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Parkinson's disease is one of the most common neurodegenerative diseases with a higher prevalence in older adults. It is a slowly progressive condition and has set the standard for research in neurodegeneration throughout the history of medicine. It was the first neurodegenerative disease for which the pathology was discovered, when I. Tretjakov first described the degenerated substantia nigra in 1919. The biochemistry was first described by A. Carlsson, and the transmitter deficit by O. Hornykiewicz. The accidental discovery of the selective neurotoxin 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) for neurones of the substantia nigra in the 1980s has been of a similar importance, as it subsequently became possible to study Parkinson's disease in animal models. All these basic research achievements were the basis for groundbreaking progress in the field of therapy. Parkinson's disease was the first neurodegenerative disease for which transmitter replacement therapy was developed; it was the first condition in medical history for which cell-replacement therapy or neurotransplantation was attempted; and it was one of the first conditions for which therapy using electrical brain stimulation was shown to be beneficial.
As we benefit from research advances, the beginner, the experienced neurologist, and the movement disorder specialist need continuous updates. New possibilities for treatment offer new choices for patients and expand the skill set and knowledge base of physicians. This new edition of the Handbook of Parkinson's Disease covers all new aspects of the condition and fills the need for an authoritative and recent update. The editors have put together an excellent group of authors, each of whom is a well-known specialist in their field, and the chapters are scholarly and easy to read.
William Koller, MD, PhD, who was among the editors of the previous editions of this handbook, was one of the founders of the research field of movement disorders in the United States. He was specifically dedicated to excellent clinical research and patient care. Kelly Lyons and Rajesh Pahwa, co-workers of Bill Koller for many years, have edited this handbook in his spirit and have succeeded in maintaining the quality of the previous editions with this fourth edition. The book will serve as an ideal reference for all those who care for patients as well as those who want to enter the field.
Günther Deuschl, MD Professor of Neurology Chairman of the Department of Neurology Christian-Albrechts-University Kiel Universiätsklinikum Schleswig-Holstein
Parkinson's disease is a progressive neurodegenerative condition with often devastating symptoms. Our knowledge of Parkinson's disease has increased tremendously in recent years. We have achieved a greater understanding of the neurochemistry, neurophysiology, and neuropathology of Parkinson's disease. Genes have been identified that are involved in the pathogenesis of some forms of familial autosomal dominant and recessive Parkinson's disease. Advancements in neuropsychological and neuroimaging techniques have led to improvements in diagnostic accuracy, and therapeutic options have been expanded. In addition, new medications have been approved, new compounds and therapeutic approaches are under investigation, and new surgical procedures and therapies are being explored. In spite of these advances, there continue to be many complications associated with the long-term management of both motor and non-motor symptoms of Parkinson's disease, and treatment remains a challenge.
We present in this edition of the Handbook of Parkinson's Disease the most up-to-date information on the scientific and therapeutic aspects of Parkinson's disease. This fourth edition offers a more integrated approach to managing parkinsonian symptoms and has been expanded to include more in-depth coverage of neuropsychiatric aspects of Parkinson's disease, sleep issues, and non-pharmacological and nontradi-tional therapies. There is comprehensive coverage of the latest pharmacologic and surgical therapeutics as well as the newest basic research. It is our hope that this volume, in the tradition of the first three editions, will serve as a reference source for physicians, researchers, and other health care professionals seeking answers to the many questions related to understanding and treating Parkinson's disease.
We would like to thank each of the authors for their time and commitment in preparing state-of-the-art reviews of the most pertinent aspects of Parkinson's disease.
Rajesh Pahwa Kelly E. Lyons
Foreword Günther Deuschl . . . . iii Preface . . . . v Contributors .. . . ix
1. Early Iconography of Parkinson's Disease 1
Christopher G. Goetz
2. Epidemiology 19
Michele Rajput, Alex Rajput, and Ali H. Rajput
3. Differential Diagnosis 29
John Morgan and Kapil D. Sethi
4. Pathophysiology and Clinical Assessment 49
5. Autonomic Dysfunction and Management 77
Richard B. Dewey, Jr.
