Huntington's Disease

This fourth edition of Huntington's Disease presents a comprehensive summary of the current knowledge of this disease, including the major scientific and clinical advances that have occurred since publication of the third edition in 2002. Completely updated and expanded, chapters in this volume are organized in five sections: · Clinical aspects of Huntington's disease, including updated chapters on historical perspectives, neurological, neuropsychiatric, and neuropsychological aspects, and new chapters on juvenile Huntington's and the premanifest and early stages · The genetics of Huntington's disease, including new information on its epidemiology discussions of new testing guidelines · Neurobiology, including recent insights into correlations between pathology and symptoms and a new chapter on neuronal circuitry · The molecular biology of Huntington's disease, including new chapters on the normal function of huntingtin, the molecular pathogenesis of Huntington's disease and the peripheral pathology of the disorder, and an extensively updated chapter on its structural biology · An updated description of the comprehensive care for Huntington's disease, featureing a new chapter on preclinical therapeutics and a completely rewritten chapter on the state of the art of experimental therapeutics and clinical trials. Gillian Bates, Professor of Neurogenetics, King's College of London, Edited by Sarah Tabrizi, Professor of Clinical Neurology, King's College of London, and Lesley Jones, Senior Lecturer in Neuropsychiatric Genetics, Wales College of Medicine Table of Contents Section 1: Clinical aspects of Huntington's disease 1. Huntington's Disease in a Historical Context Peter S Harper 2. Clinical Neurology of Huntington's disease Raymund AC Roos 3. Neuropsychiatry and Neuropsychology David Craufurd and Julie Snowden 4. Juvenile Huntington's disease Oliver W J Quarrell 5. Premanifest and Early Huntington's Disease Edward J Wild and Sarah J Tabrizi Section 2: The genetics of Huntington's disease 6. Huntington's disease: genetic and molecular studies Cécile Cazeneuve and Alexandra Durr 7. The Epidemiology of Huntington's Disease Chris Kay, Emily Fisher, Michael R. Hayden 8. Genetic Counselling and Testing Rhona MacLeod and Aad Tibben Section 3: Neurobiology 9. The Neuropathology of Huntington's Disease in the Human Brain Henry J Waldvogel, Eric H Kim, Lynette J Tippett, Jean-Paul G Vonsattel, and Richard LM Faull 10. Altered Neuronal Circuitry in Huntington's Disease Michael S. Levine, Elizabeth A. Wang, Jane Y. Chen, Carlos Cepeda, and Véronique M. André Section 4: The molecular biology of Huntington's disease 11. The normal function of huntingtin Chiara Zuccato and Elena Cattaneo 12. Order, disorder, and conformational flux in the structural biology of Huntington's Disease Ronald Wetzel and Rakesh Mishra 13. Pathogenic mechanisms in Huntington's disease Alis Hughes and Lesley Jones 14. Peripheral pathology in Huntington's disease Authors: Jorien M.M. van der Burg, N. Ahmad Aziz, Maria Björkqvist Section 5: Therapeutic interventions in Huntington's disease 15. Comprehensive care in Huntington's disease Martha A. Nance 16. Preclinical Experimental Therapeutics in Huntington's Disease Gillian P. Bates and Christian Landles 17. Experimental Therapeutics in Huntington's Disease: Moving Forward in Clinical Trials Beth Borowsky and Cristina Sampaio