Neurodegeneration

About the Editors Professor Anthony Schapira is Professor of Neurological Science and Head of the Department of Clinical Neuroscience at the UCL Institute of Neurology, London, UK and Chairman of Clinical Neurosciences Specialties. Zbigniew Wszolek, MD, is Professor of Neurology in the Department of Neurology, Mayo Clinic, Jacksonville, Florida, USA. Ted M.Dawson, MD, PhD, is Leonard and Madlyn Abramson Professor of Neurodegenerative Diseases and Scientific Director in the Institute for Cell Engineering, Johns Hopkins University, Baltimore, Maryland, USA. Professor Nicholas Wood is Research Director of the UCL Institute of Genetics and Galton Professor of Genetics at UCL National Hospital for Neurology and Neurosurgery, Institute of Neurology, London, UK.

List of Contributors, vi

Preface, ix

Dedication, x

1 Pathology of Brain Aging, 1
Janna H. Neltner and Peter T. Nelson

2 Protein Aggregation and Neurodegeneration: Tauopathies and Synucleinopathies, 12
Michel Goedert

3 Prion Degeneration, 25
James A. Mastrianni

4 Excitotoxicity, 37
Mark P. Mattson

5 Etiology and Pathogenesis of Parkinson Disease, 46
Ruth-Mary deSouza and Anthony H.V. Schapira

6 Parkinson Disease: Treatment Options – Surgical Therapy, 53
Travis S. Tierney and Andres M. Lozano

7 Multiple System Atrophy: Clinical, Genetics, and Neuropathology, 58
Lucia V. Schottlaender, Anna Sailer, Zeshan Ahmed, Dennis W. Dickson, Henry Houlden, and Owen A. Ross

8 Progressive Supranuclear Palsy, 72
Huw R. Morris

9 Dementia with Lewy Bodies, 83
Rodolfo Savica and David S. Knopman

10 Corticobasal Degeneration, 93
Keith A. Josephs, Jr.

11 Alzheimer’s Disease, 102
Qurat ul Ain Khan and Neill R. Graff-Radford

12 Frontotemporal Dementia, 115
Christian W. Wider, Barbara Jasinska-Myga, Takuya Konno, and Zbigniew K. Wszolek

13 Prion Diseases and Other Rapidly Progressive Dementias, 126
Eric Eggenberger and Daniel L. Murman

14 Amyotrophic Lateral Sclerosis and Primary Lateral Sclerosis, 137
Kevin B. Boylan, Mark A. Ross, and Eric J. Sorenson

15 Hereditary Spastic Paraplegia, 161
Pawel P. Liberski and Craig Blackstone

16 Spinal Muscular Atrophy, 179
Joseph R. Wooley, Melissa E. Crowder, Noah J. Pyles, and Charlotte J. Sumner

17 Spinal and Bulbar Muscular Atrophy (Kennedy Disease), 202
Erich Peter Bosch

18 Optic Neuropathies, 211
Desmond Kidd

19 Peripheral Nerve Neuropathies Including Charcot–Marie–Tooth Disease, 218
Amelie Pandraud and Henry Houlden

20 Axonal Loss and Neurodegeneration in Multiple Sclerosis, 238
Ranjan Dutta, Jacqueline Chen, Nobuhiko Ohno, Daniel Ontaneda, and Bruce D. Trapp

21 Huntington’s Disease and Other Choreas, 248
Salman Haider, Edward Wild, and Sarah J. Tabrizi

22 Spinocerebellar Ataxias, 284
H. Brent Clark and Harry T. Orr

23 Ataxia Telangiectasia, 296
Malcolm Taylor

24 Niemann–Pick Disease, 303
Marc C. Patterson

25 X-linked Adrenoleukodystrophy, 309
Deborah L. Renaud

26 Neurodegeneration with Brain Iron Accumulation, Wilson Disease, and Manganism, 317
Alisdair McNeill

Index, 327