much had been learned, and that there were many eager to share of accumulated knowledge and reflections of skills with the very many hundreds who came to the First Congress. The responses to the First Congress were so enthusiastic that the idea of having a continuum of World Congresses of Pediatric Cardiology was widely accepted. I do not know that the subject was even debated after the First Congress went into session.

Updated and expanded with nine additional chapters, Movement Disorder Emergencies: Diagnosis and Treatment, Second Edition is an indispensable resource for general neurologists, specialists, fellows, and residents eager to improve their approach toward the patient with a movement disorder emergency. In this comprehensive second edition, prominent neurologists from around the world logically and systematically review the major movement disorder emergencies, instructing the reader on how optimally to recognize and manage these problems. The authors cover a broad range of disorders, including acute dystonic reactions, neuroleptic malignant syndrome, startle syndromes, tic emergencies, and others; and they stress the importance of certain obvious diagnoses such as Wilson's disease, dopa-responsive dystonia, and Whipple's disease, in which delayed diagnosis in less emergent situations can lead to slowly evolving and often irreversible neurologic damage with tragic consequences. In addition, nine topics not covered in the first edition are provided, including genetic counseling and testing crises, suicide risk, psychogenic movement disorders, and others. Patient vignettes at the beginning of each chapter focus the reader's attention and highlight the urgency of the problem. Since astute clinical diagnosis of many movement disorders is still largely dependent on visual pattern recognition in the clinic, an accompanying online collection of physician-patient vignettes illustrates virtually all of the movement disorders described in the text. Importantly, the authors also discuss a range of new treatment paradigms that have emerged since publication of the first edition, especially deep brain stimulation. Authoritative and a leading text in the field, Movement Disorder Emergencies: Diagnosis and Treatment, Second Edition is an established, practical reference that continues to achieve excellence in the field of diagnosis and management of movement disorder emergencies.

This fifth edition of the Autonomic Failure covers the many recent advances made in our understanding of the autonomic nervous system. There are numerous new chapters and extensive revisions of all other contributions. This volume makes diagnosis increasingly precise by fully evaluating the underlying anatomical and functional deficits, thereby allowing more effective treatment. It continues to provide a rational guide to aid in the recognition and management of autonomic disorders for practitioners from a variety of fields, including neurology, cardiology, geriatric medicine, diabetology, and internal medicine.

I was gratified by the most favorable reception and wide usage received by the first edition of this book. A decade seems to be a short period for a book on pathology, and yet it witnessed many important changes of concepts, along with a formidable growth of knowledge. The second edition required extensive reorganization. There are new chapters on mitochondriopathies, on peroxisomal diseases and on spongy myelino­ pathies. Major revisions and new additions were necessary in many chapters, for instance those on the dysplasias of the cerebral and of the cerebellar hemispheres, which were largely reorganized. The chapters on perinatal pathology were reordered and reorganized to give a more logical sequence of prenatal, perinatal and postnatal lesions. The entire text was worked over for brevity. A wealth of new references was added with the. aim of staying abreast with the literature up to summer 1988. All refer­ ences were double checked for errors. My gratitude goes to Mrs. Gisela Ropte and Mrs. Cynthia Bunker for their untiring, diligent help. As a result, this second edition is an essentially rewritten text. Advance in the prevention of human suffering is based on a thorough understand­ ing of the nature of disease. I hope that this text will continue to be of service in this behalf. Perhaps it may also reflect and foster the intellectual curiosity which makes the'reading of brains'so interesting an occupation. Gottingen, 1989 Reinhard L.

Genomics and Health in the Developing World provides detailed and comprehensive coverage of population structures, human genomics, and genome variation--with particular emphasis on medical and health issues--in the emerging economies and countries of the developing world. With sections dedicated to fundamtals of genetics and genomics, epidemiology of human disease, biomarkers, comparative genomics, developments in translational genomic medicine, current and future health strategies related to genetic disease, and pertinent legislative and social factors, this volume highlights the importance of utilizing genetics/genomics knowledge to promote and achieve optimal health in the developing world. Grouped by geographic region, the chapters in this volume address: - Inherited disorders in the developing world, including a thorough look at genetic disorders in minority groups of every continent - The progress of diagnostic laboratory genetic testing, prenatal screening, and genetic counseling worldwide - Rising ethical and legal concerns of medical genetics in the developing world - Social, cultural, and religious issues related to genetic diseases across continents Both timely and vastly informative, this book is a unique and comprehensive resource for genetists, clinicians, and public health professionals interested in the social, ethical, economic, and legal matters associated with medical genetics in the developing world.

