This book summarizes the advances in our understanding of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), as well as the potential relationship between the two.

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.

This practical guide to the diagnosis of neurodegenerative diseases discusses modern molecular techniques, morphological classification, fundamentals of clinical symptomology, diagnostic pitfalls and immunostaining protocols. It is based on the proteinopathy concept of neurodegenerative disease, which has influenced classification and provides new strategies for therapy. Numerous high-quality images, including histopathology photomicrographs and neuroradiology scans, accompany the description of morphologic alterations and interpretation of immunoreactivities. Diagnostic methods and criteria are placed within recent developments in neuropathology, including the now widespread application of immunohistochemistry. To aid daily practice, the guide includes diagnostic algorithms and offers personal insights from experienced experts in the field. Special focus is given to the way brain tissue should be handled during diagnosis. This is a must-have reference for medical specialists and specialist medical trainees in the fields of pathology, neuropathology and neurology working with neuropathologic features of neurodegenerative diseases.

This volume covers the latest methods used in clinical neurochemistry laboratories for both clinical practice and research. Chapters in this book discuss topics such as techniques for cerebrospinal fluid (CSF) collection, pre-analytical processing, and basic CSF analysis; an examination of biomarkers including ELISA and automated immunochemical assays for amyloid and tau markers for Alzheimer’s disease; the analysis of neurofilaments by digital ELISA; and an example of successful novel immunoassay development. In the Neuromethods series style, chapters include the kind of detail and key advice from the specialists needed to get successful results in your laboratory. Cutting-edge and thorough, Cerebrospinal Fluid Biomarkers is a valuable resource for clinicians and researchers to use in CSF labs and CSF courses.

Neurofibrillary tangles (NFTs) composed of intracellular aggregates of tau protein are a key neuropathological feature of Alzheimer’s Disease (AD) and other neurodegenerative diseases, collectively termed tauopathies. The abundance of NFTs has been reported to correlate positively with the severity of cognitive impairment in AD. However, accumulating evidences derived from studies of experimental models have identified that NFTs themselves may not be neurotoxic. Now, many of tau researchers are seeking a “toxic” form of tau protein. Moreover, it was suggested that a “toxic” tau was capable to seed aggregation of native tau protein and to propagate in a prion-like manner. However, the exact neurotoxic tau species remain unclear. Because mature tangles seem to be non-toxic component, “tau oligomers” as the candidate of “toxic” tau have been investigated for more than one decade. In this topic, we will discuss our consensus of “tau oligomers” because the term of “tau oligomers” [e.g. dimer (disulfide bond-dependent or independent), multimer (more than dimer), granular (definition by EM or AFM) and maybe small filamentous aggregates] has been used by each researchers definition. From a biochemical point of view, tau protein has several unique characteristics such as natively unfolded conformation, thermo-stability, acid-stability, and capability of post-translational modifications. Although tau protein research has been continued for a long time, we are still missing the mechanisms of NFT formation. It is unclear how the conversion is occurred from natively unfolded protein to abnormally mis-folded protein. It remains unknown how tau protein can be formed filaments [e.g. paired helical filament (PHF), straight filament and twisted filament] in cells albeit in vitro studies confirmed tau self-assembly by several inducing factors. Researchers are still debating whether tau oligomerization is primary event rather than tau phosphorylation in the tau pathogenesis. Inhibition of either tau phosphorylation or aggregation has been investigated for the prevention of tauopathies, however, it will make an irrelevant result if we don’t know an exact target of neurotoxicity. It is a time to have a consensus of definition, terminology and methodology for the identification of “tau oligomers”.

This book, written by a leading panel of experts in the field of neurosciences, provides a comprehensive overview of the pathology of neurodegenerative diseases as well as the preventive measures. Prevention is important due to the lack of early diagnostic markers and the limitations/ problems of treating neurodegenerative diseases

Diagnosis and Management in Dementia: The Neuroscience of Dementia, Volume 1 consolidates different fields of dementia into a single book, covering a range of subjects, including Alzheimer's disease, Lewy body dementia, mixed dementia, vascular dementia, physical activity, risk factors, mortality, biomarkers, SPECT, CT, MRI, questionnaires, nutrition, sleep, delirium, hearing loss, agitation, aggression, delusions, anxiety, depression, hallucinations, psychosis, senile plaques, tau and amyloid-beta, neuroinflammation, molecular biology, and more. With an impact on millions globally, and billions of research dollars being invested in dementia research, this book will stimulate research in the area and inform researchers. - Offers comprehensive coverage of a broad range of topics related to dementia - Serves as a foundational collection for neuroscientists and neurologists on the biology of dementia and brain dysfunction - Contains in each chapter an abstract, key facts, mini dictionary of terms, and summary points to aid in understanding - Provides unique sections on specific subareas, intellectual components, and knowledge-based niches that will help readers navigate key areas for research and further clinical recommendations - Features preclinical and clinical studies to help researchers map out key areas for research and further clinical recommendations - Serves as a "one-stop" source for everything you need to know about dementia