Table of Contents

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

About the Author

Professor Gillian Bates obtained her PhD from the University of London in 1987. Her postdoctoral work was conducted with Hans Lehrach at the ICRF on the cloning of the Huntington's disease gene. Her work has at the forefront of Huntington's disease research since establishing her independent research programme in 1994.

Professor Lesley Jones obtained her PhD from Cardiff University in 1990 and moved to the then University of Wales College of Medicine in 1994 to work on the biochemistry of huntingtin in HD. Her work has primarily been concerned with the notable downstream effects of mutant huntingtin on gene expression. She is also leading work on genetic modifiers of HD to assist in the identification of molecular pathways important in disease pathology.

Sarah Tabrizi is Professor of Clinical Neurology in the Department of Neurodegenerative Diseases at the UCL Institute of Neurology and Honorary Consultant Neurologist at the National Hospital of Neurology and Neurosurgery. Her research focuses on the pathobiological, translational and clinical aspects of neurodegeneration with a major focus on Huntington's disease. Sarah leads two major, international multidisciplinary research initiatives, TRACK-HD and Track-On HD, aimed at understanding the
neurobiology of the neurodegenerative changes in premanifest and early stage HD gene carriers and identifying sensitive measures of disease progression.