In the last few years, derivatives of L-carnitine, such as acetyl-L-carnitine and propionyl-L-carnitine, have been made available to doctors for treatment of specific pathologies. The effects of this family of related carnitine compounds on cardiovascular systems and diseases constitute the major issue addressed in this volume. Although several books on carnitine have been published, a treatise focusing on experimental and clinical aspects of the carnitine family and cardiovascular diseases was lacking. The present book provides the reader with a concise update in this field. The information collected from experts on various aspects of the fascinating compound, carnitine, will be useful for both clinicians and basic scientists.

This clinically orientated text by an international group of experts is the first definitive reference book on disorders of the cerebellum in children. It presents a wealth of practical clinical experience backed up by a strong scientific basis for the information and guidance given. The first part sets out the theoretical underpinnings of cerebellar disorders. This is followed by sections on clinical conditions grouped according to common characteristics such as aetiology and symptomatology. The descriptions of the clinical conditions each systematically cover, as appropriate, epidemiology, prevalence, diagnostic criteria, clinical features (including course and prognosis), pathophysiology, genetics, investigations, differential diagnosis, and management and treatment.

Neurology in Clinical Practice brings you the most current clinical neurology through a comprehensive text, detailed color images, and video demonstrations. Drs. Daroff, Fenichel, Jankovic and Mazziotta, along with more than 150 expert contributors, present coverage of interventional neuroradiology, neurointensive care, prion diseases and their diagnoses, neurogenetics, and many other new developments. Online at www.expertconsult.com, you'll have access to a downloadable image library, videos, and the fully searchable text for the dynamic, multimedia content you need to apply the latest approaches in diagnosis and management.Find answers easily through an intuitive organization by both symptom and grouping of diseases that mirrors the way you practice. Diagnose and manage the full range of neurological disorders with authoritative and up-to-date guidance. Refer to key information at-a-glance through a full-color design and layout that makes the book easier to consult. Access the fully searchable text online at www.expertconsult.com, along with downloadable images, video demonstrations, and reference updates. Stay current on advances in interventional neuroradiology, neurointensive care, prion diseases, neurogenetics, and more. See exactly how neurological disorders present with online videos of EEG and seizures, movement disorders, EMG, cranial neuropathies, disorders of upper and lower motor neurons. Keep up with developments in the field through significant revisions to the text, including brand-new chapters on neuromodulation and psychogenic disorders and a completely overhauled neuroimaging section. Tap into the expertise of more than 150 leading neurologists-50 new to this edition.

The rhythm of the heart, its normal functioning and pathologic disturbances, has been a favored subject of investigation by clinical and basic scientists in recent decades. This heightened interest and attention was stimulated by the somber and surprising revelations from epidemiologists and pathologists of the enormity of the number of sudden arrhythmic deaths in the Western world, and the concurrent advancement of technology for recording and control of electrical activity of the heart. Technological advancements have included the recording of intracellular potentials from cardiac cells, the recording of intracardiac extra­ cellular potentials generated by specific cardiac structures, simultaneous record­ ings from numerous sites with computer processing for spatial mapping of activa­ tion or potential variations with time, high gain, high resolution recordings with signal averaging for detecting potentials of low amplitudes, complex stimulation protocols, various high energy stimulation modes, intracellular voltage control of multicellular preparations and single cardiac cells, and the isolation of single cardiac cells for electrophysiological study. The interest and technology have produced an increasing bounty of information and understanding, acceptable solutions to some clinical problems, and definite progress toward solutions to other problems. Progress in research in electrophysiology and arrhythmias has been reviewed and highlighted in various meetings and books in recent years. Because the body of information has become so large, general overviews of the field have neces­ sarily been superficial in certain aspects or have contained gaps.

The Encyclopedia of Clinical Child and Pediatric children, adolescents, and their families with a focus on Psychology is intended to be an authoritative and com­ understanding, preventing, diagnosing and treating psychological, cognitive, emotional, developmental, prehensive resource that provides up-to-date informa­ tion on a broad array of problems and issues related to behavioral, and family problems of children. Of partic­ children, adolescents, and their families as defined by ular importance to clinical child psychologists is an the fields of clinical child and pediatric psychology. It is understanding of the basic psychological needs of children and the social contexts that influence child designed to be of particular interest and use to layper­ sons, parents and grandparents, and undergraduate and development and adjustment. Thus, typical and atypical graduate students in training, as well as diverse medical development and the impact of life stresses are of and mental health professionals who live with and/or concern for the clinical child psychologist. work with young persons but who have limited infor­ The specialty of pediatric psychology, like clinical mation on a particular topics. Inasmuch as the scope of child psychology, is interested in the psychological needs clinical child and pediatric psychology is extensive, a of children but the focus is on the psychosocial aspects range of topics is included that cover typical and atyp­ of illness.

