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The remarkable family of proteins that can make us very ill—but can also be linked to long-term memory, immunity, and the origin of lifeOver the last decade, scientists have discovered the importance and widespread presence in the body of a remarkable family of proteins known as prion proteins. Research links various types of prion proteins to neurodegenerative diseases such as Parkinson's and Alzheimer's—and this has fueled the search for new drugs that could halt the progression of these terrible disorders. Other discoveries have revealed the essential roles prion proteins play in memory and immunity, and—in an extraordinary finding—the part they may have played in the beginnings of life on our planet. In this engaging and accessible book, Michel Brahic tells the story of these amazing and versatile proteins.Brahic, a leading researcher on diseases of the central nervous system, first describes the discovery of prions and their role in infection, beginning with early work on the animal disease scrapie and a mysterious human illness in New Guinea, apparently transmitted by cannibalism. Prions were eventually identified and named by Stanley Prusiner in the 1980s. (Brahic tells us Prusiner's alternate name for prion was “piaf.”) Prion proteins were then revealed as the cause of other illnesses, from “mad cow” disease and its human counterpart, Creutzfeldt-Jakob disease, to such noninfectious brain disorders as Parkinson's and Alzheimer's. While the prion proteins responsible for diseases are definitively “bad,” Brahic also explains that these abnormal prions are rare exceptions. Most of the time, prion proteins actually serve “good” and vital functions—and they may even have been present at the origin of life itself.

This reference is the definitive guide to common neurodegenerative diseases that affect humans. The book covers mechanisms of some of the most well-known neurodegenerative diseases, their biomarkers, neuropharmacology, and emerging treatment strategies.The book introduces the subject of neurodegeneration by outlining the biochemistry, pathophysiology and multifactorial neurological mechanisms (the role of genetics, environmental factors and mitochondrial damage, for example). Next, it explains some of the most studied diseases, namely, Parkinson amp;rsquo;s Disease, Alzheimer amp;rsquo;s Disease, Huntington amp;rsquo;s Disease, and Multiple Sclerosis. Subsequent chapters delve into current knowledge about diagnostic and immunological biomarkers, followed by a summary of novel therapeutic strategies.Special attention has been given to the role of medicinal plants in attempting to treat neurodegenerative diseases, as well as the public health burden posed by these conditions.Key Features- give readers an overview of multifactorial disease mechanisms in neurodegeneration- covers some major neurodegenerative diseases in detail- covers diagnostic and immunological biomarkers- explores current therapeutic strategies and drug targets in common neurodegenerative diseases- offers a simple presentation with references for advanced readersThe book is a suitable reference for all readers, including students, research scholars, and physicians who are interested in the mechanisms and treatment of neurodegenerative diseases.

Degenerative nerve diseases are age-related, progressive and cause irreversible neurological loss that may lead to death. Chronic diseases like amyotrophic lateral sclerosis, Huntington rsquo;s disease, Alzheimer rsquo;s disease, and Parkinson rsquo;s disease are associated with insoluble protein depositions and pose serious health challenges that may intensify in the coming decades. Current therapies only help to alleviate some of the physical or mental symptoms associated with neurodegenerative diseases, although there is currently no remedy for slow disease progression. In recent years, attempts have been made to discover a mechanism for neurodegenerative diseases and prospective treatment that may help mitigate aging effects and prevent these diseases. Natural products have been a constant source of new approaches for the treatment of neurodegenerative diseases, in particular plant alkaloids and polyphenolic compounds.Indopathy for Neuroprotection: Recent Advances highlights herbal treatments that are preferred over conventional treatments in some regions. Book chapters focus on the effects of various medicinal plants that have shown promise in reversing pathological symptoms of neurodegenerative disease and highlight the neuroprotective role of medicinal herbal phytochemicals and their mechanism of action. The book serves as a reference for pharmacology and herbal medicine scholars as well as healthcare workers interested in information about alternative and complementary therapies for neurological disorders.

Human Caspases and Neuronal Apoptosis in Neurodegenerative Diseases elucidates elaborately the role of caspase enzymes implicated in the initiation of molecular events leading to neuronal apoptosis in the neurodegenerative disease. The book starts with introduction to neuropathology, neurogenetics, and epidemiology of neurodegenerative disease and illustrates the involvement of human caspases, neuronal apoptosis, apoptotic pathways, genetic polymorphisms, and several other factors and underlying mechanisms in the pathology of Alzheimer's disease, Parkinson's disease, and Huntington's disease. An important focus in all chapters is the intricate mechanisms and interplay that occur during or leading to neuron death in neurodegenerative diseases, along with disease pathobiology. - Provides in-depth knowledge about neurotoxic potential of transition metals, impaired mitochondrial dynamics in the brain neurons, mutant proteins Aß peptide, tau protein, a-synuclein, huntingtin protein and formation of Lewy bodies, reactive oxygen and nitrogen species, ubiquitin proteasome dysregulation, and many others in neurodegenerative diseases - Elucidates neurogenetics of gene APP, gene PSEN1, gene APOE, gene LRRK2, gene DJ1, and others in the pathology of neurodegenerative diseases - Explains caspases-mediated neuronal apoptosis in pathogenesis of Alzheimer's disease covering amyloidogenesis, caspase-activated DNase, rho-associated coiled coil–containing protein kinase 1, mammalian sterile 20-like kinase 1, role of synaptic loss, microglial TREM2 receptor, microglial LRP1 receptor, microglial advanced glycation end-product receptor, astrocytic glial a 7 subtypes of nAChR, NLRP3 inflammasome, P2X purinoreceptors, miRNAs, and many other factors - Demonstrates the role of caspases and apoptosis in Parkinson's disease covering truncation of a-synuclein, neuroinflammation, parkin protein, activation of microglial cells, extrinsic and intrinsic pathways of apoptosis,?tau314, and several other factors - Explains etiopathogenesis of Huntington's disease through covering clinically important topics as role of exon 1 HTT protein, ubiquitous nature of huntingtin, length of expanded polyglutamine tract, classically and alternately activated microglia, nuclear factor kappa B, kynurenine signaling pathway, tumor suppressor protein, PGC-1a gene, advanced glycation end-products, autophagy, and many other significant topics