6. Sleep Dysfunction 91
Laura Nieder and K. Ray Chaudhuri
7. Neuropsychological Aspects 109
Alexander I. Tröster and Steven Paul Woods
8. Management of Anxiety and Depression 133
Jack J. Chen
Kelvin L. Chou and Hubert H. Fernandez
10. Neuroimaging 177
Kenneth Marek, Danna Jennings, and John Seibyl
11. Neuropathology 195
Dennis W. Dickson
12. Neurochemistry of Nigral Degeneration 209
13. Neurophysiology and Neurocircuitry 223
Erwin B. Montgomery and John T. Gale
14. Animal Models 239
Giselle M. Petzinger and Michael W. Jakowec vii
15. Genetics 269
Akiko Imamura, Matthew J. Farrer, and Zbigniew K. Wszolek
16. Environmental Risk Factors 279
Brad A. Racette
17. Amantadine and Anticholinergics 293
Khashayar Dashtipour, Joseph S. Chung, Allan D. Wu, and Mark F. Lew
18. Levodopa 309
Stewart A. Factor
19. Dopamine Agonists 335
Valerie Street and Mark Stacy
20. Monoamine Oxidase Inhibitors 349
Alex Rajput, Theresa A. Zesiewicz, and Robert A. Hauser
21. Catechol-O-Methyltransferase Inhibitors 365
Ronald F. Pfeiffer
22. Investigational Pharmacological Treatments 379
William G. Ondo
23. Lesion Surgeries 391
Michael Samuel, Keyoumars Ashkan, and Anthony E. Lang
24. Deep Brain Stimulation 409
Kelly E. Lyons and Rajesh Pahwa
25. Investigational Surgical Therapies 423
Joseph S. Neimat, Parag G. Patil, and Andres M. Lozano
26. Physical and Occupational Therapy 441
Atul T. Patel and Sean Shire
27. Voice, Speech, and Swallowing Disorders 451
Shimon Sapir, Lorraine Olson Ramig, and Cynthia Fox
28. Alternative Therapies 475
Keyoumars Ashkan Department of Neurosurgery, National Hospital for Neurology and Neurosurgery, Queen Square, London, U.K.
K. Ray Chaudhuri Movement Disorders Unit, Kings College Hospital, University Hospital Lewisham and Guy's King's and St. Thomas' School of Medicine, London, U.K.
Jack J. Chen Department of Neurology, Schools of Medicine and Pharmacy, Movement Disorders Center, Loma Linda University, Loma Linda, California, U.S.A.
Kelvin L. Chou Department of Clinical Neurosciences, Brown Medical School and NeuroHealth Parkinson's Disease and Movement Disorders Center, Warwick, Rhode Island, U.S.A.
Joseph S. Chung Department of Neurology, Movement Disorders Specialist, Southern California Kaiser Permanente, Los Angeles, California, U.S.A.
Khashayar Dashtipour Division of Movement Disorders, Department of Neurology, Loma Linda University School of Medicine, Loma Linda, California, U.S.A.
Richard B. Dewey, Jr. Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas, U.S.A.
Dennis W. Dickson Department of Pathology, Mayo Clinic College of Medicine, Jacksonville, Florida, U.S.A.
Stewart A. Factor Emory University School of Medicine, Department of Neurology, Wesley Woods Health Center, Atlanta, Georgia, U.S.A.
Matthew J. Farrer Mayo Clinic, Jacksonville, Florida, and Mayo School of Graduate Medical Education, Mayo Clinic College of Medicine, Rochester, Minnesota, U.S.A.
Hubert H. Fernandez Movement Disorders Center and Department of Neurology, University of Florida/McKnight Brain Institute, Gainesville, Florida, U.S.A.
Cynthia Fox Tucson, Arizona, National Center for Voice and Speech, Denver, Colorado, U.S.A.
John T. Gale Department of Neurosurgery, Massachusetts General Hospital/Harvard Medical School, Boston, Massachusetts, U.S.A.
Christopher G. Goetz Department of Neurological Sciences, Rush University, Chicago, Illinois, U.S.A.
Robert A. Hauser Parkinson's Disease and Movement Disorders Center, University of South Florida, NPF Center of Excellence, Tampa, Florida, U.S.A.
Akiko Imamura Mayo Clinic, Jacksonville, Florida, and Mayo School of Graduate Medical Education, Mayo Clinic College of Medicine, Rochester, Minnesota, U.S.A.
Michael W. Jakowec Department of Neurology, University of Southern California, Los Angeles, California, U.S.A.
Joseph Jankovic Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, Texas, U.S.A.
Danna Jennings Department of Neurology, The Institute for Neurodegenerative Disorders, New Haven, Connecticut, U.S.A.
Anthony E. Lang Department of Medicine, Division of Neurology, The Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada
Mark F. Lew Division of Movement Disorders, Keck/USC School of Medicine, Los Angeles, California, U.S.A.
Andres M. Lozano Division of Neurosurgery, Toronto Western Hospital, Toronto, Ontario, Canada
Kelly E. Lyons Department of Neurology, University of Kansas Medical Center, Kansas City, Kansas, U.S.A.
Kenneth Marek Department of Neurology, The Institute for Neurodegenerative Disorders, New Haven, Connecticut, U.S.A.
