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.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 ; Cecile 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 Veronique M. Andre ; 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 Bjorkqvist ; 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