The Neurobiology of Aging and Alzheimer Disease in Down Syndrome provides a multidisciplinary approach to the understanding of aging and Alzheimer disease in Down syndrome that is synergistic and focused on efforts to understand the neurobiology as it pertains to interventions that will slow or prevent disease. The book provides detailed knowledge of key molecular aspects of aging and neurodegeneration in Down Syndrome by bringing together different models of the diseases and highlighting multiple techniques. Additionally, it includes case studies and coverage of neuroimaging, neuropathological and biomarker changes associated with these cohorts. This is a must-have resource for researchers who work with or study aging and Alzheimer disease either in the general population or in people with Down syndrome, for academic and general physicians who interact with sporadic dementia patients and need more information about Down syndrome, and for new investigators to the aging and Alzheimer/Down syndrome arena. - Discusses the complexities involved with aging and Alzheimer's disease in Down syndrome - Summarizes the neurobiology of aging that requires management in adults with DS and leads to healthier aging and better quality of life into old age - Serves as learning tool to orient researchers to the key challenges and offers insights to help establish critical areas of need for further research

Plongez dans cet ouvrage pour en apprendre davantage sur les maladies neurodégénératives et les apprivoiser...Comment se manifeste la maladie d'Alzheimer? Quels sont les symptômes de la maladie de Parkinson, et de Charcot? Comment soutenir les proches qui en souffrent? Comment accepter et mieux cohabiter avec ces pathologies? Les maladies neurodégénératives touchent chaque année de plus en plus de personnes. Malgré cette importante présence, elles restent largement méconnues du grand public, ce qui rend leur diagnostic d'autant plus difficile à appréhender. Le Docteur Mespouille, neurologue et chargé de cours sur le sujet, fait le point sur ces trois maladies. Il décrit avec précision les manifestations de leur développement ainsi que les premiers signes à détecter. Cette reconnaissance précoce est essentielle puisqu'elle permet une meilleure prise en charge des personnes qui en souffrent, et facilite leur cohabitation avec la maladie. Il aborde les difficultés rencontrées par les patients et propose des réponses concrètes aux questions que ceux-ci (se) posent. Illustrant son propos de nombreux témoignages, il apporte les connaissances nécessaires pour rassurer les patients et leur entourage, et permet à ces derniers d'accompagner au mieux leurs proches. Un indispensable pour comprendre, accepter et mieux vivre les maladies neurodégénératives!À PROPOS DE L'AUTEURPascal Mespouille est un médecin spécialiste en neurologie depuis plus de 30 ans. Ancien chef de service, maître de stage et chargé de cours, il a pratiqué notamment aux Cliniques du Sud-Luxembourg, en Belgique. Il donne régulièrement des conférences sur le sujet.

Recent Developments in Neurodegeneration discusses the diverse functions of nitric oxide regarding redox regulation, the cellular energy pool, modifications in genetic material, neurogenesis, the effects on protein degradation mechanisms and functional consociation with glial cells. The authors consider the latest evidence that demonstrates that Alzheimer's disease-associated proteins and their genes play a key role in the post-ischemic development of neurodegeneration with dementia. The main processes associated with secondary tissue damage following traumatic injury to the nervous system are reviewed. In closing, a comprehensive investigation of the combination of tremor and olfactory perception characteristics among patients with Parkinson's disease and essential tremor is presented.

Molecular Aspects of Neurodegeneration, Neuroprotection, and Regeneration in Neurological Disorders presents readers with comprehensive and cutting-edge information on the neurochemical mechanisms of various types of neurological disorders. The book covers information on signal transduction processes associated with neurochemistry of neurological disorders, including neurodegenerative, neurotraumatic, and neuropsychiatric disorders. The book also discusses risk factors, symptoms, pathogenesis, biomarkers, and the potential treatments of neurological disorders. The comprehensive information in this monograph may not only help in early detection of various neurological disorders, but will also promote the discovery of new drugs. - Provides a comprehensive overview of the molecular aspects of neurodegeneration, neuroprotection, and neuro-regeneration, along with therapeutic strategies for various types of neurological disorders - Provides cutting-edge research information on the signal transduction processes associated with the neurochemistry of neurological disorders - Discusses risk factors, symptoms, pathogenesis, biomarkers, and the potential for treatments of neurological disorders