Praise for the First Edition:--'This instructive text will serve as a reference for specialists in the care of patients with these disorders and for general neurologists and others to whom a patient with Stiff-Person syndrome or Wilson's disease is a rarity. The emphasis on the neuroscientific basis for the disorders presented and their study and treatment will also make the book academically useful....This excellent and timely book will provide a valuable resource for those involved in the academic and clinical aspects of movement disorders. 5 STARS!'--Doody's Review Service

Published since 1959, International Review of Neurobiology is a well-known series appealing to neuroscientists, clinicians, psychologists, physiologists, and pharmacologists. Led by an internationally renowned editorial board, this important serial publishes both eclectic volumes made up of timely reviews and thematic volumes that focus on recent progress in a specific area of neurobiology research. This volume reviews existing theories and current research surrounding the movement disorder Dyskinesia. - Leading authors review state-of-the-art in their field of investigation and provide their views and perspectives for future research - Chapters are extensively referenced to provide readers with a comprehensive list of resources on the topics covered - All chapters include comprehensive background information and are written in a clear form that is also accessible to the non-specialist

750 EEG tracings provide the visual assistance you need to diagnose pediatric seizure activity Atlas of Pediatric EEG will prove to be an essential visual reference to for both the novice and experienced neurologist. For those new to the field, it will help develop the pattern recognition skills necessary to diagnose pediatric seizure activity. For experienced neurologists, it provides a working collection of known patterns to which they can compare their own tracings. Atlas of Pediatric EEG features a full-color presentation, easy-to-read bulleted chapter text, and detailed legends under each tracing that provide a full description and diagnosis of what is seen in the tracing. Chapters also contain case examples that add clinical relevance to the tracings. This unique atlas covers every type of seizure, both epileptic and non-epileptic and divided into nine chapters: Normal and Benign Varients Artifacts Newborn Focal Nonepileptoform Activity Generalized Noneplileptiform Activity ICU Epileptic Encephalopathy Generalized Epilepsy Focal Epilepsy Also included is a companion DVD containing 190 video clips to assist you in learning how to interpret video-EEG, which is rapidly becoming the most common modality for EEG.

This unique book provides an up-to-date reference source for university students, clinicians, researchers and policymakers concerned with the field of middle ear assessment. By drawing on the knowledge and expertise of experts from various countries, not only at the research level but also at the clinical level, this text represents as the only book that details the entire collection of audiological tests of middle ear function for young infants.

In this 3 volume collection focusing on glycomics, readers will appreciate how such discoveries were made and how such methods can be applied for readers'own research efforts - Each chapter has been designed so that enough scientific background will be given in each chapter for further development of methods by readers themselves - Useful for all levels of scientists starting from the last years of colleges, graduate students, postdoctoral fellows to professors and to all levels of scientists in research institutes including industry

Advances in genomic and proteomic profiling of disease have transformed the field of molecular diagnostics, thus leading the way for a major revolution in clinical practice. While the range of tests for disease detection and staging is rapidly expanding, many physicians lack the knowledge required to determine which tests to order and how to interpret results. Molecular Diagnostics provides a complete guide to the use and interpretation of molecular testing in the clinical arena. No other available resource offers this emphasis, comprehensive scope, and practical utility in the clinical setting. - Serves as the definitivereference for molecular pathologists worldwide - Covers a variety of molecular techniques including next generation sequencing, tumor somatic cell genotyping, infectious and genetic disease tecting, and pharmacogenetics - Discusses in the detail issues concerning quality assurance, regulation, ethics, and future directions for the science

