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Huntington's chorea --- Molecular aspects. --- Pathogenesis.
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This fourth edition of Huntington's Disease presents a comprehensive summary of the current knowledge of this disease, including the major scientific and clinical advances that have occurred since publication of the third edition in 2002. Completely updated and expanded, chapters in this volume are organized in five sections: DT Clinical aspects of Huntington's disease, including updated chapters on historical perspectives, neurological, neuropsychiatric, and neuropsychological aspects, and new chapters on juvenile Huntington's and the premanifest and early stages DT The genetics of Huntington
Huntington's chorea. --- Chronic progressive chorea --- Chronic progressive hereditary chorea --- Degenerative chorea --- HC (Disease) --- HD (Disease) --- Hereditary chorea --- Huntington chorea --- Huntington chronic progressive hereditary chorea --- Huntington disease --- Huntington's chorea --- Lund-Huntington chorea --- Microcellular striatal syndrome --- Progressive hereditary chorea, Chronic --- Chorea --- Dementia --- Genetic disorders --- Nervous system --- Degeneration --- Huntington's disease.
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Huntington's chorea. --- Brain --- Brain diseases --- Psychology, Pathological --- Chronic progressive chorea --- Chronic progressive hereditary chorea --- Degenerative chorea --- HC (Disease) --- HD (Disease) --- Hereditary chorea --- Huntington chorea --- Huntington chronic progressive hereditary chorea --- Huntington disease --- Huntington's chorea --- Lund-Huntington chorea --- Microcellular striatal syndrome --- Progressive hereditary chorea, Chronic --- Chorea --- Dementia --- Genetic disorders --- Nervous system --- Diseases. --- Degeneration --- Huntington's disease.
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Huntington's disease (HD) is an inherited progressive neurodegenerative disorder. Although onset of Huntington's Disease usually occurs in adulthood, the age of onset of the condition is extremely variable with approximately 5-10% of cases having an onset of less than 20 years, or Juvenile Huntington's Disease (JHD). While JHD shares many of the clinical features of adult HD (e.g., chorea and personality disorders), patients with JHD often experience additional problems includingseizures, dystonia and Parkinsonism. Diagnosis in patients with JHD is often delayed because of the failure of clini
Huntington's chorea. --- Genetic disorders in children. --- Trinucleotide repeats. --- Repeats, Trinucleotide --- Repeats, Triplet (Nucleotides) --- Triplet repeats (Nucleotides) --- Microsatellites (Genetics) --- Nucleotides --- Children --- Chronic progressive chorea --- Chronic progressive hereditary chorea --- Degenerative chorea --- HC (Disease) --- HD (Disease) --- Hereditary chorea --- Huntington chorea --- Huntington chronic progressive hereditary chorea --- Huntington disease --- Huntington's chorea --- Lund-Huntington chorea --- Microcellular striatal syndrome --- Progressive hereditary chorea, Chronic --- Chorea --- Dementia --- Genetic disorders --- Nervous system --- Diseases --- Genetic aspects --- Degeneration --- Adolescent. --- Child. --- Huntington Disease. --- Trinucleotide Repeat Expansion. --- Huntington's disease .
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It is now almost a decade since the identification of the Huntington's Disease gene and its mutation, during which time, major advances in our understanding of this disorder have been achieved. Since publication of the first two editions of this book, there have been considerable insights into how the mutation leads to the molecular pathology, neuropathology and clinical symptoms of Huntington's Disease, and experimental tools are now in place to take this research further towards new therapeutic approaches. As a result of these major advances, this well-established series of books has required radical updating. An international group of researchers and clinicians with specialist interests in HD has been commissioned to document the recent advances in our understanding of this disease. Developments in the fields of structural biology, cell biology, neurochemistry and neuropathology, with full coverage of transgenic animal models, are discussed in detail. The clinical sections cover genetic, neurological and psychiatric aspects as well as new developments in therapy.; This book will continue to provide an invaluable source of information for clinicians and scientists involved with Huntington's Disease, including geneticists, psychiatrists and neurologists, and basic science research workers in genetics and neurobiology.
Huntington's disease --- Huntington Disease. --- Medicine --- Health & Biological Sciences --- Neurology --- Akinetic-Rigid Variant of Huntington Disease --- Chorea, Chronic Progressive Hereditary (Huntington) --- Chronic Progressive Hereditary Chorea (Huntington) --- Huntington Chronic Progressive Hereditary Chorea --- Huntington Disease, Akinetic-Rigid Variant --- Huntington Disease, Juvenile --- Huntington Disease, Juvenile-Onset --- Huntington Disease, Late Onset --- Huntington's Chorea --- Huntington's Disease --- Juvenile-Onset Huntington Disease --- Late-Onset Huntington Disease --- Progressive Chorea, Chronic Hereditary (Huntington) --- Progressive Chorea, Hereditary, Chronic (Huntington) --- Huntington Chorea --- Juvenile Huntington Disease --- Akinetic Rigid Variant of Huntington Disease --- Chorea, Huntington --- Chorea, Huntington's --- Huntington Disease, Akinetic Rigid Variant --- Huntington Disease, Juvenile Onset --- Huntington Disease, Late-Onset --- Juvenile Onset Huntington Disease --- Late Onset Huntington Disease --- Chronic progressive chorea --- Chronic progressive hereditary chorea --- Degenerative chorea --- HC (Disease) --- HD (Disease) --- Hereditary chorea --- Huntington chorea --- Huntington chronic progressive hereditary chorea --- Huntington disease --- Huntington's chorea --- Lund-Huntington chorea --- Microcellular striatal syndrome --- Progressive hereditary chorea, Chronic --- Chorea --- Dementia --- Genetic disorders --- Nervous system --- Degeneration --- Huntington's chorea.