Multiple advanced neuroimaging applications in various neurodegenerative diseases including Parkinson's disease (PD), frontotemporal dementia (FTD), vascular dementia (VaD) and autism spectrum disorder (ASD) are covered in this book. Relatively novel techniques such as integrated PET/MRI and independent component analysis (ICA)-based dual regression (DR) methods were developed to capture multi-level molecular/functional and structural/microstructural as well as high-order inter-network coordination abnormalities. For instance, both PET dopamine transporter and striatal binding ratio reductions in the caudate and putamen were found in PD, consistent with the diffusion tensor imaging (DTI) fractional anisotropy (FA) reduction and fMRI voxel-mirrored homotopic correlation (VMHC) in the substantia nigra (swallow tail sign signature of PD). Furthermore, dopamine storage and pathway labeled with the vesicular monoamine transporter tracer identified decreased densities in the bilateral mesial temporal cortex, caudate, orbitofrontal cortex, left frontal and occipital cortices, consistent with the morphological atrophy, functional connectivity and conductivity deficits in PD. Similarly in FTD patients, the advanced MRI methods such as ICA-DR, VMHC, voxel-based morphometry (VBM) as well as PET tracer for amyloid accumulation and FDG glucose uptake identified typical brain atrophy, structural dis-connectivity, glucose hypometabolism, higher neuropathological burden, lower interhemispheric correlation as well as disrupted intra- and inter-network modulation in the orbitofrontal and anterior temporal cortices together with insular and frontoparietal networks, with the cerebellum and dorsolateral attentional network as typical compensations. Functional and structural abnormalities had further been elucidated in the VaD dependent participants and autistic children. For instance, both lower FA and VMHC, brain atrophy and functional connectivity deficits, demyelination, axonal degeneration and white matter integrity damage in several white matter tracts were present in the dependent compared to independent participants in VaD data cohort. Increased neuronal activity with higher global fractional amplitude of low frequency fluctuation (fALFF) in the conventional and slow-wave sub-band was confirmed with less efficiency of systematic integration in VaD dependent group. Moreover, in ASD compared to controls, regional gray matter volume and cortical thickness in all four brain lobes increased, whereas white matter volume were decreased in addition to the lower temporal, visual and superior frontal but higher inferior and dorsolateral prefrontal cortical functional connectivities exhibited in ASD. The differences in each type of disease could also be revealed with the same imaging method based on either unique region or distinct brain circuit inter-connection, using VMHC, ICA-DR, DTI, VBM, fALFF and graph-theory based small-worldness analysis. In this book, we have developed and generalized conventional and advanced imaging methodologies to several common neurodegenerative diseases. For instance, we have identified the unique imaging signature for each disease type and the underlying neuropathological mechanism connections with conductivity, structural and microstructural connectivity, intra- and inter-network correlation, systematic integration and efficiency analyses. Our objective, comprehensive and confirmative results indicated great potential in utilizing these quantifications for accurate disease classification and staging. With solid imaging evidence, thorough analysis and generalized applications, this book should capture the interests of readers in the broad fields of brain science, disease diagnosis and effective treatment.

El envejecimiento poblacional es una realidad global, y Colombia no es ajena a dicho proceso; este cambio demográfico trae consigo la necesidad de diagnosticar e intervenir las enfermedades neurodegenerativas que afectan a dicha población; la evaluación neuropsicológica es un proceso fundamental para responder a esta necesidad. Este libro presenta el resultado de un Proyecto multicéntrico de normalización de instrumentos de evaluación en población mayor de 49 años, ofreciendo una herramienta estandarizada y fiable para el diagnóstico neuropsicológico. Se presentan las características metodológicas y de interpretación, así como la descripción rigurosa de casos clínicos con diferentes patologías del sistema Nervioso Central estudiados con la batería. Se ilustra su utilidad clínica y su rol dentro del proceso de evaluación. También se presentan perfiles gráficos realizados a través de la Unidad de Trabajo, herramienta que unifica la calificación, interpretación y presentación de los perfiles.

Demencia y memoria narra el desarrollo de la investigación sobre enfermedades cada vez más comúnes y con tanto impacto en la vida social, económica e íntima de los humanos, como lo son las enfermedades neurodegenerativas (alzheimer, párkinson). Escrita por el premio Nobel de Medicina, Stanley B. Prusiner, quien descubrió el papel de ciertas proteínas en su origen. Se trata de la historia de un descubrimiento médico y sus implicaciones en nuestra vida.

