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
Dennis W. Dickson
2 Cell Death and Neurodegeneration, 6
Violetta N. Pivtoraiko and Kevin A. Roth
3 Oxidative Stress and Balance in Neurodegenerative Diseases, 10
George Perry, Siddhartha Mondragón-Rodríguez, Akihiko Nunomura, Xiongwei Zhu, Paula I. Moreira and Mark A. Smith
4 Protein Aggregation in Neurodegeneration, 13
Adriano Aguzzi and Veronika Kana
5 Protein Degradation in Neurodegeneration: The Ubiquitin Pathway, 18
Lynn Bedford, Robert Layfi eld, Nooshin Rezvani, Simon Paine, James Lowe and R. John Mayer
6 Genetics of Neurodegeneration, 22
John Hardy
7 Transgenic Animal Models of Proteinopathies, 26
Naruhiko Sahara, Heather Melrose, Simon D'Alton and Jada Lewis
Part 2 Alzheimer's Disease and Aging
8 Clinical Aspects of Alzheimer's Disease, 39
David Knopman
9 Genetics of Alzheimer's Disease, 51
Lars Bertram and Rudolph E. Tanzi
10 Neuropathology of Alzheimer's Disease and its Variants, 62
Charles Duyckaerts and Dennis Dickson
11 Amyloid-ß Production, 92
Colin L. Masters and Konrad Beyreuther
12 Elimination of Amyloid ß from the Brain, its Failure in Alzheimer's Disease and Implications for Therapy, 97
Roy O. Weller, Seth Love and James A.R. Nicoll
Part 3 Tauopathies
13 Introduction to the Tauopathies, 105
Michel Goedert
14 Frontotemporal Dementia and Parkinsonism Linked to Chromosome 17, 110
Bernardino Ghetti, Zbigniew K. Wszolek, Bradley F. Boeve, Salvatore Spina and Michel Goedert
15 Progressive Supranuclear Palsy and Corticobasal Degeneration, 135
Dennis W. Dickson, Jean-Jaques Hauw, Yves Agid and Irene Litvan
16 Pick's Disease, 156
David G. Munoz, Huw R. Morris and Martin Rossor
17 Argyrophilic Grain Disease, 165
Markus Tolnay and Heiko Braak
18 Parkinsonism-Dementia Complex of Guam, 171
Kiyomitsu Oyanagi, Tomoyo Hashimoto and Mineo Yamazaki
19 Postencephalitic Parkinsonism, 179
Kurt A. Jellinger
Part 4 Synucleinopathies
20 Introduction to a-Synucleinopathies, 191
Maria Grazia Spillantini
21 Parkinson's Disease, 194
Kurt A. Jellinger
22 Dementia with Lewy Bodies and Parkinson's Disease Dementia, 224
Paul G. Ince
23 Lewy Bodies in Conditions other than Disorders of a-Synuclein, 238
Coro Paisán-Ruiz, Laura Parkkinen and Tamas Revesz
24 Multiple System Atrophy, 242
Janice L. Holton, Andrew J. Lees and Tamas Revesz
Part 5 Trinucleotide Repeat Disorders
25 Introduction to Trinucleotide Repeat Diseases, 255
H. Brent Clark
26 Huntington's Disease, 258
John C. Hedreen and Raymund A.C. Roos
27 Spinocerebellar Ataxias, 273
Hidehiro Mizusawa, H. Brent Clark and Arnulf H. Koeppen
28 Friedreich's Ataxia, 288
Arnulf H. Koeppen
29 Dentatorubral-pallidoluysian Atrophy, 299
Hitoshi Takahashi, Mitsunori Yamada and Shoji Tsuji
30 Spinal and Bulbar Muscular Atrophy, 307
Gen Sobue, Hiroaki Adachi and Masahisa Katsuno
Part 6 Prion Disorders
31 Introduction to Prion Disorders, 315
Adriano Aguzzi and Veronika Kana
32 Sporadic Creutzfeldt-Jakob Disease, 322
Herbert Budka, Mark W. Head, James W. Ironside, Pierluigi Gambetti, Piero Parchi and Fabrizio Tagliavini
33 Genetic Creutzfeldt-Jakob Disease, 336
Piero Parchi, Pierluigi Gambetti and Sabina Capellari
34 Fatal Familial and Sporadic Insomnia, 346
Piero Parchi, Sabina Capellari and Pierluigi Gambetti
35 A New Prion Disease: Protease-Sensitive Prionopathy, 350
Pierluigi Gambetti, Gianfranco Puoti, Qingzhong Kong and Wenquan Zou
36 Variant Creutzfeldt-Jakob Disease, 354
James W. Ironside, Mark W. Head and Robert G. Will
37 Gerstmann-Sträussler-Scheinker Disease, 364
Bernardino Ghetti, Fabrizio Tagliavini, Gabor G. Kovacs and Pedro Piccardo
38 Kuru, 378
Catriona Ann McLean
39 Iatrogenic Creutzfeldt-Jakob Disease, 381
James W. Ironside, Richard S.G. Knight and Mark W. Head
Part 7 Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis/Motor Neuron Disease
40 Introduction, 389
James Lowe
41 Frontotemporal Lobar Degeneration with TDP-43 Pathology, 393
Ian R.A. Mackenzie and Manuela Neumann
42 Neuronal Intermediate Filament Inclusion Disease, 404
Nigel J. Cairns
43 Frontotemporal Lobar Degeneration with FUS Immunoreactive Inclusions, 412
Manuela Neumann and Ian R.A. Mackenzie
44 Amyotrophic Lateral Sclerosis, Primary Lateral Sclerosis and Spinal Muscular Atrophy, 418
Michael J. Strong, Tibor Hortobágyi, Koichi Okamoto and Shinsuke Kato
Part 8 Other Neurodegenerative Disorders
45 Introduction: Genetic Analysis as a Lumper and Splitter in Neurodegenerative Disease, 437
John E. Duda
46 Inherited Amyloidoses and Neurodegeneration: Familial British Dementia and Familial Danish Dementia, 439
Tamas Revesz, Agueda Rostagno, Gordon Plant, Tammaryn Lashley, Blas Frangione, Jorge Ghiso and Janice L. Holton
47 Neurodegeneration with Brain Iron Accumulation, 446
John E. Duda and Kurt A. Jellinger
48 Familial Encephalopathy with Neuroserpin Inclusion Bodies, 456
Richard L. Davis and George H. Collins
49 Hereditary Ferritinopathies, 461
Ruben Vidal, Marie Bernadette Delisle, Olivier Rascol and Bernardino Ghetti
Index, 467
