Neurologia

The term "muscular dystrophy" (MD) describes a group of primary genetic disorders of muscle that often have a distinctive and recognizable clinical p- notype, accompanied by characteristic, but frequently not pathognomonic, pathological features. Research into the molecular basis of the MDs by a c- bination of positional cloning and candidate gene analysis has provided the basis for a reclassification of these disorders, with genetic and protein data augmenting traditional clinically based nomenclature. These findings have brought insights into the molecular pathogenesis of MD, with an increasing number of potential pathways involved in arriving at a dystrophic phenotype. Some common themes can be recognized, however, including the involvement of five members of the dystrophin-associated complex (dystrophin and four sarcoglycans) in different types of MD, and the involvement of two nuclear envelope proteins in producing an Emery-Dreifuss MD phenotype. Other d- ease-associated genes appear to cause MD in a completely unrelated way, such as the involvement of calpain 3 in a form of limb-girdle muscular dystrophy. Section 1 of Muscular Dystrophy: Methods and Protocols reviews tra- tional strategies used to identify MDs. Meantime, techniques developed as a result of the research strategies described previously have become an integral part of the management of many patients with MD and their families, and these techniques are addressed in Sections 2 (DNA-based tests) and 3 (p- tein-based analyses). The continued effort to translate this enhanced und- standing into a molecular cure or treatment for MD is reviewed in Section 4.

Parkinson's disease (PD) is the second most common neurodegenerative disease. As the population of the industrialized world ages the impact of this disease on clinical neurology will expand. The growing literature regarding PD reflects this phenomenon. The time is now appropriate for a comprehensive text on PD that provides the reader with an up-to-date review on the multiple aspects of the disease and puts them into perspective. The scholarly contributions from many of the leading researchers in the field has made such a text a reality. This book will be useful to movement disorder specialists, general neurologists and neurology residents, gerontologists, neurosurgeons, neuropsychologists, and neuroscience researchers. The text provides both theoretical and practical approaches to PD.

The huge expansion of information available about MS, not least on the Internet, has put the individual with MS in a position to take increasing responsibility for her or his own care. There is thus a need for a comprehensive, readily accessible guide to the present therapeutic options, which will give the inquirer a balanced guide to the relative effectiveness of individual treatments. This book is designed to do just that. This bestselling title is a unique compendium of the most frequently used treatments for multiple sclerosis, an authoritative reference for all physicians, and a factual guide for patients who need information about therapies. This new edition has been extensively revised both in content and format from previous editions. Its organization into sections dealing with the treatment of acute exacerbations, disease-modifying therapies, symptom management, and alternative therapies makes the book much more accessible to the reader. The title has been changed from "Therapeutic Claims in Multiple Sclerosis" to reflect the major restructuring of this edition and to better describe the range and focus of the content.

The model for this work was the description of the physical world by mathemati- cal laws. It were always the simplest phenomena which were treated by this scientific method. Physicists studied simple motions inorder to find the mathematical laws. Astronomists observed the orbits of planets in order to find the laws of gravity. One of the simplest measurable phenomenon in the brain is the stimulus- response task. Suchtasks have beenknown since the lastcentury bypsychiatrists and psycholo- gists (v. Helmholtz). There existsa vast literature about the measurement and theory of simple reaction tasks and various choice reaction tasks, visualor auditory. They have beenmeasured and havebeendescribedmathematically.One of the firstmodels for the reaction times used a logarithmic function. But many intriguing questions remained open aboutreaction tasks especiallythe neural explanation ofthe findings. The new tool to investigate the neural structure of stimulus-response sequences was the computer. Now it was possible to measure the reaction times by using spe- cial programs, to compute the elementary times and the pathway structures from these reaction times, to evaluate the results statistically, to simulate the results, and to write this text. It was this instrument which permitted to save large amounts of data and evaluate them by special software written for this purpose. Thus it was possible to compute the time quanta and the pathways and to understand each re- action time as an integer multiple of this time quantum (plus a constant value).

This is a practical reference and guide for physicians, surgeons, therapists, orthotists, bioengineers and other health professionals involved in the management of a disabled individual with upper motor neurone syndrome and spasticity. It is a thorough review, covering all aspects of management from physiotherapy, seating, and orthoses to the use of drugs. It examines the neurophysiology of spasticity, measurement of outcome, and the newer treatment techniques, such as phenol and alcohol nerve blocks and botulinum toxin injections. More invasive procedures, including intrathecal baclofen and surgical intervention for more complex and severe spasticity, are covered in detail, and the final chapter looks closely at the management of spasticity in children.