Prions and Neurodegenerative Diseases, Volume 172, in the Progress in Molecular Biology and Translational Science series, provides the most topical, informative and exciting monographs available on a wide variety of research topics. The series includes in-depth knowledge on the molecular biological aspects of organismal physiology, with this release including chapters on Cell-free amplification of prions: where do we stand? Transgenic mouse models for the study of prion diseases and much more. - Includes comprehensive coverage of molecular biology - Presents ample use of tables, diagrams, schemata and color figures to enhance the reader's ability to rapidly grasp the information provided - Contains contributions from renowned experts in the field - Includes comprehensive coverage of molecular biology - Presents ample use of tables, diagrams, schemata and color figures to enhance the reader's ability to rapidly grasp the information provided - Contains contributions from renowned experts in the field

A statistics textbook that delivers essential data analysis techniques for Alzheimer's and other neurodegenerative diseases.Alzheimer's disease is a devastating condition that presents overwhelming challenges to patients and caregivers. In the face of this relentless and as-yet incurable disease, mastery of statistical analysis is paramount for anyone who must assess complex data that could improve treatment options. This unique book presents up-to-date statistical techniques commonly used in the analysis of data on Alzheimer's and other neurodegenerative diseases. With examples drawn from the real world that will make it accessible to disease researchers, practitioners, academics, and students alike, this volume• presents code for analyzing dementia data in statistical programs, including SAS, R, SPSS, and Stata• introduces statistical models for a range of data types, including continuous, categorical, and binary responses, as well as correlated data• draws on datasets from the National Alzheimer's Coordinating Center, a large relational database of standardized clinical and neuropathological research data• discusses advanced statistical methods, including hierarchical models, survival analysis, and multiple-membership• examines big data analytics and machine learning methodsEasy to understand but sophisticated in its approach, Fundamental Statistical Methods for Analysis of Alzheimer's and Other Neurodegenerative Diseases will be a cornerstone for anyone looking for simplicity in understanding basic and advanced statistical data analysis topics. Allowing more people to aid in analyzing data—while promoting constructive dialogues with statisticians—this book will hopefully play an important part in unlocking the secrets of these confounding diseases.

Holbourne's theory that rotational head movement and shear strains were limiting factors in producing acute parenchymal brain damage was a watershed moment in understanding traumatic brain injury (TBI). Long term effects, and in particular neurodegenerative proteinopathy subsequent to TBI, remain theoretical, notwithstanding the poorly understood ‘punch drunk'syndrome of the early and mid-20th century, and the 21st century concept of chronic traumatic encephalopathy. This book, the Handbook of Traumatic Brain Injury and Neurodegeneration, has as its theme the marriage between neurodegenerative disease and neurotrauma through TBI surrogates such as sport, military service, and experimental models, and the legitimacy of that marriage. In the 32 contributions included here, this handbook not only explores the deleterious effects of genuine TBI, but also, and more importantly, the relationship between TBI and neurodegeneration. Controversy notwithstanding, there is much to be learned about the biological effects of TBI, substrates for long-term sequelae, the relationship between TBI and diverse neuropsychiatric disorders, and targets for therapy. The overall message to the neuroscience community from these papers may be a cautionary tale. The null hypothesis, that there is no causal relationship between TBI and progressive neurodegenerative disease, appears to be very much in play, and the book will be of interest to all those working in the field.

Metabolic Drivers and Bioenergetic Components of Neurodegenerative Disease reviews how the different aspects of metabolic dysfunction and consequent pathology associated with neurodegenerative diseases, including Alzheimer's and Parkinson's, can be targeted by novel treatment approaches. Topics covered include Cellular Senescence in Aging and Age-Related Disorders: Implications for Neurodegenerative Diseases; Repurposing GLP1 agonists for Neurodegenerative Diseases; Ketotherapeutics for Neurodegenerative Diseases; Enhancing Mitophagy as a Therapeutic Approach for Neurodegenerative Diseases; Harnessing Neurogenesis in the Adult Brain – A Role in Type 2 Diabetes Mellitus and Alzheimer's disease; and much more. - Summarizes the impact of the metabolic hypothesis on underlying mechanisms of neurodegenerative diseases - Presents novel, potential treatment strategies based on the metabolic hypothesis for neurodegenerative diseases

An estimated 80 million people live with a neurodegenerative disease. That number is expected to increase rapidly as populations age, lifespans increase, and exposure to toxins rises. Despite decades of research and billions in funding, there are no medications that can slow, much less stop, the progress of these diseases. This is because diseases such as Parkinson's and Alzheimer's do not exist in biology. Yet, hundreds of clinical trials around the world are examining the potential of single therapies in thousands of people sharing one of these labels. Compounding the problem, these therapies were developed on evidence from models that do not come close to capturing the complexity of these diseases in the affected humans. These practices must end. Brain Fables is a call to refocus on understanding living and aging to create the personalized treatments each affected individual desperately needs.

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

This book provides a comprehensive overview of the role of neuroglia in neurodegenerative diseases. Neuroglia are the most abundant cells in the nervous system and consist of several distinct cell types, such as astrocytes, oligodendrocytes,and microglia. Accumulating evidence suggests that neuroglia participate in the neurodegenerative process, and as such are essential players in a variety of diseases, including Alzheimer's, Parkinson's, and Huntington's. Intended for researchers and students, the book presents recent advances concerning the biology of neuroglia as well as their interaction with neurons during disease progression. In addition, to highlight the function of neuroglia in different types of neurodegenerative disease, it also discusses their mechanisms and effects on protecting or damaging neurons.

