Neurodegeneration

Volume Editor: Dr. Dennis W. Dickson, Department of Pathology, Neuropathology Laboratory, Mayo Clinic, Jacksonville, Florida, USA.

• List of Contributors, viii Preface, xiiList of Abbreviations, xiiiPart 1 Introduction: Basic Mechanisms of Neurodegeneration1 Introduction to Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders, 3Dennis W. Dickson2 Cell Death and Neurodegeneration, 6Violetta N. Pivtoraiko and Kevin A. Roth3 Oxidative Stress and Balance in Neurodegenerative Diseases, 10George Perry, Siddhartha Mondragón-Rodríguez, Akihiko Nunomura, Xiongwei Zhu, Paula I. Moreira and Mark A. Smith4 Protein Aggregation in Neurodegeneration, 13Adriano Aguzzi and Veronika Kana5 Protein Degradation in Neurodegeneration: The Ubiquitin Pathway, 18Lynn Bedford, Robert Layfi eld, Nooshin Rezvani, Simon Paine, James Lowe and R. John Mayer6 Genetics of Neurodegeneration, 22John Hardy7 Transgenic Animal Models of Proteinopathies, 26Naruhiko Sahara, Heather Melrose, Simon D'Alton and Jada LewisPart 2 Alzheimer's Disease and Aging8 Clinical Aspects of Alzheimer's Disease, 39David Knopman9 Genetics of Alzheimer's Disease, 51Lars Bertram and Rudolph E. Tanzi10 Neuropathology of Alzheimer's Disease and its Variants, 62Charles Duyckaerts and Dennis Dickson11 Amyloid-ß Production, 92Colin L. Masters and Konrad Beyreuther12 Elimination of Amyloid ß from the Brain, its Failure in Alzheimer's Disease and Implications for Therapy, 97Roy O. Weller, Seth Love and James A.R. NicollPart 3 Tauopathies13 Introduction to the Tauopathies, 105Michel Goedert14 Frontotemporal Dementia and Parkinsonism Linked to Chromosome 17, 110Bernardino Ghetti, Zbigniew K. Wszolek, Bradley F. Boeve, Salvatore Spina and Michel Goedert15 Progressive Supranuclear Palsy and Corticobasal Degeneration, 135Dennis W. Dickson, Jean-Jaques Hauw, Yves Agid and Irene Litvan16 Pick's Disease, 156David G. Munoz, Huw R. Morris and Martin Rossor17 Argyrophilic Grain Disease, 165Markus Tolnay and Heiko Braak18 Parkinsonism–Dementia Complex of Guam, 171Kiyomitsu Oyanagi, Tomoyo Hashimoto and Mineo Yamazaki19 Postencephalitic Parkinsonism, 179Kurt A. JellingerPart 4 Synucleinopathies20 Introduction to a-Synucleinopathies, 191Maria Grazia Spillantini21 Parkinson's Disease, 194Kurt A. Jellinger22 Dementia with Lewy Bodies and Parkinson's Disease Dementia, 224Paul G. Ince23 Lewy Bodies in Conditions other than Disorders of a-Synuclein, 238Coro Paisán-Ruiz, Laura Parkkinen and Tamas Revesz24 Multiple System Atrophy, 242Janice L. Holton, Andrew J. Lees and Tamas ReveszPart 5 Trinucleotide Repeat Disorders25 Introduction to Trinucleotide Repeat Diseases, 255H. Brent Clark26 Huntington's Disease, 258John C. Hedreen and Raymund A.C. Roos27 Spinocerebellar Ataxias, 273Hidehiro Mizusawa, H. Brent Clark and Arnulf H. Koeppen28 Friedreich's Ataxia, 288Arnulf H. Koeppen29 Dentatorubral-pallidoluysian Atrophy, 299Hitoshi Takahashi, Mitsunori Yamada and Shoji Tsuji30 Spinal and Bulbar Muscular Atrophy, 307Gen Sobue, Hiroaki Adachi and Masahisa KatsunoPart 6 Prion Disorders31 Introduction to Prion Disorders, 315Adriano Aguzzi and Veronika Kana32 Sporadic Creutzfeldt–Jakob Disease, 322Herbert Budka, Mark W. Head, James W. Ironside, Pierluigi Gambetti, Piero Parchi and Fabrizio Tagliavini33 Genetic Creutzfeldt–Jakob Disease, 336Piero Parchi, Pierluigi Gambetti and Sabina Capellari34 Fatal Familial and Sporadic Insomnia, 346Piero Parchi, Sabina Capellari and Pierluigi Gambetti35 A New Prion Disease: Protease-Sensitive Prionopathy, 350Pierluigi Gambetti, Gianfranco Puoti, Qingzhong Kong and Wenquan Zou36 Variant Creutzfeldt–Jakob Disease, 354James W. Ironside, Mark W. Head and Robert G. Will37 Gerstmann–Sträussler–Scheinker Disease, 364Bernardino Ghetti, Fabrizio Tagliavini, Gabor G. Kovacs and Pedro Piccardo38 Kuru, 378Catriona Ann McLean39 Iatrogenic Creutzfeldt–Jakob Disease, 381James W. Ironside, Richard S.G. Knight and Mark W. HeadPart 7 Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis/Motor Neuron Disease40 Introduction, 389James Lowe41 Frontotemporal Lobar Degeneration with TDP-43 Pathology, 393Ian R.A. Mackenzie and Manuela Neumann42 Neuronal Intermediate Filament Inclusion Disease, 404Nigel J. Cairns43 Frontotemporal Lobar Degeneration with FUS Immunoreactive Inclusions, 412Manuela Neumann and Ian R.A. Mackenzie44 Amyotrophic Lateral Sclerosis, Primary Lateral Sclerosis and Spinal Muscular Atrophy, 418Michael J. Strong, Tibor Hortobágyi, Koichi Okamoto and Shinsuke KatoPart 8 Other Neurodegenerative Disorders45 Introduction: Genetic Analysis as a Lumper and Splitter in Neurodegenerative Disease, 437John E. Duda46 Inherited Amyloidoses and Neurodegeneration: Familial British Dementia and Familial Danish Dementia, 439Tamas Revesz, Agueda Rostagno, Gordon Plant, Tammaryn Lashley, Blas Frangione, Jorge Ghiso and Janice L. Holton47 Neurodegeneration with Brain Iron Accumulation, 446John E. Duda and Kurt A. Jellinger48 Familial Encephalopathy with Neuroserpin Inclusion Bodies, 456Richard L. Davis and George H. Collins49 Hereditary Ferritinopathies, 461Ruben Vidal, Marie Bernadette Delisle, Olivier Rascol and Bernardino GhettiIndex, 467

“Overall, this textbook provides useful and systematic information on the basic mechanisms of neurodegenerative diseases.  It will provide useful for resident trainees, neurologists and pathologists.”  (Canadian Journal of Neurological Sciences, 1 November 2012)