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This monograph describes the progress in neuropathological HD research made during the last century, the neuropathological hallmarks of HD and their pathogenic relevance. Starting with the initial descriptions of the progressive degeneration of the striatum as one of the key events in HD, the worldwide practiced Vonsattel HD grading system of striatal neurodegeneration will be outlined. Correlating neuropathological data with results on the functional neuroanatomy of the human brain, subsequent chapters will highlight recent HD findings: the neuronal loss in the cerebral neo-and allocortex, the neurodegeneration of select thalamic nuclei, the affection of the cerebellar cortex and nuclei, the involvement of select brainstem nuclei, as well as the pathophysiological relevance of these pathologies for the clinical picture of HD. Finally, the potential pathophysiological role of neuronal huntingtin aggregations and the most important and enduring challenges of neuropathological HD research are discussed.
It is a pleasure to send Dr. Hayden's monograph on its way to as yet unknown but hopefully widespread destinations with all the valedictions a Foreword may contain. Since I met the author in Cape Town in 1978 I have been struck, on numerous occasions, by the fortuitous combination of an inquisitive mind, a creative drive, a sharp awareness of the historical and social setting of phenomena, and a solid discipline which his personality displays behind a good-natured laugh. If a tree is known by its fruits, both Dr Hayden's PhD thesis and the present monograph afford quite an insight into the auctor intellectualis. The amalgamation of the terrible mise ry behind scientifIc facts and the elegantly artistic presentation of this book will leave none of its readers unperturbed. It reminds me of Nietzsche's 'Denn das Schöne ist nichts als des Schrecklichen Anfang, und wir bewundern es so weil es gelassen verschmäht uns zu zerstören' (Beauty is but Horror's beginning, and we admire it because it resignedly spurns to destroy us). The book is a denial, a testimony against Juvenal's spurious 'Sternmata quid faciunt ... ' (of what value are pedigrees). For it is the very genetical prolongation of misery over the centuries that brought Huntington's chorea to South Africa, Australia and the USA from the shores of sea-faring seventeenth-century England and Holland.
This very well-received book, now in its second edition, equips the radiologist with the information needed in order to diagnose internal medicine disorders and their complications from the radiological perspective. It offers an easy-to-consult tool that documents the most common and most important radiological signs of a wide range of diseases, across diverse specialties, with the aid of an excellent gallery of images and illustrations. Compared with the first edition, numerous additions and updates have been made, with coverage of additional disorders and inclusion of many new images. Entirely new chapters focus on occupational medicine and toxicology imaging, chiropractic medicine, and energy and quantum medicine. Internal Medicine – An Illustrated Radiological Guide puts the radiologist in the internal medicine physician’s shoes. It teaches radiologists how to think in terms of disease progression and complications, explains where to look for and to image these complications, and identifies the best modalities for reaching a diagnosis. It will also benefit internal medicine physicians by clarifying the help that radiology can offer them and assisting in the choice of investigation for diagnostic confirmation.
Huntington's Disease is a genetically inherited condition, the result of severe nerve-cell damage in the brain. Due to the recent identification of the gene involved, and the debilitating nature of the disease, a great many more people are now affected either directly or indirectly (familiesand carers) by this condition. The majority of people develop the disease between the ages of 35 and 55, so for those that are aware of a genetic inheritance, there are enormous problems to confront - should you carry on life as normal? Should you start a family? In this, the first book onHuntington's disease written for sufferers and their families, advice is given on living with this disabling illness. Written as much for carers as for the patients themselves, the book aims to answer some of the questions that both sufferer and carer might have. With the identification of theresponsible gene, genetic counselling is now available for those at risk of developing the disease. Though some may wish not to use these services, the book clearly explains the role of the counsellor, and what help is additionally available from the various patient organisations worldwide.
Hyperkinetic movement disorders comprise a range of diseases characterized by unwanted and uncontrollable, or poorly controllable, involuntary movements. The phenomenology of these disorders is quite variable encompassing chorea, tremor, dystonia, myoclonus, tics, other dyskinesias, jerks and shakes. Discerning the underlying condition can be very difficult given the range and variability of symptoms. But recognizing the phenomenology and understanding the pathophysiology are essential to ensure appropriate treatment. Hyperkinetic Movement Disorders provides a clinical pathway for effective diagnosis and management of these disorders. The stellar international cast of authors distils the evidence so you can apply it into your practice. The judicious use of diagnostic criteria algorithms rating scales management guidelines Provides a robust framework for clear patient management. Throughout the text, QR codes* provide smartphone access to case-study videos of hyperkinetic symptoms. Purchase includes an enhanced Wiley Desktop Edition.* This is an interactive digital version featuring: all text and images in fully searchable form integrated videos of presentations View a sample video: www.wiley.com/go/albanese highlighting and note taking facilities book marking linking to additional references Hyperkinetic Movement Disorders provides you with the essential visual and practical tools you need to effectively diagnose and treat your patients. *Full instructions for using QR codes and for downloading your digital Wiley DeskTop Edition are inside the book.
"The Differential Diagnosis of Chorea provides a comprehensive account of the various neurological conditions, both genetic and acquired, that lead to this involuntary movement disorder.
This book represents the final work of the late Professor C. David Marsden, who was the most influential figure in the field of movement disorders, in terms of his contributions to both research and clinical practice, in the modern era. It was conceived and written by David Marsden and his colleague at the Institute of Neurology, Prof. Ivan Donaldson. It was their intention that this would be the most comprehensive book on movement disorders and also that it would serve as the 'clinical Bible' for the management of these conditions. It provides a masterly survey of the entire topic, which has been made possible only by vast laboratory and bedside experience. Marsden's Book of Movement Disorders covers the full breadth of movement disorders, from the underlying anatomy and understanding of basal ganglia function to the diagnosis and management of specific movement disorders, including the more common conditions such as Parkinson's Disease through to rare, and very rare conditions such as Niemann-Pick disease. Chapters follow a structured format with historical overviews, definitions, clinical features, differential diagnosis, investigations and treatment covered in a structured way. It is extensively illustrated with many original photographs and diagrams of historical significance. Among these illustrations are still images of some original film clips of some of Dr. Marsden's patients published here for the first time. Comprehensively referenced and updated by experts from the Institute of Neurology at Queen Square, this book is a valuable reference for, not just movement disorder specialists and researchers, but also for clinicians who care for patients with movement disorders.