This book includes a series of reviews on general aspects of biomarker use in the study of psychiatric and neurodegenerative diseases and the development of medications involved in their treatment. It describes the pros and cons of the various approaches and covers the successes and failures in this research field. It is only by a thorough understanding of the shortcomings that progress can be made. The overall goal is to facilitate the understanding and treatment of these disorders, by providing a viable mechanism of catching up with other areas of modern medicine, such as diabetes and heart disease. Finally, it is anticipated that the development and application of valid biomarker tests and the leveraging of novel drug targets will help the fields of psychiatry on neurodegenerative disorders move into the area of personalized medicine where the right patients can receive the right medication at the right time for the best possible outcome.

This book focuses on neurodegenerative diseases which have become a major threat to human health. Neurodegenerative diseases are age related disorders and have become increasingly prevalent in the elderly population in recent years. Hence, there is an urgent need to study and develop new strategies and alternative methods for the treatment of neurodegenerative diseases. This book showcases the promises that nanobiotechnology brings in research, diagnosis, and treatment of neurodegenerative diseases. It is very beneficial for varied group of readers including nanotechnologists, biotechnologists, pharmacists, medical professionals, bioengineers, biochemists and researchers working in this field. Nanobiotechnology in Neurodegenerative Diseases include various chapters including neurodegeneration and neurodegenerative diseases, nanotechnology for the rescue of neurodegenerative diseases, promising potential of nanomaterials for diagnosis and therapy of neurodegenerativediseases, nanotechnology mediated nose-to-brain drug delivery, and formulation and characterization of intranasal nanoparticles of antiretroviral drugs.

Clinical Neuroscience offers a comprehensive overview of the biological bases of major psychological and psychiatric disorders, and provides foundational information regarding the anatomical and physiological principles of brain functioning. In addition, the book presents information concerning neuroplasticity, pharmacology, brain imaging, and brain stimulation techniques. Subsequent chapters address specific psychological disorders and neurodegenerative diseases, including major depressive and bipolar disorders, anxiety, schizophrenia, disorders of childhood origin, and addiction, as well as neurodegenerative disorders, such as Parkinson's and Alzheimer's diseases. This highly readable textbook expands case examples and illustrations to discuss the latest research findings in clinical neuroscience from an empirical, interdisciplinary perspective.

Neuroimaging techniques that can help elucidate and characterize the nature and mechanism of tissue injury and disease progression in neurodegenerative disease are of particular importance given its their roles in seeking successful preventive and therapeutic treatments. Studying large-scale samples with various disease mechanisms using multi-parametric imaging, as well as revealing the correlations between the neuroimaging metrics and clinical data including neurocognitive function and neuropsychological inventories to elucidate multiple factors affecting the neurodegeneration processes in brain are the main topics of this book. In addition, the neural underpins of cognitive and psychological functions with advanced functional imaging techniques can provide better cross-validation and clinical symptom relevance of multi-parametric data. Expanding the current findings with higher diagnosis accuracy and detection specificity in multiple neurodegenerative diseases as well as better differentiation of each type are the ultimate goal. The results in this book will extend the current notion of diagnosis value of various relatively new imaging techniques in multiple neurodegenerative diseases including traumatic brain injury, post-traumatic stress disorder, multiple sclerosis and early stage of Alzheimer's disease such as mild cognitive impairment. Specifically, the neurobiology and related imaging findings of the four representative neurodegenerative diseases will be introduced and reviewed, including brain region-specific and disease-related alterations, unique clinical symptom of each disease, as well as previous findings and challenges. There is an increasing body of literature suggesting that damage to the default mode network, hypothalamus, thalamus and hippocampus neuronal networks and local injuries might be under-diagnosed and may account for some of the sequelae following the neurodegenerative injuries including trauma and dementia. The relatively novel imaging results to differentiate each disease using advanced functional connectivity, neuronal activity, microstructure integrity analysis based on structural connectivity, multi-dimensional morphometry and molecular imaging tracers including amyloid and tau for neuropathological burden quantification were presented to differentiate each type of disease. We then briefly reviewed some of the therapeutic effects of traditional Chinese medicine with neuroimaging quantifications to help treating neurodegenerative diseases. Finally, our work proves that the multi-parametric neuroimaging methods with more than twelve metrics and numerous tight clinical association data presented in this book are the most forefront and up-to-date with enough sensitivity, precision and resolution. Taken together, multiple neuroimaging metrics haved been demonstrated in this book to identify and quantify significant and distinct brain alterations at function, microstructure, morphology and molecular scales in different types of neurodegenerative diseases with high sensitivity and specificity. These comprehensive imaging features could be combined to improve disease diagnosis accuracy. The aim of this book is thus intended to provide both beginners and experts in biomedical imaging and health care a broad and complete picture as well as the new developments of using multiple metrics in improving disease identification and diagnosis accuracy. This book would hopefully capture the interests of colleagues interested in neurodegenerative disease diagnosis and treatment, and could help convey the methodological and integrative perspectives of multi-parametric neuroimaging applications.

