About the EditorsProfessor 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, viPreface, ixDedication, x1 Pathology of Brain Aging, 1Janna H. Neltner and Peter T. Nelson2 Protein Aggregation and Neurodegeneration: Tauopathies and Synucleinopathies, 12Michel Goedert3 Prion Degeneration, 25James A. Mastrianni4 Excitotoxicity, 37Mark P. Mattson5 Etiology and Pathogenesis of Parkinson Disease, 46Ruth-Mary deSouza and Anthony H.V. Schapira6 Parkinson Disease: Treatment Options – Surgical Therapy, 53Travis S. Tierney and Andres M. Lozano7 Multiple System Atrophy: Clinical, Genetics, and Neuropathology, 58Lucia V. Schottlaender, Anna Sailer, Zeshan Ahmed, Dennis W. Dickson, Henry Houlden, and Owen A. Ross8 Progressive Supranuclear Palsy, 72Huw R. Morris9 Dementia with Lewy Bodies, 83Rodolfo Savica and David S. Knopman10 Corticobasal Degeneration, 93Keith A. Josephs, Jr.11 Alzheimer’s Disease, 102Qurat ul Ain Khan and Neill R. Graff-Radford12 Frontotemporal Dementia, 115Christian W. Wider, Barbara Jasinska-Myga, Takuya Konno, and Zbigniew K. Wszolek13 Prion Diseases and Other Rapidly Progressive Dementias, 126Eric Eggenberger and Daniel L. Murman14 Amyotrophic Lateral Sclerosis and Primary Lateral Sclerosis, 137Kevin B. Boylan, Mark A. Ross, and Eric J. Sorenson15 Hereditary Spastic Paraplegia, 161Pawel P. Liberski and Craig Blackstone16 Spinal Muscular Atrophy, 179Joseph R. Wooley, Melissa E. Crowder, Noah J. Pyles, and Charlotte J. Sumner17 Spinal and Bulbar Muscular Atrophy (Kennedy Disease), 202Erich Peter Bosch18 Optic Neuropathies, 211Desmond Kidd19 Peripheral Nerve Neuropathies Including Charcot–Marie–Tooth Disease, 218Amelie Pandraud and Henry Houlden20 Axonal Loss and Neurodegeneration in Multiple Sclerosis, 238Ranjan Dutta, Jacqueline Chen, Nobuhiko Ohno, Daniel Ontaneda, and Bruce D. Trapp21 Huntington’s Disease and Other Choreas, 248Salman Haider, Edward Wild, and Sarah J. Tabrizi22 Spinocerebellar Ataxias, 284H. Brent Clark and Harry T. Orr23 Ataxia Telangiectasia, 296Malcolm Taylor24 Niemann–Pick Disease, 303Marc C. Patterson25 X-linked Adrenoleukodystrophy, 309Deborah L. Renaud26 Neurodegeneration with Brain Iron Accumulation, Wilson Disease, and Manganism, 317Alisdair McNeillIndex, 327