Juvenile Huntington's Disease

Huntington's disease (HD) is an inherited progressive neurodegenerative disorder. Although onset of Huntington's Disease usually occurs in adulthood, the age of onset of the condition is extremely variable with approximately 5-10% of cases having an onset of less than 20 years, or Juvenile Huntington's Disease (JHD). While JHD shares many of the clinical features of adult HD (e.g., chorea and personality disorders), patients with JHD often experience additional problems including seizures, dystonia and Parkinsonism. Diagnosis in patients with JHD is often delayed because of the failure of clinicians to recognise the characteristic features of the condition. While several textbooks have been published on HD, no books have been published to date focussing solely on the juvenile onset form of the disease. This book summarises, for the first time, the clinical and scientific knowledge available on JHD. It also collects together accounts from families affected by the condition, putting the clinical and scientific chapters into context. Edited by members of the working group on Juvenile Huntington's Disease within the European Huntington Disease Network (EHDN), this book forms the first comprehensive text on JHD and is of interest to neurologists, geneticists, academic/research scientists and other healthcare professionals.

ISBN 0199236127   Pages 224 ISBN13 9780199236121 (What's this?)   Volumes 1 Publisher Oxford University Press   Weight (grammes) 470 Imprint Oxford University Press   Published in Oxford Format Hardback   Height (mm) 240 Publication date 08 Jan 2009   Width (mm) 163 DEWEY 618.920042   Spine width (mm) 19 DEWEY edition DC22   Academic level Postgraduate, Professional / Scholarly

1		Family experiences: Part I, Diagnosis and early stages by Helen M. Brewer and Marie McGill		1
2		Family experiences: Part II, Later stages by Helen M. Brewer and Marie McGill		19
3		The history of juvenile Huntington's disease by Raymund A. C. Roos		33
4		The clinical phenotype of juvenile Huntington's disease by Roger A. Barker and Ferdinando Squitieri		39
5		Juvenile Huntington's disease: neuropathology by Jean Paul G. Vonsattel and Etty P. Cortes and Christian E. Keller		51
6		Molecular mechanisms in juvenile Huntington's disease by Roman Gonitel and Ferdinando Squitieri		79
7		Juvenile Huntington's disease and mouse models of Huntington's disease by Gillian P. Bates and Ben Woodman		101
8		Clinical features of early and juvenile onset in polyglutamine disorders other than Huntington's disease: autosomal dominant cerebellar ataxias and dentatorubral pallidoluysian atrophy by Andre R. Troiano and Alexandra Durr		117
9		The diagnostic challenge by Oliver W. J. Quarrell and Martha A. Nance		135
10		The treatment of juvenile Huntington's disease by Martha A. Nance		151
11		Psychosocial issues surrounding juvenile Huntington's disease by Helen M. Brewer and Aimee Aubeeluck		167
12		Challenges in assessment by Helen M. Brewer and Roger A. Barker and Oliver W. J. Quarrell		181
Appendices		Proposed scales for juvenile Huntington's disease		189
1		JHD total functional capacity		189
2		JHD functional assessment		190
3		JHD motor assessment		191
		Index		199

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