This book provides comprehensive coverage of small fiber neuropathy (SFN), from diagnosis to therapy. It focuses on nerve degeneration and neuropathic pain, and their underlying pathology, physiology, psychophysics, genetics and imaging. In particular, this book describes and discusses the major advances in diagnostic techniques for assessing SFN. These include skin biopsy, evoked potentials, quantitative sensory testing and functional studies, as biomarkers of SFN.SFN is a common peripheral nerve disorder, but was often overlooked due to a lack of objective and specific diagnostic tests for the assessment of small nerve fibers. These fibers mediate thermal sensation, pain detection (nociception), and autonomic regulation. Major symptoms of SFN include neuropathic pain, impaired sensation and autonomic dysfunction. Neuropathic pain poses a diagnostic challenge to clinicians, an essential step for selecting appropriate treatment to relieve suffering.SFN frequently develops in systemic diseases such as diabetes mellitus, following chemotherapy, infections etc., or presents as a major feature of various genetic neuropathies (e.g. channelopathy and familial amyloidosis). In addition to describing these conditions which lead to SFN, this book also describes related syndromes of neurodegeneration and pain, including fibromyalgia, visceral pain and hypersensitivity.This definitive book covers both clinical aspects and research progress, which provides in-depth and up-to-date information on SFN. It would be immensely useful for clinicians, neurologists, neuroscientists, diabetologists, and pain specialists. Dr. Sung-Tsang Hsieh is a professor at Department of Neurology and Institute of Anatomy and Cell Biology, College of Medicine, National Taiwan University, Taiwan. He is also the associate dean of College of Medicine, National Taiwan University, Taiwan. Dr. Praveen Anand is a professor at Department of Clinical Neurology and head of Centre for Clinical Translation, Hammersmith Hospital, UK. Dr. Christopher Gibbons is an associate professor of Neurology, Harvard Medical School, Beth Israel Deaconess Medical Center, USA. Dr. Claudia Sommer is a professor of Neurology at the Department of Neurology, Würzburg University Hospital, Germany.

Late aging associated changes in alcohol sensitivity, neurobehavioral function, and neuroinflammation, Volume 148, the latest release in the International Review of Neurobiology series, highlights new advances in the field, with this new volume presenting interesting chapters on a variety of timely topics. Each chapter is written by an international board of authors. - Provides the authority and expertise of leading contributors from an international board of authors - Presents the latest release in the International Review of Neurobiology series - Includes the latest information on late aging-associated changes in alcohol sensitivity, inflammation and cognitive decline

Mitochondrial Neuropathies, Volume 146, brings together experts in a range of diseases that damage the nervous system to present the role of mitochondrial dysfunction in their particular field, with this new release focusing on Mitochondrial dysfunction in Alzheimer's Disease, HIV and the mitochondrial: immune interface in the CNS, The Impact of mitochondrial damage in HIV-induced peripheral neuropathy, Mitochondrial dysfunction and the pathogenesis of chemotherapy-induced peripheral neuropathy, Disorders of mitochondrial biogenesis in peripheral neuropathy, Mitochondrial dysfunction and the pathogenesis of diabetic neuropathy, Manipulating mitochondria to treat peripheral neuropathy, and DCA therapy - the yin and yang of mitochondrial activation. - Contains contributions from a diverse group of experts - Presents a timely resource that brings together different fields to highlight a common pathogenic mechanism - Focuses on the role of mitochondria in diverse neuropathies

Leveraging Biomedical and Healthcare Data: Semantics, Analytics and Knowledge provides an overview of the approaches used in semantic systems biology, introduces novel areas of its application, and describes step-wise protocols for transforming heterogeneous data into useful knowledge that can influence healthcare and biomedical research. Given the astronomical increase in the number of published reports, papers, and datasets over the last few decades, the ability to curate this data has become a new field of biomedical and healthcare research. This book discusses big data text-based mining to better understand the molecular architecture of diseases and to guide health care decision. It will be a valuable resource for bioinformaticians and members of several areas of the biomedical field who are interested in understanding more about how to process and apply great amounts of data to improve their research. - Includes at each section resource pages containing a list of available curated raw and processed data that can be used by researchers in the field - Provides demonstrative and relevant examples that serve as a general tutorial - Presents a list of algorithm names and computational tools available for basic and clinical researchers

This book gives an overview of the current knowledge on the most common neurodegenerative diseases, including Alzheimer's disease, frontotemporal lobar degeneration, Lewy body dementia,Parkinson's disease, amyotrophic lateral sclerosis, and additional less common neurodegenerative diseases. Both clinical and basic aspects of each disease are treated, including novel diagnostic criteria, old and new treatments, basic discoveries (genetics, epigenetics and molecular biology), and translation of basic research into biomarkers for early diagnosis, particularly to identify peripheral biomarkers. In addition, emerging data indicate that neurodegeneration seems to also be present in classically non-degenerative disorders. Therefore, a chapter about overlapping mechanisms between dementias and psychiatric disorders is included, as well as a description of the role of neurodegeneration in multiple sclerosis.Neurodegenerative Diseases is aimed at clinicians, particularly those workingin academic hospitals. This multidisciplinary book will also be of interest to basic researchers in medical fields.

