Table of Contents
Table of Contents
This edition first published 2011, © 2003, 2011 by International Society of Neuropathology
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List of Contributors
Hiroaki Adachi
Department of Neurology
Nagoya University Graduate School of Medicine
Nagoya, Japan
Yves Agid
Institut du Cerveau et de la Moelle épinière
Hôpital Pitié-Salpêtrière
Paris, France
Adriano Aguzzi
Institute of Neuropathology
University Hospital Zürich
Zürich, Switzerland
Lynn Bedford
School of Biomedical Sciences
University of Nottingham Medical School
Queen’s Medical Centre
Nottingham, UK
Lars Bertram
Neuropsychiatric Genetics Group
Department of Vertebrate Genomics
Max-Planck Institute for Molecular Genetics
Berlin, Germany
Konrad Beyreuther
Network for Aging Research
University of Heidelberg
Heidelberg, Germany
Bradley F. Boeve
Department of Neurology
Mayo Clinic
Rochester, MN, USA
Heiko Braak
Center for Clinical Research
Department of Neurology
University of Ulm
Ulm, Germany
Herbert Budka
Institute of Neurology
Medical University Vienna
Vienna, Austria
Nigel J. Cairns
Department of Pathology and Immunology
Washington University School of Medicine
St Louis, MO, USA
Sabina Capellari
Department of Neurological Sciences
University of Bologna
Bologna, Italy
H. Brent Clark
Department of Laboratory Medicine and Pathology
University of Minnesota Medical School
Minneapolis, MN, USA
George H. Collins
Department of Pathology
SUNY Upstate Medical University
Syracuse, NY, USA
Simon D’Alton
Center for Translational Research of Neurodegenerative Disease and Department of Neuroscience
University of Florida
Gainesville, FL, USA
Richard L. Davis
Department of Pathology
SUNY Upstate Medical University
Syracuse, NY, USA
Marie Bernadette Delisle
University Hospital of Toulouse
Toulouse, France
Dennis W. Dickson
Department of Pathology (Neuropathology) and Neuroscience
Mayo Clinic
Jacksonville, FL, USA
John E. Duda
Parkinson’s Disease Research, Education and Clinical Center
Philadelphia VA Medical Center, and Department of Neurology
University of Pennsylvania School of Medicine
Philadelphia, PA USA
Charles Duyckaerts
Raymond Escourolle Neuropathology Laboratory
Hôpital de la Salpêtrière
Paris, France
Blas Flangione
Department of Pathology
New York University School of Medicine
New York, NY, USA
Pierluigi Gambetti
Institute of Pathology and
National Prion Disease Pathology Surveillance Center
Case Western Reserve University
Cleveland, OH, USA
Bernardino Ghetti
Department of Pathology and Laboratory Medicine
Indiana University School of Medicine
Indianapolis, IN, USA
Jorge Ghiso
Department of Pathology
New York University School of Medicine
New York, NY, USA
Michel Goedert
Medical Research Council Laboratory of Molecular Biology
Cambridge, UK
Maria Grazia Spillantini
Brain Repair Centre
Department of Clinical Neurosciences
University of Cambridge
Cambridge, UK
John Hardy
Department of Molecular Neuroscience and Reta Lilla Weston Laboratories
Institute of Neurology
Queen Square
London, UK
Tomoyo Hashimoto
Department of Neuropathology
Tokyo Metropolitan Institute for Neuroscience;
Department of Safety and Health
Tokyo Gas Co. Ltd.,
Tokyo, Japan
Jean-Jaques Hauw
Anatomopathologie (Neurologique) Hôpital Pitiè-Salpêtrière
Paris, France
Mark W. Head
National CJD Surveillance Unit
Western General Hospital
Edinburgh, UK
John C. Hedreen
Harvard Brain Tissue Resource Center
Department of Psychiatry
Harvard Medical School and McLean Hospital
Belmont, MA, USA
Janice L. Holton
Department of Molecular Neuroscience
UCL Institute of Neurology
University College London
London, UK
Tibor Hortobágy
Department of Clinical Neuroscience
NIHR Biomedical Research Centre for Mental Health and MRC Centre for Neurodegeneration Research
Institute of Psychiatry
King’s College London
London, UK
Paul G. Ince
Sheffield Institute for Translational Neuroscience
Department of Neuroscience
University of Sheffield
Sheffield, UK
James W. Ironside
National CJD Surveillance Unit
Western General Hospital
Edinburgh, UK
Kurt A. Jellinger
Institute of Clinical Neurobiology
Medical University Vienna
Vienna, Austria
Veronika Kana
University Hospital Zürich
Institute of Neuropathology
Zürich, Switzerland
Shinsuke Kato
Department of Neuropathology
Institute of Neurological Sciences
Tottori University
Yonago, Japan
Masahisa Katsuno
Department of Neurology
Nagoya University Graduate School of Medicine;
Institute for Advanced Research
Nagoya University,
Nagoya, Japan
Richard S.G. Knight
National CJD Surveillance Unit
Western General Hospital
Edinburgh, UK
David Knopman
Department of Neurology
Mayo Clinic
Rochester, MN, USA
Arnulf H. Koeppen
VA Medical Center and Departments of Neurology and Pathology
Albany Medical College
Albany, NY, USA
Quingzhong Kong
National Prion Disease Pathology Surveillance Center
Case Western Reserve University
Cleveland, OH, USA
Gabor G. Kovacs
Institute of Neurology Medical University Vienna
Vienna, Austria
Tammaryn Lashley
Department of Molecular Neuroscience
