Michael Swash - Böcker

Amyotrophic lateral sclerosis or motor neurone disease is one of the most debilitating and devastating of the neurological diseases. The only comprehensive textbook available on the topic, this completely reorganized and expanded new edition examines all aspects of ALS from pathology to patient care and provides the reader with a ready reference to help with the difficulties encountered in patient diagnosis and treatment. Undoubtedly an important work in the field, this book will be of great interest to clinical neurologists and trainees, and to all those concerned with the care of people with ALS.

During the last 20 years the development of enzyme histochemical techniques has contributed greatly to knowledge of muscle pathology. However, these and other new methods, such as electron microscopy and immunocytochemistry, have only relatively recently become gener- ally available for routine use in histopathology. Muscle biopsy is a long-established technique in clinical practice, having been introduced by Duchenne in 1868 (Arch. Gen. Med. , 11, 5-179). However, the needle method used by Duchenne was not generally adopted, although Shank and Hoagland described a similar technique in 1943 (Science, 98, 592). During this time muscle biopsies required a surgical procedure and this was a considerable disincentive to their use. It was not until Bergstrom (1962; Scand. J. Clin. Lab. Invest. , 14, Suppl. 68) and Edwards (1971; Lancet, ii, 593--6) developed a simple biopsy needle suitable for muscle work in connection with exercise physiology that the advantages of needle muscle biopsies came to be appreciated. Since then, muscle biopsies have become a relatively minor procedure.This has led to the increasing use of muscle biopsy in clinical practice, both for diagnosis and for assessing progress in repeated biopsies during the course of a disorder and its treatment. The full range of enzyme histochemical and ultrastructural histological techniques can be applied to these small biopsies and many of the older histological staining methods can also be used. This book is intended to serve as a practical guide in muscle pathology, particularly for histopathologists, and for those in training.

This text is intended to serve as a practical guide to muscle pathology, particularly for histopathologists and for those in training. In this edition, the authors have taken account of advances in classification and in histological techniques, as well as new knowledge of neuromuscular diseases that have emerged since the first edition was published in 1984. The book should continue to be of value as a practical guide in the diagnosis and understanding of these disorders, and should be available in every hospital histopathology laboratory in which muscle biopsies are examined. This book should be of interest to muscle pathologists, general histopathologists and trainees.

Natural history and treatment outcome of disease are of critical importance to patients, clinicians and health care providers across the full range of medical disciplines. In many specialities, such as neurology and neurosurgery, this sort of information can be hard to obtain in the absence of extensive outcome studies and agreed measures of outcome in different conditions. This volume seeks to inform about effective care and rehabilitation by providing a succinct review of whatever data is available for the majority of conditions encountered in clinical practice. Particular attention has been given to the evaluation of severity and outcome of each disorder and to advice on comparing risks and benefits of treatment. This resource will prove invaluable to all involved in informing patients of treatment options and making important cost efficient decisions on care. It should also be welcomed in the planning of clinical trials for potential therapies and as a reference in medicolegal spheres.

Natural history and treatment outcome of disease are of critical importance to patients, clinicians and health care providers across the full range of medical disciplines. In many specialities, such as neurology and neurosurgery, this sort of information can be hard to obtain in the absence of extensive outcome studies and agreed measures of outcome in different conditions. This volume seeks to inform about effective care and rehabilitation by providing a succinct review of whatever data is available for the majority of conditions encountered in clinical practice. Particular attention has been given to the evaluation of severity and outcome of each disorder and to advice on comparing risks and benefits of treatment. This resource will prove invaluable to all involved in informing patients of treatment options and making important cost efficient decisions on care. It should also be welcomed in the planning of clinical trials for potential therapies and as a reference in medicolegal spheres.

The development of specialty skills in medical and surgical practice in the late 19th and in the 20th century transformed medical practice. For the first time, a patient could visit a doctor with the expectation of an accurate diagnosis and effective treatment. Disease prevention became a realistic proposition. Traditional practice methods became obsolescent, but a yearning for generalist medical wisdom and respect continued until the 2nd World War disrupted progress. In the 19th century, the London Hospital was remarkably open to new ideas, and the Chairman of the Board, Viscount Knutsford, was a master fundraiser. Investment in novel facilities and staff, including the establishment of special departments, and consequent changes in clinical practice led to a growing national and international reputation in clinical practice and education. Specialty skills defined innovations, both in hospital and family practice. More recently, merging St Bartholomew's, the Royal London Hospital and other hospitals has reactivated the advance of specialism.

Here is the first book to provide a comprehensive overview of the clinical, pathological, and research aspects of motor neuron disease (MND).

Hughlings Jackson, the noted English neurologist, fathered many ideas that today still underlie our understanding of common clinical phenomena. This is a reappraisal of Jackson's work, both within its historical framework and in light of modern concepts of neurology. The approach is new, combining historical, clinical and basic scientific information in one synthesis on the organization and function of the nervous system. The concept of levels of function is addressed, specifically with regard to areas of brain function; and the hierarchical strategy is considered as part of the current concept of a distributed system of neurons. Clinicians and scientists alike will find much food for thought in this modern treatise of Jacksonian concepts.

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.