The Molecular and Clinical Pathology of Neurodegenerative Disease brings together in one volume our current understanding of the molecular basis of neurodegeneration in humans, targeted at neuroscientists and graduate students in neuroscience, and the biomedical and biological sciences. Bringing together up-to-date molecular biology data with clinical evidence, this book sheds a light on common molecular mechanisms that underlie many different neurodegenerative diseases and addresses the molecular pathologies in each. The combined research and clinical background of the authors provides a unique perspective in relating clinical experiences with the molecular understanding needed to examine these diseases and is a must-read for anyone who wants to learn more about neurodegeneration. - Provides an up-to-date summary of neurodegeneration at a molecular, cellular, and tissue level for the most common human disorders - Describes the clinical background and underlying molecular processes for Alzheimer's disease, Parkinson's, Prion, Motor Neuron, Huntington's, and Multiple Sclerosis - Highlights the state-of-the-art treatment options for each disorder - Details examples of relevant cutting edge experimental systems, including genome editing and human pluripotent stem cell-derived neuronal models

Neuroinflammation, Second Edition, discusses the role inflammation plays in neuro-inflammatory diseases. As research in neuroinflammation has dramatically increased in recent years, this new edition covers all of the new developments and findings since the first publication nearly a decade ago. Comprehensive coverage of topics includes chapters on multiple sclerosis, optic neuritis and Susac syndrome. New chapters cover the latest research in Epilepsy and Neuroinflammation, Pediatric MS, Pediatric Lupus and Traumatic Brain Injury, amongst other topics. Inflammation is a central mechanism in many neurological diseases, including stroke, multiple sclerosis, brain trauma and meningitis, and it contributes to the generation of pain. Researchers are now beginning to understand the impact of the immune system on different nervous system functions and diseases, ranging from damage, through tolerance, and to modulation and repair. - Comprehensively reviews the latest developments in neuroinflammation and neuro-inflammatory diseases - Written for researchers and clinical practitioners in clinical neuroscience, neurology and immunology - Includes new chapters on Epilepsy and Neuroinflammation, Pediatric MS, Pediatric Lupus and Traumatic Brain Injury

It has become evident over the last years that abnormalities in RNA processing play a fundamental part in the pathogenesis of neurodegenerative diseases. Cellular viability depends on proper regulation of RNA metabolism and subsequent protein synthesis, which requires the interplay of many processes including transcription, pre--‐mRNA splicing, mRNA editing as well as mRNA stability, transport and translation. Dysfunction in any of these processes, often caused by mutations in the coding and non--‐ coding RNAs, can be very destructive to the cellular environment and consequently impair neural viability. The result of this RNA toxicity can lead to a toxic gain of function or a loss of function, depending on the nature of the mutation. For example, in repeat expansion disorders, such as the newly discovered hexanucleotide repeat expansion in theC9orf72 gene found in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), a toxic gain of function leads to the formation of RNA foci and the sequestration of RNA binding proteins (RBPs). This in return leads to a loss of function of those RBPs, which is hypothesized to play a significant part in the disease progression of ALS and FTD. Other toxicities arising from repeat expansions are the formation of RNA foci, bi--‐directional transcription and production of repeat associated non--‐ATG (RAN) translation products.This book will touch upon most of these disease mechanisms triggered by aberrant RNA metabolism and will therefore provide a broad perspective of the role of RNA processing and its dysfunction in a variety of neurodegenerative disorders, including ALS, FTD, Alzheimer's disease, Huntington's disease, spinal muscular atrophy, myotonic dystrophy and ataxias. The proposed authors are leading scientists in the field and are expected to not only discuss their own work, but to be inclusive of historic as well as late breaking discoveries. The compiled chapters willtherefore provide a unique collection of novel studies and hypotheses aimed to describe the consequences of altered RNA processing events and its newest molecular players and pathways.

Neurodegenerative diseases are widespread disorders, with prevalence increasing with age. The first chapter of this book discusses autophagy, a cellular tool for the elimination of proteins that accumulate in neurodegenerative disorders and have pathogenetic significance. Parkinson's disease (PD) is one of the most common degenerative diseases of the central nervous system. Chapter Two examines evidence on impairment of social cognition in patients with PD. Chapter Three discusses how finding the molecular cause of the PD could contribute to early diagnosis, and facilitate the development of effective neuroprotective or modulatory drugs. The final chapter reviews the functional role of adult hippocampal neurogenesis in learning and memory, and how this form of structural plasticity is altered in neurodegenerative diseases known to involve cognitive impairment such as Alzheimer's disease, Parkinson's disease, and Huntington's disease.

Prion Protein, Volume 150, the latest volume in the Progress in Molecular Biology and Translational Science series, focuses on Prion Protein, a protein that is considered to be the archetype of intrinsically disordered proteins. This updated volume includes comprehensive sections on a variety of timely topics, including Functions of Prion Protein, Copper and Prion protein, Cell Biology of Prion Protein, Structural Studies of Prion Proteins, Molecular Simulations on Wild Type and Mutant Prion Proteins, Genetics of Prion Protein, The Prion Concept and Synthetic Prions, and Transgenic Mouse Models. As neurodegenerative diseases represent a health issue that is receiving increasing attention from the scientific community due to their social and economic impact, this series is an ideal resource for the latest research in molecular biology and translational science. - Presents the latest volume in the Progress in Molecular Biology and Translational Science series - Accessible to students and researcher alike - Written by leading authorities in the field of prion protein

