Book Review: Juvenile Huntington’s Disease and Other Trinucleotide Repeat Disorders

Abstract

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. 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.