Frontotemporal Disorders

Few people have heard of frontotemporal disorders, which lead to dementias that affect personality, behavior, language, and movement. These disorders are little known outside the circles of researchers, clinicians, patients, and caregivers who study and live with them. Although frontotemporal disorders remain puzzling in many ways, researchers are finding new clues that will help them solve this medical mystery and better understand other common dementias. The symptoms of frontotemporal disorders gradually rob people of basic abilities?thinking, talking, walking, and socializing?that most of us take for granted. They often strike people in the prime of life, when they are working and raising families. Families suffer, too, as they struggle to cope with the person's daily needs as well as changes in relationships and responsibilities.

Frontotemporal dementia (FTD) is a cruel disease, robbing patients of core human characteristics and wreaking havoc with relationships. Clinical and scientific interest in FTD and related disorders continues to grow rapidly, with major advances having occurred since this book's last publication. New clinical diagnostic criteria were published in 2011; new pathological discoveries have led to new diagnostic criteria; and major genetic discoveries have been made. This new edition covers these developments, providing the leading resource on FTD, PPA, PSP, CBD, FTD-ALS, and related disorders, now written by a more internationally representative group of authors than before. Providing an in-depth and expert synthesis of the status of our knowledge of FTD and related syndromes, the content includes chapters reviewing clinical, neuropsychiatric, neuropsychological, imaging, and other features of FTD and multidisciplinary approaches to patient management. Essential reading for specialist and generalist neurologists, psychiatrists, geriatricians, neuropsychologists, neuropathologists, and basic scientists in relevant fields.

Under the name of Frontotemporal Dementias (FTD) numerous hereditary and sporadic disorders are listed. FTD may take away speech and language, social skills and ethical judgement, wishes and will, empathy and emotions; it may also impair motor functions. FTD may affect men and women in midlife or during old age leading to the demolition of the uniqueness of the human mind. In the last decade of the 20th century and in the first two decades of the 21st century, progress in the understanding of clinical, neuropathological, biochemical, and genetic aspects of FTD has accelerated. The novel awareness about FTD has directed young generations of researchers toward the study of this complex group of disorders. This Volume has been formulated with the participation of some of the leading scientists who have contributed to the development of knowledge in the clinical and basic science arenas. It captures the current central elements that are relevant to an up-to-date understanding of causes and pathogenesis of multiple forms of FTD. The volume is an opus that represents a distillation of the work of many scientists and addresses the current directions in the study of one of the most complex groups of diseases. In view of its structure, the book could also be used as a textbook, that offers both a broad and deep analysis of major areas in FTD. This book, planned by the International Society for Frontotemporal Dementias, is distinctive as it opens a window to a wide landscape about the biology of FTD. Thus, the book represents a moment of reflection on the present state of our knowledge of FTD and a collective vision toward scientific progress. The authors of each chapter share their knowledge and vision aimed at reducing the suffering which is caused by FTD.

Alzheimer’s disease and frontotemporal dementia, two of the most prevalent neurodegenerative diseases, are dreaded disorders that attack the neural networks underlying memory and personality, systems that make us who we are. Major breakthroughs in recent years have spurred new research approaches to these conditions. In Alzheimer's Disease and Frontotemporal Dementia: Methods and Protocols, expert investigators bring together the many divergent areas of expertise used to study these diseases, including behavior, electrophysiology, confocal microscopy, and hardcore biochemistry. Beginning with an overview of the two diseases and contemporary research on them, the book continues with specialized protocols for working with amyloid-β peptide, tau, and apolipoprotein E as well as experimental systems for studying AD and FTD, including cell and animal models, and outcome measures that can be used to assess neuronal function in these systems. Written in the highly successful Methods in Molecular BiologyTM series format, chapters include brief introductions to their respective topics, lists of the necessary materials and reagents, step-by-step, readily reproducible laboratory protocols, and notes on troubleshooting and avoiding known pitfalls. Authoritative and easy to use, Alzheimer's Disease and Frontotemporal Dementia: Methods and Protocols serves as an ideal guide for all scientists pursuing the cause and cure of these widespread and nightmarishly debilitating disorders.

This open access book offers an essential overview of brain, head and neck, and spine imaging. Over the last few years, there have been considerable advances in this area, driven by both clinical and technological developments. Written by leading international experts and teachers, the chapters are disease-oriented and cover all relevant imaging modalities, with a focus on magnetic resonance imaging and computed tomography. The book also includes a synopsis of pediatric imaging. IDKD books are rewritten (not merely updated) every four years, which means they offer a comprehensive review of the state-of-the-art in imaging. The book is clearly structured and features learning objectives, abstracts, subheadings, tables and take-home points, supported by design elements to help readers navigate the text. It will particularly appeal to general radiologists, radiology residents, and interventional radiologists who want to update their diagnostic expertise, as well as clinicians from other specialties who are interested in imaging for their patient care.

Most textbooks on neurodegenerative disorders have used a classification scheme based upon either clinical syndromes or anatomical distribution of the pathology. In contrast, this book looks to the future and uses a classification based upon molecular mechanisms, rather than clinical or anatomical boundaries. Major advances in molecular genetics and the application of biochemical and immunocytochemical techniques to neurodegenerative disorders have generated this new approach. Throughout most of the current volume, diseases are clustered according to the proteins that accumulate within cells (e.g. tau, α-synuclein and TDP-43) and in the extracellular compartments (e.g. β-amyloid and prion proteins) or according to a shared pathogenetic mechanism, such as trinucleotide repeats, that are a feature of specific genetic disorders. Chapters throughout the book conform to a standard lay-out for ease of access by the reader and are written by a panel of International Experts Since the first edition of this book, major advances have been made in the discovery of common molecular mechanisms between many neurodegenerative diseases most notably in the frontotemporal lobar degenerations (FTLD) and motor neuron disease or amyotrophic lateral sclerosis. This book will be essential reading for clinicians, neuropathologists and basic neuroscientists who require the firm up-to-date knowledge of mechanisms, diagnostic pathology and genetics of Neurodegenerative diseases that is required for progress in therapy and management.

The subject of this book is a stranger than fiction change in personalities, behaviors and relationships including the gradual loss of language and the meaning of words. Nineteen lives are chronicled as told by caregivers, each selected for the drama and strangeness of behavior or cognition. These clinical tales are intended for the general reader who is interested in how the brain processes language, behavior, emotions, meaning and morality and in particular for the families, who sometimes suffer more than the patients. In each chapter the recurrent symptoms, the biology of an underlying brain disorder and the social and cultural aspects of the behavior change is explored. Technical terms and their origin is explained. The vivid and accurate case histories and the last three chapters reviewing and updating the biology, genetics and the pharmacology have educational value for professionals as well. There is a special chapter: “Tips for caregivers” and a glossary.

Frontotemporal Degeneration (FTD) is now recognized as one of the most common forms of dementia in individuals under age 65, second only to Alzheimer’s. Shedding light on a little known brain disease, this volume examines FTD from a few angles, beginning with the author’s insightful memoir of her husband’s struggle with FTD and its impact on their family. Detailed background information on the disease is provided along with discussion of related issues, and information on how to minimize the chances of becoming a victim.