TY  - BOOK
AU  - Quarrell,Oliver
TI  - Juvenile Huntington's disease: (and other trinucleotide repeat disorders) 
SN  - 9780191575280
PY  - 2009///
CY  - Oxford, New York
PB  - Oxford University Press
KW  - Huntington's disease
KW  - Genetic disorders in children
KW  - Trinucleotide repeats
KW  - Children
KW  - Adolescent
KW  - Child
KW  - Huntington Disease
KW  - Trinucleotide Repeat Expansion
N1  - Includes bibliographical references and index; Dedication; Preface; Foreword; Contents; List of contributors; 1 Family experiences: Part I, Diagnosis and early stages; 2 Family experiences: Part II, Later stages; 3 The history of juvenile Huntington's disease; 4 The clinical phenotype of juvenile Huntington's disease; 5 Juvenile Huntington's disease: neuropathology; 6 Molecular mechanisms in juvenile Huntington's disease; 7 Juvenile Huntington's disease and mouse models of Huntington's disease; 8 Clinical features of early and juvenile onset in polyglutamine disorders other than Huntington's disease: autosomal dominant cerebellar ataxias and dentatorubral pallidoluysian atrophy9 The diagnostic challenge; 10 The treatment of juvenile Huntington's disease; 11 Psychosocial issues surrounding juvenile Huntington's disease; 12 Challenges in assessment; Appendices: Proposed scales for juvenile Huntington's disease; 1 JHD total functional capacity; 2 JHD functional assessment; 3 JHD motor assessment; Index; A; B; C; D; E; F; G; H; I; J; K; L; M; N; O; P; Q; R; S; T; U; V; W
N2  - 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 includingseizures, dystonia and Parkinsonism. Diagnosis in patients with JHD is often delayed because of the failure of clini
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