Given the aging population of the developed world, and the increasing survival rates from traditional diseases such as cancer, the numbers of patients suffering from neurodegenerative diseases is increasing. Not only do these diseases inflict pain and emotional toil on their sufferers and their families, they also require years of costly health care
Many of the neurodegenerative diseases have been considered within more traditional domains of neurology, such as movement disorders or behavioral neurology. However, there is increasing recognition that this symptom–based classification does not correspond to underlying pathologies for which evidence of similarity is emerging.
This book draws together clinical and research information on neurodegenerative diseases, beginning with the common elements before treating each disease individually using a standard template. Uniquely diseases will be grouped by anatomical regions of degeneration – which are largely but not exclusively related to symptomatic considerations.
General chapter topics cover what can be considered normal aging versus neurodegenerative diseases, and discuss the initial indications of neurodegeneration progression of symptoms. Diagnostic guidelines include how to apply cognitive examinations, movement examinations, and lab and imaging investigations to reach a diagnosis – an often very complex process in neurodegenerative disease. Also discussed are patient health care issues, and the effect on carers and families, and palliative and end–of–life care.
- Presents a clinically oriented guide to the diseases caused by neurodegeneration
- Includes templated chapters with helpful pedagogy
- Groups diseases by anatomical regions of degeneration, including common disorders such as Parkinson′s disease, Alzheimer′s disease, amyotrophic lateral sclerosis/motor neuron disease, and multiple sclerosis as well as less common diseases
- Offers expert advice from globally leading authorities on the subject
This book will be of use to neurologists, and will be of interest to neuropsychiatrists who deal with psychiatric aspects of neurological disease. It will also be relevant to neuroscientists who need to know more of the clinical aspects of the conditions they are working on.
List of Contributors, vi
1 Pathology of Brain Aging, 1Janna H. Neltner and Peter T. Nelson
2 Protein Aggregation and Neurodegeneration: Tauopathies and Synucleinopathies, 12Michel Goedert
3 Prion Degeneration, 25James A. Mastrianni
4 Excitotoxicity, 37Mark P. Mattson
5 Etiology and Pathogenesis of Parkinson Disease, 46Ruth–Mary deSouza and Anthony H.V. Schapira
6 Parkinson Disease: Treatment Options – Surgical Therapy, 53Travis S. Tierney and Andres M. Lozano
7 Multiple System Atrophy: Clinical, Genetics, and Neuropathology, 58Lucia V. Schottlaender, Anna Sailer, Zeshan Ahmed, Dennis W. Dickson, Henry Houlden, and Owen A. Ross
8 Progressive Supranuclear Palsy, 72Huw R. Morris
9 Dementia with Lewy Bodies, 83Rodolfo Savica and David S. Knopman
10 Corticobasal Degeneration, 93Keith A. Josephs, Jr.
11 Alzheimer’s Disease, 102Qurat ul Ain Khan and Neill R. Graff–Radford
12 Frontotemporal Dementia, 115Christian W. Wider, Barbara Jasinska–Myga, Takuya Konno, and Zbigniew K. Wszolek
13 Prion Diseases and Other Rapidly Progressive Dementias, 126Eric Eggenberger and Daniel L. Murman
14 Amyotrophic Lateral Sclerosis and Primary Lateral Sclerosis, 137Kevin B. Boylan, Mark A. Ross, and Eric J. Sorenson
15 Hereditary Spastic Paraplegia, 161Pawel P. Liberski and Craig Blackstone
16 Spinal Muscular Atrophy, 179Joseph R. Wooley, Melissa E. Crowder, Noah J. Pyles, and Charlotte J. Sumner
17 Spinal and Bulbar Muscular Atrophy (Kennedy Disease), 202Erich Peter Bosch
18 Optic Neuropathies, 211Desmond Kidd
19 Peripheral Nerve Neuropathies Including Charcot–Marie–Tooth Disease, 218Amelie Pandraud and Henry Houlden
20 Axonal Loss and Neurodegeneration in Multiple Sclerosis, 238Ranjan Dutta, Jacqueline Chen, Nobuhiko Ohno, Daniel Ontaneda, and Bruce D. Trapp
21 Huntington’s Disease and Other Choreas, 248Salman Haider, Edward Wild, and Sarah J. Tabrizi
22 Spinocerebellar Ataxias, 284H. Brent Clark and Harry T. Orr
23 Ataxia Telangiectasia, 296Malcolm Taylor
24 Niemann–Pick Disease, 303Marc C. Patterson
25 X–linked Adrenoleukodystrophy, 309Deborah L. Renaud
26 Neurodegeneration with Brain Iron Accumulation, Wilson Disease, and Manganism, 317Alisdair McNeill
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.