Código: 6711

Livro Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders

Marca: Wiley-Blackwell Modelo: 9781405196932 Referência: 9781405196932

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Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders
Autor: Dennis Dickson

Especialidade: NEUROLOGIA
ISBN: 1405196939
ISBN13: 9781405196932
Páginas: 496
Publicação: 2012
Edição: 2 º
Encadernação: Capa Dura

 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.

List of Contributors, viii
Preface, xii
List of Abbreviations, xiii
Part 1 Introduction: Basic Mechanisms of Neurodegeneration
1 Introduction to Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders, 3
2 Cell Death and Neurodegeneration, 6
3 Oxidative Stress and Balance in Neurodegenerative Diseases, 10
4 Protein Aggregation in Neurodegeneration, 13
5 Protein Degradation in Neurodegeneration: The Ubiquitin Pathway, 18
6 Genetics of Neurodegeneration, 22
7 Transgenic Animal Models of Proteinopathies, 26
Part 2 Alzheimer's Disease and Aging
8 Clinical Aspects of Alzheimer's Disease, 39
9 Genetics of Alzheimer's Disease, 51
10 Neuropathology of Alzheimer's Disease and its Variants, 62
11 Amyloid-ß Production, 92
12 Elimination of Amyloid ß from the Brain, its Failure in Alzheimer's Disease and Implications for Therapy, 97
Part 3 Tauopathies
13 Introduction to the Tauopathies, 105
14 Frontotemporal Dementia and Parkinsonism Linked to Chromosome 17, 110
15 Progressive Supranuclear Palsy and Corticobasal Degeneration, 135
16 Pick's Disease, 156
17 Argyrophilic Grain Disease, 165
18 Parkinsonism–Dementia Complex of Guam, 171
19 Postencephalitic Parkinsonism, 179
Part 4 Synucleinopathies
20 Introduction to a-Synucleinopathies, 191
21 Parkinson's Disease, 194
22 Dementia with Lewy Bodies and Parkinson's Disease Dementia, 224
23 Lewy Bodies in Conditions other than Disorders of a-Synuclein, 238
24 Multiple System Atrophy, 242
Part 5 Trinucleotide Repeat Disorders
25 Introduction to Trinucleotide Repeat Diseases, 255
26 Huntington's Disease, 258
27 Spinocerebellar Ataxias, 273
28 Friedreich's Ataxia, 288
29 Dentatorubral-pallidoluysian Atrophy, 299
Hitoshi Takahashi, Mitsunori Yamada and Shoji Tsuji
30 Spinal and Bulbar Muscular Atrophy, 307
Part 6 Prion Disorders
31 Introduction to Prion Disorders, 315
32 Sporadic Creutzfeldt–Jakob Disease, 322
33 Genetic Creutzfeldt–Jakob Disease, 336
34 Fatal Familial and Sporadic Insomnia, 346
35 A New Prion Disease: Protease-Sensitive Prionopathy, 350
36 Variant Creutzfeldt–Jakob Disease, 354
37 Gerstmann–Sträussler–Scheinker Disease, 364
38 Kuru, 378
39 Iatrogenic Creutzfeldt–Jakob Disease, 381
Part 7 Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis/Motor Neuron Disease
40 Introduction, 389
41 Frontotemporal Lobar Degeneration with TDP-43 Pathology, 393
42 Neuronal Intermediate Filament Inclusion Disease, 404
43 Frontotemporal Lobar Degeneration with FUS Immunoreactive Inclusions, 412
44 Amyotrophic Lateral Sclerosis, Primary Lateral Sclerosis and Spinal Muscular Atrophy, 418
Part 8 Other Neurodegenerative Disorders
45 Introduction: Genetic Analysis as a Lumper and Splitter in Neurodegenerative Disease, 437
46 Inherited Amyloidoses and Neurodegeneration: Familial British Dementia and Familial Danish Dementia, 439
47 Neurodegeneration with Brain Iron Accumulation, 446
48 Familial Encephalopathy with Neuroserpin Inclusion Bodies, 456
49 Hereditary Ferritinopathies, 461
Index, 467


Livro Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders

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