Jill Marjama-Lyons Albuquerque, New Mexico, U.S.A.
Erwin B. Montgomery Department of Neurology, National Primate Research Center, University of Wisconsin-Madison, Madison, Wisconsin, U.S.A.
John Morgan Department of Neurology, Medical College of Georgia, Augusta, Georgia, U.S.A.
Joseph S. Neimat Department of Neurological Surgery, Vanderbilt University Medical Center, Nashville, Tennessee, U.S.A.
Laura Nieder Neurology Department, Guy's King's and St. Thomas' School of Medicine, London, U.K.
William G. Ondo Department of Neurology, Baylor College of Medicine, Houston, Texas, U.S.A.
Atul T. Patel Department of Rehabilitation, Research Medical Center, Kansas City Bone and Joint Clinic, Overland Park, Kansas, U.S.A.
Parag G. Patil Department of Neurosurgery, University of Michigan, Ann Arbor, Michigan, U.S.A.
Rajesh Pahwa Department of Neurology, University of Kansas Medical Center, Kansas City, Kansas, U.S.A.
Giselle M. Petzinger Department of Neurology, University of Southern California, Los Angeles, California, U.S.A.
Ronald F. Pfeiffer Department of Neurology, University of Tennessee Health Science Center, Memphis, Tennessee, U.S.A.
Brad A. Racette Department of Neurology and American Parkinson Disease Association Advanced Center for Parkinson Research, Washington University School of Medicine, St. Louis, Missouri, U.S.A.
Alex Rajput Division of Neurology, Department of Medicine, University of Saskatchewan, Saskatoon, Saskatchewan, Canada
Ali H. Rajput Division of Neurology, Department of Medicine, University of Saskatchewan, Saskatoon, Saskatchewan, Canada
Michele Rajput Division of Neurology, Department of Medicine, University of Saskatchewan, Saskatoon, Saskatchewan, Canada
Lorraine Olson Ramig Department of Speech, Language, Hearing Sciences, University of Colorado, Boulder, and National Center for Voice and Speech, Denver, Colorado, U.S.A.
Jayaraman Rao Department of Neurology, Parkinson's Disease and Movement Disorders Center, Ochsner Foundation Clinic, New Orleans, Louisiana, U.S.A.
Michael Samuel Department of Neurology, King's College Hospital, Denmark Hill, London, U.K.
Shimon Sapir Department of Communication Sciences and Disorders, University of Haifa, Haifa, Israel
John Seibyl Department of Neurology, The Institute for Neurodegenerative Disorders, New Haven, Connecticut, U.S.A.
Kapil D. Sethi Department of Neurology, Medical College of Georgia, Augusta, Georgia, U.S.A.
Sean Shire Department of Rehabilitation, Research Medical Center, RMC-Brookeside, Kansas City, Missouri, U.S.A.
Mark Stacy Division of Neurology, Duke University Medical School, Durham, North Carolina, U.S.A.
Valerie Street Division of Neurology, Duke University Medical School, Durham, North Carolina, U.S.A.
Alexander I. Tröster Department of Neurology, University of North Carolina School of Medicine at Chapel Hill, Chapel Hill, North Carolina, U.S.A.
Steven Paul Woods Department of Psychiatry, University of California at San Diego, San Diego, California, U.S.A.
Zbigniew K. Wszolek Mayo Clinic, Jacksonville, Florida, and Mayo School of Graduate Medical Education, Mayo Clinic College of Medicine, Rochester, Minnesota, U.S.A.
Allan D. Wu Division of Movement Disorders, Department of Neurology, David Geffen School of Medicine, University of California-Los Angeles, Los Angeles, California, U.S.A.
Theresa A. Zesiewicz Parkinson's Disease and Movement Disorders Center, University of South Florida, NPF Center of Excellence, Tampa, Florida, U.S.A.
Early Iconography of Parkinson's Disease
Christopher G. Goetz
Department of Neurological Sciences, Rush University, Chicago, Illinois, U.S.A.
Parkinson's disease was first described in a medical context in 1817 by James Parkinson, a general practitioner in London. Numerous essays have been written about Parkinson himself and the early history of Parkinson's disease (Paralysis agitans), or the shaking palsy. Rather than repeat or resynthesize such prior studies, this introductory chapter focuses on a number of historical visual documents with descriptive legends. Some of these are available in prior publications, but the entire collection has not been presented before. As a group, they present materials from the 19th century and will serve as a base, on which the subsequent chapters that cover progress of the twentieth and budding twenty-first centuries are built. In 2005, as part of the Movement Disorder Society annual international congress, an extensive history exhibit was developed. Interested readers can access the core of this exhibit (1).
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