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Huntington's disease is a genetically inherited condition which results in severe nerve-cell damage in the brain. The hereditary and debilitative nature of the disease means that many people are involved either directly or indirectly by this condition. This book has been written for patients and the families and carers of people with Huntington's disease (HD). It provides an accessible introduction to the condition that explains the physical, behavioural, and emotional features of the disease, the important role of genetics and options for those at risk of developing the condition, plus advice on managing and treating symptoms. Patient perspectives are included throughout, offering real-life accounts from people who have or know someone with the condition.This new edition has been fully updated to include all the latest research in HD. It gives more detail on the standard treatments, as well as explaining new trials aimed at reducing the level of the abnormal protein (huntingtin) in the brains of patients.
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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.