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 recognize the characteristic features of the condition. While several textbooks have been published on HD, no books have been published to date focusing solely on the juvenile onset form of the disease. This book summarizes, 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 party European Huntington's Disease Network (EDHN), this book forms the first comprehensive text on JHD and is of interest to neurologists, geneticists, academic/research scientists, and other healthcare professionals.