Human Prion Diseases


Book Description

Human Prion Diseases, Volume 153 is designed to update the reader on the latest advances and clinical aspects of prion diseases. The book is organized into five sections, including the pathophysiology of prions and a description of animal and human diseases. This is followed by detailed reports on recent advances in diagnosis strategies for the development of novel anti-prion molecules and possible designs of clinical trials in such a rare disease. An introductory chapter gives an extensive historical background of prion research, with a final chapter highlighting recent progress, and more importantly, unsolved problems. - Offers an authoritative overview of prion diseases in humans, detailing the pathogenesis of the disease, clinical investigations, and the diagnosis of both the genetic and acquired forms - Provides clarity and context by presenting prion diseases in relation to other neurodegenerative diseases in humans - Emphasizes the unique properties of prion diseases and consequent problems they can cause, both clinically and in public health terms




Advancing Prion Science


Book Description

In Advancing Prion Science, the Institute of Medicine's Committee on Transmissible Spongiform Encephalopathies Assessment of Relevant Science recommends priorities for research and investment to the Department of Defense's National Prion Research Program (NPRP). Transmissible spongiform encephalopathies (TSEs), also called prion diseases, are invariably fatal neurodegenerative infectious diseases that include bovine spongiform encephalopathy (commonly called mad cow disease), chronic wasting disease, scrapie, and Creutzfeldt-Jakob disease. To develop antemortem diagnostics or therapies for TSEs, the committee concludes that NPRP should invest in basic research specifically to elucidate the structural features of prions, the molecular mechanisms of prion replication, the mechanisms of TSE pathogenesis, and the physiological function of prions' normal cellular isoform. Advancing Prion Science provides the first comprehensive reference on present knowledge about all aspects of TSEs' from basic science to the U.S. research infrastructure, from diagnostics to surveillance, and from prevention to treatment. This report summarizes the progress thus far.




Prions Prions Prions


Book Description

A series of remarkable discoveries in the past three decades have led to the molecular and genetic characterization of the transmissible pathogen causing scrapie in animals and a quartet of human illnesses: kuru, Jakob-Creutzfeld disease, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia. To distinguish this pathogen from viruses and viroids, the term "prion" was introduced to emphasize its proteinaceous and infectious nature. Stanley B. Prusiner, editor of this volume, was awarded the 1997 Nobel Prize in Physiology and Medicine for his pioneering discovery of prions. The book reviews advances in studies of prions, which - as considereable evidence indicates - are novel pathogens composed only of protein.




Harrison's Neurology in Clinical Medicine, 3E


Book Description

Neurology – as only Harrison’s can cover it Featuring a superb compilation of chapters related to neurology that appear in Harrison’s Principles of Internal Medicine, Eighteenth Edition, this concise, full-color clinical companion delivers the latest knowledge in the field backed by the scientific rigor and authority that have defined Harrison’s. You will find content from renowned editors and contributors in a carry-anywhere presentation that is ideal for the classroom, clinic, ward, or exam/certification preparation. Features Current, complete coverage of clinically important topics in neurology, including Clinical Manifestations of Neurologic Diseases, Diseases of the Nervous System, Chronic Fatigue Syndrome, Psychiatric Disorders, and Alcoholism and Drug Dependency NEW CHAPTERS discuss the pathogenesis and treatment and syncope; dizziness and vertigo; peripheral neuropathy; neuropsychiatric problems among war veterans; and advances in deciphering the pathogenesis of common psychiatric disorders Integration of pathophysiology with clinical management 118 high-yield questions and answers drawn from Harrison’s Principles of Internal Medicine Self-Assessment and Board Review, 18e Content updates and new developments since the publication of Harrison’s Principles of Internal Medicine, 18e 58 chapters written by physicians who are recognized experts in the field of clinical neurology Helpful appendix of laboratory values of clinical importance




Prions


Book Description

Prion-related diseases, known as transmissible spongiform encephalopathies (TSEs), are infectious, fatal neurodegenerative disorders for which there is no cure, treatment, nor even a means for early diagnosis. The horrific advent of Mad Cow Disease -- transmitted to humans through eating meat from steers sickened by bovine spongiform encephalopathy




Madness and Memory


Book Description

The author, a 1997 recipient of the Noble Prize in medicine, describes the years he spent researching and demonstrating how the infectious proteins known as prions were responsible for brain diseases and how his theory has now become widely accepted in the science establishment.




Neurodegeneration and Prion Disease


Book Description

This is the first and only book on the subject of prions to cover the cause of cell death in the disease. It covers the full range of competing theories on the subject, from broad description and basic points up to the final details of the basic science.




Neurogenetics, Part II


Book Description

Neurogenetics, Part II, Volume 148, the latest release in the Handbook of Clinical Neurology, provides the latest information on the genetic methodologies that are having a significant impact on the study of neurological and psychiatric disorders. Using genetic science, researchers have identified over 200 genes that cause or contribute to neurological disorders. Still an evolving field of study, defining the relationship between genes and neurological and psychiatric disorders is expected to dramatically grow in scope. Part II builds on the foundation of Part I, expanding the coverage to dementias, paroxysmal disorders, neuromuscular disorders, white matter and demyelination diseases, cerebrovascular diseases, adult psychiatric disorders and cancer and phacomatoses. - Contains comprehensive coverage of neurogenetics - Details the latest science and its impact on our understanding of neurological, psychiatric disorders - Presents a focused reference for clinical practitioners and the neuroscience/neurogenetics research community




Prions and Mad Cow Disease


Book Description

The alarm sounded by Canada's confirmed case of bovine spongiform encephalopathy (BSE) has reaffirmed the exigency of establishing improved safeguards and more aggressive surveillance protocols in North America and around the world. Research converging on the probable causative agent--prion proteins--calls for intensive assessment of the headway gained in tracing prions, testing for transmissible neurodegenerative diseases, and developing methods for cornering the epidemic. With an illustrious panel of 36 international contributors, this timely book marshals techniques for prion protein assay and diagnosis of transmissible spongiform encephalopathies (TSEs).




Brain Trust


Book Description

When the cattle-borne sickness known as Mad Cow Disease first appeared in America in 2003, authorities were quick to assure the nation that the outbreak was isolated, quarantined, and posed absolutely no danger to the general public. What we were not told was that the origins of the sickness may already have been here and suspected for a quarter of a century. This illuminating exposé of the threat to our nation's health reveals for the first time how Mad Cow Disease (a.k.a. Bovine Spongiform Encephalopathy) has jumped species, infecting humans in the form of Creutzfeldt-Jakob Disease (CJD), and may be hidden in the enormous increase in the number of Alzheimer's cases since 1979. Detailing the history and biology of Mad Cow Disease, Brain Trust discloses how an investigation into the mysterious deaths in a group of cannibals in a remote part of the world evolved into a research program in the United States that may have had unforeseen and frightening consequences. The shocking questions examined include: • Have millions of Americans already been exposed to the prions known to cause Mad Cow Disease through years of eating tainted beef? • Does the epidemic of prion disease spreading like wildfire through the nation's deer and elk pose a threat to hunters and venison eaters? • Are the cattle mutilations discovered in the last 30 years part of a covert, illegal sampling program designed to learn how far the deadly prions have spread throughout the nation's livestock and beef products? Exposing the devastating truth about Mad Cow Disease and a new theory of the possible consequences of a little-known government research program and the potential national health catastrophe that may be the result, Brain Trust inoculates Americans with an effective cure: the truth.