UCL Institute of Neurology
University College London
London,UK
Robert Layfield
School of Biomedical Sciences
University of Nottingham Medical School
Queen’s Medical Centre
Nottingham, UK
Andrew J. Lees
Department of Molecular Neuroscience
UCL Institute of Neurology
University College London
London, UK
Jada Lewis
Center for Translational Research of Neurodegenerative Disease and Department of Neuroscience University of Florida
Gainesville, FL, USA
Irene Litvan
Department of Neurology
University of Louisville School of Medicine
Louisville, KY, USA
Seth Love
Department of Neuropathology
Institute of Clinical Neurosciences
University of Bristol
Bristol, UK
James Lowe
School of Molecular Medical Sciences
University of Nottingham Medical School
Queen’s Medical Centre
Nottingham, UK
Ian R.A. Mackenzie
Department of Pathology and Laboratory Medicine
University of British Columbia
Vancouver, BC, Canada
Colin L. Masters
Mental Health Research Institute
National Neuroscience Facility
The University of Melbourne
Melbourne, Australia
R. John Mayer
School of Biomedical Sciences
University of Nottingham Medical School
Queen’s Medical Centre
Nottingham, UK
Catriona Ann McLean
Department of Anatomical Pathology
The Alfred Hospital
Prahran, Australia
Heather Melrose
Department of Neuroscience
Mayo Clinic
Jacksonville, FL, USA
Hidehiro Mizusawa
Department of Neurology
Tokyo Medical and Dental University
Tokyo, Japan
Siddhartha Mondragón-Rodríguez
Départment de Physiologie
Université de Montréal
Montreal, QC, Canada
Paula I. Moreira
Center for Neuroscience and Cell Biology
University of Coimbra
Coimbra, Portugal
Huw R. Morris
MRC Centre for Neuropsychiatric Genetics and Genomics
Cardiff University School of Medicine
Cardiff, UK
David G. Munoz
Department of Laboratory Medicine and Pathobiology
St. Michael’s Hospital and Keenan Research Centre at the Li Ka Shing Knowledge Institute of St. Michael’s Hospital
University of Toronto
Toronto, ON, Canada
Manuela Neumann
Institute of Neuropathology
University Hospital of Zürich
Zürich, Switzerland
James A.R. Nicoll
Clinical Neurosciences
University of Southampton School of Medicine
Southampton, UK
Akihiko Nunomura
Department of Neuropsychiatry
Interdisciplinary Graduate School of Medicine and Engineering
University of Yamanashi
Yamanashi, Japan
Koichi Okamoto
Department of Neurology
Gunma University Graduate School of Medicine
Gunma, Japan
Kiyomitsu Oyanagi
Department of Neuropathology
Tokyo Metropolitan Institute for Neuroscience
Tokyo, Japan;
Division of Neuropathology
Department of Brain Disease Research
Shinshu University School of Medicine
Nagano, Japan
Simon Paine
School of Biomedical Sciences
University of Nottingham Medical School
Queen’s Medical Centre
Nottingham, UK
Coro Paisán-Ruiz
Departments of Neurology,
Psychiatry and Genetics and Genomic Sciences
Mount Sinai School of Medicine
New York, NY, USA
Piero Parchi
Department of Neurological Sciences
University of Bologna
Bologna, Italy
Laura Parkkinen
Department of Molecular Neuroscience
UCL Institute of Neurology
University College London
London, UK
George Perry
Neurosciences Institute and Department of Biology
University of Texas at San Antonio
San Antonio, TX, USA
Pedro Piccardo
Center for Biologic Evaluation and Research
Food and Drug Administration
Rockville, MD, USA
Violetta N. Pivtoraiko
Department of Pathology
University of Alabama at Birmingham
Birmingham, AL, USA
Gordon Plant
The National Hospital for Neurology and Neurosurgery
London, UK
Gianfranco Puoti
National Prion Disease Pathology Surveillance Center
Case Western Reserve University
Cleveland, OH, USA
Olivier Rascol
Departments of Neurosciences and Clinical Pharmacology
University Hospital of Toulouse
Toulouse, France
Tamas Revesz
Department of Molecular Neuroscience
UCL Institute of Neurology
University College London
London, UK
Nooshin Rezvani
School of Biomedical Sciences
University of Nottingham Medical School
Queen’s Medical Centre
Nottingham, UK
Raymund A.C. Roos
Department of Neurology
Leiden University Medical Center
Leiden, The Netherlands
Martin Rossor
Dementia Research Centre
Department of Neurodegeneration
UCL Institute of Neurology
University College London
London, UK
Agueda Rostagno
Department of Pathology
New York University School of Medicine
New York, NY, USA
Kevin A. Roth
Department of Pathology
University of Alabama at Birmingham
Birmingham, AL, USA
Naruhiko Sahara
Center for Translational Research of Neurodegenerative Disease and Department of Neuroscience University of Florida
Gainesville, FL, USA
Mark A. Smith
Deceased
Department of Pathology
Case Western Reserve University
Cleveland, OH, USA
Gen Sobue
Department of Neurology
Nagoya University Graduate School of Medicine
Nagoya, Japan
Salvatore Spina
Department of Pathology and Laboratory Medicine
Indiana University School of Medicine
Indianapolis, IN, USA
Michael J. Strong
Department of Clinical Neurological Sciences
The University of Western Ontario and the Robarts Research Institute