Disease-Modifying Targets in Neurodegenerative Disorders: Paving the Way for Disease-Modifying Therapies examines specific neurodegenerative disorders in comprehensive chapters written by experts in the respective fields. Each chapter contains a summary of the disease management field, subsequently elaborating on the molecular mechanisms and promising new targets for disease-modifying therapies. This overview is ideal for neuroscientists, biomedical researchers, medical doctors, and caregivers, not only providing readers with a summary of the way patients are treated today, but also offering a glance at the future of neurodegenerative disorder treatment. - Provides a comprehensive overview of how key proteins in neurodegenerative disorders can be used as targets to modify disease progress - Summarizes how patients are treated today, providing a glance at future disease management - Includes intelligible and informative information that is perfect for non-specialists, medical practitioners, and scientists - Written and peer reviewed by outstanding scientists in their respective fields

Provides a timely overview of critical advances in molecular and cellular neurobiology, covers key methodologies driving progress, and highlights key future directions for research on neuronal injury and neurodegeneration relevant to neuronal brain pathologies. The editors bring together contributions from internationally recognized workers in the field to provide an up to date account of how and why molecular and cellular neurobiology is such an important area for clinical neuroscience. Understanding the molecular aspects of a number of neurodegenerative conditions such as Parkinson's or Alzheimer's disease for the purpose of improving patient management remains a major challenge of neurobiology be it from the basic or clinical perspective. A strategic evaluation of research contributions and the power of modern methods will help advance knowledge over the next years.

Craniospinal Hydrodynamics in Neurodegenerative and Neurological Disorders is for students and doctors of chiropractic, osteopathy, neurology, neurosurgery, physical therapists, and researchers interested in finding the causes, new treatments, potential cures and prevention of neurodegenerative and neurological conditions related to faulty craniospinal hydrodynamics. This book proposes a completely new theory regarding the causative and contributory roles of faulty craniospinal hydrodynamics and morphology in neurodegenerative and neurological conditions. Relevant morphology includes malformations and misalignments in the craniocervical junction and spondylosis, stenosis, scoliosis and pelvic obliquity in the lower spine. Malformations of the craniocervical skeleton include: Chiari malformation, Dandy-Walker syndrome, mega cisterna magna, occipitalization, malformations of the dens, platybasia, basilar invagination, hypoplasia of the posterior fossa, craniosynostosis, craniofacial anomalies and more. Malformations of the lower spine include: Tarlov cysts, spina bifida cystica, tethered cord, short leg syndrome and others. A chapter on whiplash and traumatic brain and cord injuries is included, as well as a chapter on microgravity and hydrocephalus, and the latter's similarities to glaucoma. Finally, there is a chapter on treatment. Conditions include hydrocephalus, Chiari malformations, Dandy-Walker syndrome, Alzheimer's disease, Parkinson's disease, Parkinson's Plus, multiple sclerosis, amyotrophic lateral sclerosis, Huntington's disease, midbrain/Parinaud's syndrome, idiopathic intracranial hypertension, optic neuritis, transverse myelitis, migraines, epilepsy, silent strokes and more. The goal of this book is to inspire new directions and collaborations in research, treatment and prevention, as well as co-management of the above conditions.

Originally confused with opioid receptors and then orphan receptors with no biological function, Sigma Receptors are now recognized as relevant to many degenerative diseases with remarkable potential as therapeutic targets. In this text, new information about the structure of sigma 1 receptor, its binding sites are provided as well as its expression in many cell types. It's putative role in degenerative neuronal diseases including amyotrophic lateral sclerosis, Alzheimer's disease, Parkinson's disease, Huntington's disease, pain, drug addiction and locomotor activity. Their roles in possible treatments for blinding retinal diseases emphasize the tremendous far-reaching potential for ligands for these receptors. Exciting breakthroughs in this dynamic field in the last decade are reported herein, which will guide future investigators in determining the full potential of this unique, yet abundantly expressed protein.

Biometals in Neurodegenerative Diseases: Mechanisms and Therapeutics is an authoritative and timely resource bringing together the major findings in the field for ease of access to those working in the field or with an interest in metals and their role in brain function, disease, and as therapeutic targets. Chapters cover metals in Alzheimer's Disease, Parkinson's Disease, Motor Neuron Disease, Autism and lysosomal storage disorders. This book is written for academic researchers, clinicians and advanced graduate students studying or treating patients in neurodegeneration, neurochemistry, neurology and neurotoxicology. The scientific literature in this field is advancing rapidly, with approximately 300 publications per year adding to our knowledge of how biometals contribute to neurodegenerative diseases. Despite this rapid increase in our understanding of biometals in brain disease, the fields of biomedicine and neuroscience have often overlooked this information. The need to bring the research on biometals in neurodegeneration to the forefront of biomedical research is essential in order to understand neurodegenerative disease processes and develop effective therapeutics. - Authoritative and timely resource bringing together the major findings in the field for those with an interest in metals and their role in the brain function, disease, and as therapeutic targets - Written for academic researchers, clinicians, and advanced graduate students studying, or treating, patients in neurodegeneration, neurochemistry, neurology and neurotoxicology - Edited by international leaders in the field who have contributed greatly to the study of metals in neurodegenerative diseases