Two-volume set consists of: Pediatric Neuroradiology Brain Pediatric Neuroradiology Head and Neck Spine TORT-title-Brain. indd TORT-title-Brain. indd II II 02. 02. 2005 02. 02. 2005 07:46:44 07:46:44 Preface III Pediatric Neuroradiology Brain Paolo Tortori-Donati and Andrea Rossi In collaboration with Roberta Biancheri Foreword by Charles Raybaud With 1635 Figures in 4519 Separate Illustrations, 207 in Color and 202 Tables 123 TORT-title-Brain. indd TORT-title-Brain. indd III III 02. 02. 2005 02. 02. 2005 07:46:44 07:46:44 IV Preface Paolo Tortori-Donati, MD Head, Department of Pediatric Neuroradiology G. Gaslini Children's Research Hospital, Genoa, Italy Andrea Rossi, MD Senior Staff Neuroradiologist Department of Pediatric Neuroradiology G. Gaslini Children's Research Hospital, Genoa, Italy Roberta Biancheri, MD, PhD Consultant Pediatric Neurologist Department of Pediatric Neuroradiology G. Gaslini Children's Research Hospital, Genoa, Italy Library of Congress Control Number: 2004118036 ISBN 3-540-41077-5 Springer Berlin Heidelberg New York This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, speci? cally the rights of translation, reprinting, reuse of illustrations, recitations, broadcasting, reproduction on micro? lm or in any other way, and storage in data banks. Duplication of this publication or parts thereof is permitted only under the provisions of the German Copyright Law of September 9, 1965, in its current version, and permission for use must always be obtained from Springer-Verlag. Violations are liable for prosecution under the German Copyright Law. Springer is part of Springer Science+Business Media http//www. springeronline.

A disorder which affects 4 percent of children and 1-2 percent of the general population, epilepsy is one of the most common neurological disorders. The original edition of C.P. Panayiotopoulos'guide proved to be the only one of its kind, covering many important aspects of diagnosis and treatment. Due to the continued advances being made in the subject, a second edition is now due. The text includes detailed diagnostics, concise descriptions of syndromes, a wealth of practical tips, and is supported by useful tables to enable clear diagnoses. Building on the sell-out success of the first edition this thorough revision reflects the latest report of the ILAE classification core group and the significant progress made in the diagnosis, classification and treatment of the epilepsies. This new edition is indispensable reading for all those who care for patients with epileptic seizures.

The new edition of this popular reference continues to present the critical answers you need to address your most difficult clinical challenges. It explores the latest advances in the treatment of pediatric disease, equipping you with unparalleled coverage of immunology, epidemiology, public health, preventive medicine, clinical manifestations, diagnosis, treatment, and much more. Stay on track with updates, including para- and post-infectious demyelinating diseases of the central nervous system, antibiotic resistance, and molecular techniques in laboratory diagnosis - just to name a few-enable you to put the very latest knowledge to work for your patients.Offers unmatched, comprehensive coverage of immunology, epidemiology, public health, preventive medicine, clinical manifestations, diagnosis, treatment, and much more, for convenient consultation in one comprehensive reference.Organizes information both by organ system and by etiologic microorganism, enabling you to find the answers you need quickly by approaching any topic from either direction. Offers extensive updates on topics including para- and post-infectious demyelinating diseases of the central nervous system, antibiotic resistance, molecular techniques in laboratory diagnosis, fever without source and fever of unknown origin, infections associated with transplantation, human immunodeficiency disease, immunomodulating agents and passive immunizations-and many, many more-to help you achieve better patient management outcomes by implementing the very latest knowledge.Features the contributions of new authors-representing nearly one-third of the text-who offer you fresh insights on today's practice.

This authoritative textbook offers in-depth coverage of all aspects of molecular pathology practice and embodies the current standard in molecular testing. Since the successful first edition, new sections have been added on pharmacogenetics and genomics, while other sections have been revised and updated to reflect the rapid advances in the field. The result is a superb reference that encompasses molecular biology basics, genetics, inherited cancers, solid tumors, neoplastic hematopathology, infectious diseases, identity testing, HLA typing, laboratory management, genomics and proteomics. Throughout the text, emphasis is placed on the molecular variations being detected, the clinical usefulness of the tests and important clinical and laboratory issues.The second edition of Molecular Pathology in Clinical Practice will be an invaluable source of information for all practicing molecular pathologists and will also be of utility for other pathologists, clinical colleaguesand trainees.

This new edition fills an important gap in the literature by providing a concise treatment of pediatric neurology that focuses on the most commonly seen diseases with clinical guidelines that help today« busy practitioner find answers quickly. The book is divided into three sections starting with the tools required for a pediatric neurologic evaluation, then moving through classic disease states and disorders with the last section focusing on approaches to key clinical problems in children and adolescents. Each section is edited by the key opinion leaders in the field with dynamic features that get to the information quickly including: Tools for diagnosis Chapter opening outlines Disease'Features'tables'Pearls and Perils'boxes'Consider Consultation When«'boxes Selected annotated bibliographies Key Clinical Questions

