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Author:   G. Bruyn ,  Michael R. Hayden
Publisher:   Springer-Verlag Berlin and Heidelberg GmbH & Co. KG
Imprint:   Springer-Verlag Berlin and Heidelberg GmbH & Co. K
Edition:   Edition. ed.
Weight:   0.535kg
ISBN:  

9783540105886

ISBN 10:   3540105883
Pages:   192
Publication Date:   01 October 1981
Audience:   Professional and scholarly ,  Professional & Vocational
Format:   Hardback
Publisher's Status:   Active
Availability:   Out of stock  
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Table of Contents

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.- 2.2.1. United States of America.- 2.2.2. South Africa.- 2.2.3. Canada.- 2.2.4. Australia.- 2.2.5. The Caribbean.- 2.2.6. The Indian Subcontinent.- 2.2.7. Venezuela.- 2.2.8. Moray Firth Area of Scotland.- 2.2.9. Japan.- 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.- 3.2.1. Problems in the Comparison of Prevalence Data.- 3.2.2. Areas of High Prevalence: Possible Contributing Factors.- 3.2.3. Areas of Low Prevalence: Possible Contributing Factors.- 3.3. The Epidemiology of Juvenile Huntington's Chorea.- 3.4. Incidence.- 3.4.1. International Comparison.- 3.5. Mortality Data.- 3.5.1. International Comparison.- 4 Natural History.- 4.1. Age at Onset.- 4.1.1. International Comparison.- 4.1.2. The Relevance of Age at Onset to Genetic Counselling.- 4.2. Age at Death.- 4.2.1. International Comparison.- 4.3. Duration.- 4.4. Factors Modifying the Action of the Gene.- 4.4.1. Genetic Considerations.- 4.4.2. Environmental Factors.- 5 Clinical Features.- 5.1. The Presenting Symptoms and Signs.- 5.2. General Manifestations.- 5.3. Neurological Features.- 5.3.1. Chorea.- 5.3.2. Hypertonicity: Rigidity and Spasticity.- 5.3.3. Dysarthria.- 5.3.4. Dysphagia.- 5.3.5. Disturbance of Gait.- 5.3.6. Oculomotor Dysfunction.- 5.3.7. Epilepsy.- 5.3.8. Incontinence.- 5.3.9. Cerebellar Signs.- 5.3.10. Other Neurological Signs.- 5.4. Mental Disturbance.- 5.4.1. Dementia.- 5.4.2. Affective Disturbance.- 5.4.3. Change of Personality.- 5.4.4. Schizophreniform Psychosis.- 5.4.5. Other Psychiatric Symptoms.- 5.5. Staging.- 5.6. Variants.- 5.6.1. The Westphal Variant.- 5.6.2. Juvenile Huntington's Chorea.- 5.6.3. Other Variants.- 5.7. The Clinical Features of Juvenile Huntington's Chorea.- 5.8. Diagnostic Techniques.- 5.8.1. Electroencephalographic Studies (EEG).- 5.8.2. Pneumoencephalographic Studies (PEG).- 5.8.3. Computerised Axial Tomography (CAT).- 5.8.4. Cerebral Angiography.- 5.9. Problems of Diagnosis.- 5.9.1. Misdiagnosis.- 5.9.2. The Differential Diagnosis of Inherited Chorea.- 6 Neuropathology.- 6.1. Gross Pathology.- 6.2. Findings on Light Microscopy.- 6.2.1. Leptomeninges.- 6.2.2. Cerebral Cortex.- 6.2.3. The Caudate Nucleus and Putamen.- 6.2.4. Pathological Changes in Other Parts of the NervousSystem.- 6.3. Ultrastructural Features.- 6.3.1. Cerebral Cortex.- 6.3.2. The Striatum.- 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.- 7.5.1. Predominance of Paternal Descent Ill.- 7.5.2. Familial Aggregation.- 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.- 8.1.1. Psychological Defence Mechanisms.- 8.1.2. Suicide.- 8.1.3. Coping: Guidelines for Management.- 8.2. The Experience of Being At Risk.- 8.2.1. Some Guidelines for Counselling Those At Risk.- 8.2.2. Marriage and Parenthood: The Options.- 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 Economic Burden.- 9 Management.- 9.1. A Rational Approach to Pharmacotherapy.- 9.1.1. Chorea.- 9.1.2. Rigidity.- 9.1.3. Epilepsy.- 9.1.4. Dementia.- 9.1.5. Affective Disturbance.- 9.1.6. Psychosis.- 9.2. Surgery.- 9.3. Other Therapeutic Modalities.- 9.3.1. Physical Therapy.- 9.3.2. Speech Therapy.- 9.3.3. Occupational Therapy.- 9.3.4. Nutrition.- 9.3.5. Nursing Care.- 9.3.6. Social Work.- 9.3.7. Psychotherapy.- 9.3.8. Lay Groups.- 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.- 10.4.1. Membrane Abnormalities.- 10.4.2. Fibroblast Activity.- 10.4.3. Immunological Abnormalities.- 10.5. Viruses.- 10.6. Neurochemistry.- 10.6.1. Dopamine.- 10.6.2. Gamma-aminobutyric acid (GABA).- 10.6.3. Acetylcholine.- 10.6.4. Serotonin.- 10.6.5. Peptides.- 10.6.6. Neurotransmitter Receptor Abnormalities.- 10.7. Neuroendocrine Disturbances.- 10.8. Predictive Tests.- 10.8.1. Ethical Dilemmas.- 10.8.2. Attempts at Presymptomatice Diagnosis.- 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.

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