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Huntington's disease is a progressive neurodegenerative disorder of the brain. It is one of the quite devastating and currently incurable human conditions. Degeneration of specific types of neurons in the brain results in a triad of clinical features: serious behavioral disturbances, uncontrolled movements of body parts, and deterioration of intellectual capabilities. The underlying complex mechanisms and molecular players of the cellular cascades still need to be deciphered in detail despite considerable advances. Once solved, the related molecular mechanisms will not only enlighten the HD story but will also shed light on other polyglutamine diseases and similar brain disorders. This book, Huntington's Disease-Molecular Pathogenesis and Current Models, is planned to cover recent scientific achievements in understanding the cellular mechanisms of HD. The chapters provide comprehensive description of the key issues in HD research. In this regard, this book will serve as a source for clinicians and researchers in the field and also for life science readers in increasing their understanding and awareness of the clinical correlates, genetic aspects, neuropathological findings, and potential therapeutic interventions related to HD.
Huntington's disease. --- Chronic progressive chorea --- Chronic progressive hereditary chorea --- Degenerative chorea --- HC (Disease) --- HD (Disease) --- Hereditary chorea --- Huntington chorea --- Huntington chronic progressive hereditary chorea --- Huntington disease --- Huntington's chorea --- Lund-Huntington chorea --- Microcellular striatal syndrome --- Progressive hereditary chorea, Chronic --- Chorea --- Dementia --- Genetic disorders --- Nervous system --- Degeneration --- Medicine --- Neurology --- Mental and Behavioural Disorders and Diseases of the Nervous System --- Health Sciences
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This book summarizes the most recent findings related to Huntington Disease, providing coverage for clinical/research specialists looking to expand their knowledge, as well as groundwork on the disease for advanced students from various backgrounds, including neurology, psychiatry, neuropsychology, and genetics.
Huntington's disease. --- Chronic progressive chorea --- Chronic progressive hereditary chorea --- Degenerative chorea --- HC (Disease) --- HD (Disease) --- Hereditary chorea --- Huntington chorea --- Huntington chronic progressive hereditary chorea --- Huntington disease --- Huntington's chorea --- Lund-Huntington chorea --- Microcellular striatal syndrome --- Progressive hereditary chorea, Chronic --- Chorea --- Dementia --- Genetic disorders --- Nervous system --- Degeneration
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'This is a moving true story of how people can find the inner strength to rise above it when their world is turned upside down.'. - Richard Branson. Huntington's Disease (HD) is a hereditary illness passed on via a defective gene. There is a fifty per cent chance of inheriting it from a parent and there is yet no cure. Learning to Live with Huntington's Disease is one family's poignant story of coping with the symptoms, the diagnosis and the effects of HD. This book presents the struggles and strengths of the whole family when one member loses their future to a terminal illness. Told by the su
Huntington's chorea --- Chronic progressive chorea --- Chronic progressive hereditary chorea --- Degenerative chorea --- HC (Disease) --- HD (Disease) --- Hereditary chorea --- Huntington chorea --- Huntington chronic progressive hereditary chorea --- Huntington disease --- Lund-Huntington chorea --- Microcellular striatal syndrome --- Progressive hereditary chorea, Chronic --- Chorea --- Dementia --- Genetic disorders --- Nervous system --- Patients --- Family relationships. --- Degeneration --- Sulaiman, Sandy, --- Sulaiman, Sandra Margaret, --- Health.
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When Phebe Hedges, a woman in East Hampton, New York, walked into the sea in 1806, she made visible the historical experience of a family affected by the dreaded disorder of movement, mind, and mood her neighbors called St.Vitus's dance. Doctors later spoke of Huntington's chorea, and today it is known as Huntington's disease. This book is the first history of Huntington's in America. Starting with the life of Phebe Hedges, Alice Wexler uses Huntington's as a lens to explore the changing meanings of heredity, disability, stigma, and medical knowledge among ordinary people as well as scientists and physicians. She addresses these themes through three overlapping stories: the lives of a nineteenth-century family once said to "belong to the disease"; the emergence of Huntington's chorea as a clinical entity; and the early-twentieth-century transformation of this disorder into a cautionary eugenics tale. In our own era of expanding genetic technologies, this history offers insights into the social contexts of medical and scientific knowledge, as well as the legacy of eugenics in shaping both the knowledge and the lived experience of this disease.
Huntington's chorea --- Chronic progressive chorea --- Chronic progressive hereditary chorea --- Degenerative chorea --- HC (Disease) --- HD (Disease) --- Hereditary chorea --- Huntington chorea --- Huntington chronic progressive hereditary chorea --- Huntington disease --- Lund-Huntington chorea --- Microcellular striatal syndrome --- Progressive hereditary chorea, Chronic --- Chorea --- Dementia --- Genetic disorders --- Nervous system --- History --- Degeneration --- Hedges, Phebe, --- Huntington, George, --- Muncey, E. B. --- Muncey, Elizabeth B. --- Tillinghast, Phebe,
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Huntington's disease. --- Chronic progressive chorea --- Chronic progressive hereditary chorea --- Degenerative chorea --- HC (Disease) --- HD (Disease) --- Hereditary chorea --- Huntington chorea --- Huntington chronic progressive hereditary chorea --- Huntington disease --- Huntington's chorea --- Lund-Huntington chorea --- Microcellular striatal syndrome --- Progressive hereditary chorea, Chronic --- Chorea --- Dementia --- Genetic disorders --- Nervous system --- Degeneration
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