Neurological disorders in children are common: families want to know what is wrong, why it happened, and whether it will happen again. Management and treatment depend on establishing the diagnosis, which usually requires investigations, but the number of possible neurological investigations is now very large indeed, and uncritical investigations may be seriously misleading and often costly. This book, based on the authors'vast combined personal experience, gives practical guidance on how to target any specific condition with the minimum of tests. Part 1 is a brief distillation of advice on clinical history and examination, introducing the problems that beset those who deal with neurological disorders in childhood. Part 2 outlines the investigations available to solve these diagnostic problems. Part 3 takes a problem-oriented approach to the most appropriate investigations in the various clinical scenarios that may present to the practitioner: the starting point is the patient's presentation, not the diagnosis. Throughout, vignettes of real cases help to illustrate the use of the tests in different clinical situations. The authors co-wrote the hugely popular Handbook of Neurological Investigations (‘the Blue Book') 20 years ago. This new book, while closely following the highly successful approach of the earlier title, is thoroughly up to date, with fresh material, new case vignettes, and additional investigations covered.

The premier textbook of clinical child neurology. Comprehensive descriptions of the neurological disorders of the neonate, infant, child and adolescent. Third edition comprehensively revised and updated.

Huntington's disease (HD) is an inherited progressive neurodegenerative disorder. Although onset of Huntington's Disease usually occurs in adulthood, the age of onset of the condition is extremely variable with approximately 5-10% of cases having an onset of less than 20 years, or Juvenile Huntington's Disease (JHD). While JHD shares many of the clinical features of adult HD (e.g., chorea and personality disorders), patients with JHD often experience additional problems including seizures, dystonia and Parkinsonism. Diagnosis in patients with JHD is often delayed because of the failure of clinicians to recognise the characteristic features of the condition. While several textbooks have been published on HD, no books have been published to date focussing solely on the juvenile onset form of the disease. This book summarises, for the first time, the clinical and scientific knowledge available on JHD. It also collects together accounts from families affected by the condition, putting the clinical and scientific chapters into context. Edited by members of the working group on Juvenile Huntington's Disease within the European Huntington Disease Network (EHDN), this book forms the first comprehensive text on JHD and is of interest to neurologists, geneticists, academic/research scientists and other healthcare professionals.

This book is written for those who evaluate the many quirks and behaviors encountered in the lives of developing children. The number of important pathologic conditions that befall this age group is staggering. However, equally staggering is the number of episodic and often benign or developmentally programmed paroxysmal events encountered in this population. The nature of these episodic events in many circumstances leads to their erroneous identification as epileptic in nature. This book serves as a compendium of those episodically manifest entities often uniquely observed in childhood. The book is organized with some foundations in terminology and an in depth analysis of epidemiology of many of these disorders. As best possible, epidemiologic data are compiled with incidence and prevalence figures provided for comparisons. Discussion on the deductive approach to their clinical assessment is provided along with emphasis on the diagnostic clues contained within the descriptive and observational information provided during clinical evaluation. A categorization of the differential features and ages of presentation are provided as a means to help direct logical diagnostic considerations. Subsequent chapters are organized by major topic (i.e.) Syncopes, Sleep Phenomena, Somatization, Factitious Illness, Movement Disorders, and Migraine Syndromes. Each chapter provides discourse on the general principles of underlying pathophysiologic mechanisms and current knowledge about the chapter topic under discussion. Specific clinical entities encompassed within the general topic are then discussed individually with particular attention to their clinical descriptions and presentations, natural history, particular pathophysiology, genetics, pertinent laboratory, and treatments. The text is amplified by pertinent tables highlighting diagnostic criteria where this applies and reference information where needed. The text is generously referenced to encompass the most relevant information available.

This book provides a definitive reference work on the developing brain, from conception through the first year of life. Its purpose is to provide the range of specialists involved in the management of the fetus and the neonate with the latest information on the developmental neurology and pathology of the developing central nervous system, so that they can provide prompt and informed treatment of neurological disability – the most feared complication of pregnancy and the early months of the life of the newborn child.A cross-specialty book: brings together the work of specialists on the immature brain from the fields of obstetrics, paediatrics and neurosurgery.An international book: edited and written by an international team of experts.Highly illustrated: includes over 800 images and line drawings that capture the latest investigative techniques.Focused on treatment: key points boxes pick out the most important information for the busy specialist.Evidence-based information: extensively referenced with the latest and most important papers and articles.Includes the latest ultrasound techniques – to reflect the massive improvements since the last edition.Medico-legal issues highlighted – in recognition of the role of specialists as expert witnesses.Covers the latest MR techniques: fast sequences for fetal brain imaging, DWI, tractography.