Huntington's Disease

by Bates, Gillian; Harper, Peter; Jones, Lesley

• ISBN13: 9780198510604
• ISBN10: 0198510608
• Edition: 3rd
• Format: Hardcover
• Copyright: 2002-11-14
• Publisher: Oxford University Press

Summary

It is now almost a decade since the identification of the Huntington's Disease gene and its mutation. Major advances in our understanding of the disorder have been made during this time. Since publication of the first two editions, much more extensive evidence exists on how the HD mutation actually causes brain pathology. Experimental tools are now available to take this research further towards new therapeutic approaches. Due to these major changes, this well-established book has been radically updated. An international group of leaders in their particular fields cover the major recent advances in the genetics and neurobiology of the disease. Developments in our understanding of how the molecular basis of the disorder results in brain degeneration, with full coverage of transgenic animal models, neurochemical studies and advances in neuropathology are discussed in detail. The clinical sections cover both neurological and psychiatric aspects as well as new developments in therapy. This book will continue to provide an invaluable source of information for clinicians and scientists involved with Huntington's Disease, including geneticists, psychiatrists and neurologists and basic research workers in genetics and neurobiology.

Table of Contents

List of Contributors	xv
Section 1 Clinical aspects of Huntington's disease
Huntington's disease: a historical background	3	(25)
Peter S. Harper
Clinical neurology of Huntington's disease	28	(34)
Berry Kremer
Neuropsychological and neuropsychiatric aspects of Huntington's disease	62	(33)
David Craufurd
Julie Snowden
Imaging Huntington's disease	95	(18)
David J. Brooks
Thomasin Andrews
Section 2 The genetics of Huntington's disease
Huntington's disease: genetic and molecular studies	113	(46)
Peter S. Harper
Lesley Jones
The epidemiology of Huntington's disease	159	(39)
Peter S. Harper
Genetic counselling and presymptomatic testing	198	(53)
Aad Tibben
Section 3 Neurobiology
The neuropathology of Huntington's disease	251	(25)
Claire-Anne Gutekunst
Francine Norflus
Steven M. Hersch
Neurochemistry of Huntington's disease	276	(33)
George J. Yohrling IV
Jang-Ho J. Cha
Energy metabolism and Huntington's disease	309	(18)
C. Turner
A.H.V. Schapira
Section 4 Molecular biology of Huntington's disease
Structural biology of Huntington's disease	327	(21)
Erich E. Wanker
Anja Droge
The cell biology of Huntington's disease	348	(39)
Lesley Jones
Mouse models of Huntington's disease	387	(42)
Gillian P. Bates
Kerry P.S.J. Murphy
Section 5 Other polyglutamine diseases
The polyglutamine diseases	429	(46)
Gillian P. Bates
Caroline Benn
Section 6 Therapeutic interventions
Comprehensive care in Huntington's disease	475	(26)
Martha A. Nance
Beryl Westphal
Therapeutic trials in Huntington's disease	501	(11)
Karl Kieburtz
Ira Shoulson
Cell and tissue transplantation	512
Stephen B. Dunnett
Anne E. Rosser
Index	547