Huntington’s Chorea

1 Historical Background.- 1.1. Chorea in the Middle Ages: The Dancing Mania.- 1.2. Thomas Sydenham and Chorea.- 1.3. From Sydenham to Huntingdon: The First Descriptions of Inherited Chorea.- 1.4. Huntington’s Chorea.- 2 Genealogy and Geographic Distribution.- 2.1. Genealogical Methods.- 2.2. Genealogical Investigations in Various Parts of the World.- 2.3. The Importance of Genealogical Investigations.- 2.4. The Original Source of the Gene for Huntington’s Chorea.- 3 Epidemiology.- 3.1. Diagnostic Critera.- 3.2. Prevalence.- 3.3. The Epidemiology of Juvenile Huntington’s Chorea.- 3.4. Incidence.- 3.5. Mortality Data.- 4 Natural History.- 4.1. Age at Onset.- 4.2. Age at Death.- 4.3. Duration.- 5 Clinical Features.- 5.1. The Presenting Symptoms and Signs.- 5.2. General Manifestations.- 5.3. Neurological Features.- 5.4. Mental Disturbance.- 5.5. Staging.- 5.6. Variants.- 5.7. The Clinical Features of Juvenile Huntington’s Chorea.- 5.8. Diagnostic Techniques.- 5.9. Problems of Diagnosis.- 6 Neuropathology.- 6.1. Gross Pathology.- 6.2. Findings on Light Microscopy.- 6.3. Ultrastructural Features.- 6.4. The Importance of Post-mortem Examination.- 6.5. The Brain and Tissue Bank.- 7 Genetics.- 7.1. Mutations.- 7.2. Heterozygote Frequency.- 7.3. The Homozygous Form.- 7.4. Heterogeneity.- 7.5. Unusual Aspects of the Genetics of Juvenile Huntington’s Chorea Ill.- 7.6. Genetic Registers.- 7.7. Genetic Counselling.- 7.8. Reproductive Fitness.- 8 Living with Huntington’s Chorea: The Social Perspective.- 8.1. Psychosocial Consequences for the Affected Person.- 8.2. The Experience of Being At Risk.- 8.3. The Burden on the Unaffected Spouse.- 8.4. Huntington’s Chorea: A Family Disease.- 8.5. Antisocial Behaviour: Huntington’s Chorea and the Law.- 8.6. The EconomicBurden.- 9 Management.- 9.1. A Rational Approach to Pharmacotherapy.- 9.2. Surgery.- 9.3. Other Therapeutic Modalities.- 9.4. Future Prospects.- 10 Current Trends in Research.- 10.1. A Unifying Conceptual Approach.- 10.2. Investigations of the Abnormal Gene.- 10.3. The Search for the Altered Gene Product.- 10.4. The Investigation of Disturbed Cell Function/Structure.- 10.5. Viruses.- 10.6. Neurochemistry.- 10.7. Neuroendocrine Disturbances.- 10.8. Predictive Tests.- 10.9. Conclusion.- Appendixes.- 1. The Use of Conditional Probabilities in Genetic Counselling for Huntington’s Chorea.- 2. Method for Determination of the Mutation Rate in Huntington’s Chorea...- 3. Method for Determination of the Heterozygote Frequency in Huntington’s Chorea.- 4. Name of Addresses of Lay Organisations and Other Centres for Information on Huntington’s Chorea.- 5. Brain Donation Programme.