London, ON, Canada
Fabrizio Tagliavini
Division of Neuropathology
Carlo Besta Neurological Institute
Milan, Italy
Hitoshi Takahashi
Department of Pathology
Brain Research Institute
Niigata University
Niigata, Japan
Rudolph E. Tanzi
Genetics and Aging Research Unit
Massachusetts General Hospital
Charlestown, MA, USA
Markus Tolnay
Institute of Pathology
Basel University Hospital
Basel, Switzerland
Shoji Tsuji
Department of Neurology
Graduate School of Medicine
University of Tokyo
Tokyo, Japan
Ruben Vidal
Department of Pathology and Laboratory Medicine
Indiana University School of Medicine
Indianapolis, IN, USA
Roy O. Weller
Clinical Neurosciences
University of Southampton School of Medicine
Southampton, UK
Robert G. Will
National CJD Surveillance Unit
Western General Hospital
Edinburgh, UK
Zbigniew K. Wszolek
Department of Neurology
Mayo Clinic
Jacksonville, FL, USA
Mitsunori Yamada
Department of Clinical Research
Saigata National Hospital
Niigata, Japan
Mineo Yamazaki
Department of Neuropathology
Tokyo Metropolitan Institute for Neuroscience;
Department of Neurology
Nippon Medical School
Tokyo, Japan
Xiongwei Zhu
Department of Pathology
Case Western Reserve University
Cleveland, OH, USA
Wenquan Zou
National Prion Disease Pathology Surveillance Center
Case Western Reserve University
Cleveland, OH, USA
Preface
One of the major roles of the International Society of Neuropathology (ISN) is to promote world-wide education and research in the pathology of neurological disease for the enhancement of care and treatment of patients. As the interface between neuropathology and other scientific and clinical disciplines became increasingly blurred, a series of books on the “Pathology and Genetics” of neurological disease was initiated by Dr Paul Kleihues to promote mutual understanding and collaboration.
The first book in the series was on Brain Tumours (1997); that is now in its fourth edition as the WHO Classification of Tumours of the Central Nervous System, edited by David N. Louis, Hiroko Ohgaki, Otmar D. Wiestler and Webster K. Cavenee and published by WHO Press (2007). A further four books were published by ISN between 2001 and 2005 with Yngve Olsson as Series Editor: Structural and Molecular Basis of Skeletal Muscle Diseases, Volume Editor George Karpati (2002); Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders, Volume Editor Dennis Dickson (2003); Developmental Neuropathology , Volume Editors Jeffrey A. Golden and Brian Harding (2004); Pathology and Genetics of Cerebrovascular Diseases, Volume Editor Hannu Kalimo (2005). Each volume brought together expertise from multiple international authors writing in a standard format that allowed readers to navigate the different chapters with ease. These features led to the considerable success of the series.
Publication of the “Pathology and Genetics” series has entered a new phase with a new publisher and a new Series Editor. As publisher of the very successful ISN Journal Brain Pathology, Wiley-Blackwell has been appointed to publish the ISN book series. Following wide international consultation, a second edition of Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders was selected as a priority for publication with Dennis Dickson and Roy Weller as Volume Editors. This reflects the considerable and momentous advances that have occurred in the field of neurodegeneration since the publication of the first edition. Many of the authors from the first edition have been retained and every chapter has been rewritten and thoroughly updated. The format that is familiar to readers of the first edition has also been retained but new sections have been added to reflect advances in the field. In its multidisciplinary approach, the volume concentrates on pathology, genetics, molecular biology and biochemistry in relation to clinical aspects of neurodegenerative disease, imaging and the new therapies that are on the horizon. There is some overlap between volumes in the series, such that vascular dementia is covered in the previous book on cerebrovascular diseases.
I would like to thank Dennis Dickson as Volume Editor for establishing the structure of the current book on neurodegeneration and for inspiring the authors. Our thanks go to the 96 authors who put so much effort and expertise into ensuring the high quality of the text and illustrations. Sadly, one author, Mark Smith, died in a road accident at the end of 2010 but will be remembered through his contribution to this book.
Finally, who will benefit from reading this book on neurodegeneration? Our aim was to provide succinct and well-ordered chapters for instant access to the concepts and many of the details of the pathology and genetics of neurodegenerative disease to further the multidisciplinary interests of pathologists, clinicians and neuroscientists involved in research and in the diagnosis and care of patients with neurodegenerative disease. We trust that our aspirations will be fulfilled.
Roy O. Weller
Series Editor