Bates Gillian - Huntingtons Disease

Huntingtons Disease

OXFORD MONOGRAPHS ON MEDICAL GENETICS

General Editors:

Judith G. Hall

Peter S. Harper

Louanne Hudgkins

Evan Eichler

Charles J. Epstein (deceased 2011)

Arno G. Motulsky (resigned 2011)

1. R.B. McConnell: The genetics of gastrointestinal disorders

2. A.C. Kopc: The distribution of the blood groups in the United Kingdom

3. E. Slater and V.A. Cowie: The genetics of mental disorders

4. C.O. Carter and T.J. Fairbank: The genetics of locomotor disorders

5. A.E. Mourant, A.C. Kopc, and K. Domaniewska-Sobezak: The distribution of the human blood groups and other polymorphisms

6. A.E. Mourant, A.C. Kopc, and K. Domaniewska-Sobezak: Blood groups and diseases

7. A.G. Steinbert and C.E. Cook: The distribution of the human immunoglobulin allotypes

8. D. Tills, A.C. Kopc, and R.E. Tills: The distribution of the human blood groups and other polymorphisms: supplement I

10. D.Z. Loesch: Quantitative dermatoglyphics: classification, genetics, and pathology

11. D.J. Bond and A.C. Chandley: Aneuploidy

12. P.F. Benson and A.H. Fensom: Genetic biochemical disorders

13. G.R. Sutherland and F. Hecht: Fragile sites on human chromosomes

14. M. dA Crawfurd: The genetics of renal tract disorders

16. C.R. Scriver and B. Child: Garrods inborn factors in disease

18. M. Baraitser: The genetics of neurological disorders

19. R.J. Gorlin, M.M. Cohen, Jr., and L.S. Levin: Syndromes of the head and neck, third edition

21. D. Warburton, J. Byrne, and N. Canki: Chromosome anomalies and prenatal development: an atlas

22. J.J. Nora, K. Berg, and A.H. Nora: Cardiovascular disease: genetics, epidemiology, and prevention

24. A.E.H. Emery: Duchenne muscular dystrophy, second edition

25. E.G.D. Tuddenham and D.N. Cooper: The molecular genetics of haemostasis and its inherited disorders

26. A. Bou: Foetal medicine

27. R.E. Stevenson, J.G. Hall, and R.M. Goodman: Human malformations

28. R.J. Gorlin, H.V. Toriello, and M.M. Cohen, Jr.: Hereditary hearing loss and its syndromes

29. R.J.M. Gardner and G.R. Sutherland: Chromosomes abnormalities and genetic counseling, second edition

30. A.S. Teebi and T.I. Farag: Genetic disorders among Arab populations

31. M.M. Cohen, Jr.: The child with multiple birth defects

32. W.W. Weber: Pharmacogenetics

33. V.P. Sybert: Genetic skin disorders

34. M. Baraitser: Genetics of neurological disorders, third edition

35. H. Ostrer: Non-Mendelian genetics in humans

36. E. Traboulsi: Genetic factors in human disease

37. G.L. Semenza: Transcription factors and human disease

38. L. Pinsky, R.P. Erickson, and R.N. Schimke: Genetic disorders of human sexual development

39. R.E. Stevenson, C.E. Schwartz, and R.J. Schroer: X-linked mental retardation

40. M.J. Khoury, W. Burke, and E. Thomson: Genetics and public health in the 21st century

41. J. Weil: Psychosocial genetic counseling

42. R.J. Gorlin, M.M. Cohen, Jr., and R.C.M. Hennekam: Syndromes of the head and neck, fourth edition

43. M.M. Cohen, Jr., G. Neri, and R. Weksberg: Overgrowth syndromes

44. R.A. King, J.I. Rotter, and A.G. Motulsky: The genetic basis of common diseases, second edition

45. G.P. Bates, P.S. Harper, and L. Jones: Huntingtons disease, third edition

46. R.J.M. Gardner and G.R. Sutherland: Chromosome abnormalities and genetic counseling, third edition

47. I.J. Holt: Genetics of mitochondrial disease

48. F. Flinter, E. Maher, and A. Saggar-Malik: The genetics of renal disease

49. C.J. Epstein, R.P. Erickson, and A. Wynshaw-Boris: Inborn errors of development: the molecular basis of clinical disorders of morphogenesis

50. H.V. Toriello, W. Reardon, and R.J. Gorlin: Hereditary hearing loss and its syndromes, second edition

51. P.S. Harper: Landmarks in medical genetics

52. R.E. Stevenson and J.G. Hall: Human malformations and related anomalies, second edition

53. D. Kumar and S.D. Weatherall: Genomics and clinical medicine

54. C.J. Epstein, R.P. Erickson, and A. Wynshaw-Boris: Inborn errors of development: the molecular basis of clinical disorders of morphogenesis, second edition

55. W. Weber: Pharmacogenetics, second edition

56. P.L. Beales, I.S. Farooqi, and S. ORahilly: The genetics of obesity syndromes

57. P.S. Harper: A short history of medical genetics

58. R.C.M. Hennekam, I.D. Krantz, and J.E. Allanson: Gorlins syndromes of the head and neck, fifth edition

59. D. Kumar and P. Elliot: Principles and practices of cardiovascular genetics

60. V.P. Sybert: Genetic skin disorders, second edition

61. R.J.M. Gardner, G.R. Sutherland, and L.C. Shaffer: Chromosome abnormalities and genetic counseling, fourth edition

62. D. Kumar: Genomics and health in the developing world

63. P.S. Harper: A short history of medical genetics, second edition (online)

64. G. Bates, S. Tabrizi, and L. Jones: Huntingtons Disease, fourth edition

4TH EDITION

EDITED BY

GILLIAN P. BATES,

SARAH J. TABRIZI

AND LESLEY JONES

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Library of Congress Cataloging-in-Publication Data
Huntingtons disease (2014)
Huntingtons disease/edited by Gillian P. Bates, Sarah J. Tabrizi, Lesley Jones.4th edition.
p. ; cm.

Preceded by Huntingtons disease/[edited by] Gillian P. Bates, Peter S. Harper, Lesley Jones. 3rd ed. 2002.
ISBN 9780199929146 (alk. paper)

I. Bates, Gillian, editor of compilation. II. Tabrizi, Sarah, editor of compilation.
III. Jones, Lesley, editor of compilation. IV. Title.
[DNLM: 1. Huntington Disease. WL 359.5]
RC394.H85
616.851dc23
2013035308

1 3 5 7 9 8 6 4 2
Printed in the United States of America
on acid-free paper

Vronique M. Andr, PhD

Neurosciences TA Biology

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N. Ahmad Aziz, MD, PhD

Department of Neurology

Leiden University Medical Center