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The Neuropathology of Huntington’s Disease: Classical Findings, Recent Developments and Correlation to Functional Neuroanatomy

  • Book
  • © 2015

Overview

Authors:
  1. Udo Rüb

    1. Dr. Senckenbergisches Chronomedizinisches Institut, Goethe University Frankfurt, Frankfurt, Germany

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  2. Jean Paul G. Vonsattel

    1. Medical Center Neurological Institute, Columbia University, New York, USA

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  3. Helmut Heinsen

    1. Division Psychiatric clinic Morphological Brain Research Unit, Julius Maximilians University Würzburg, Würzburg, Germany

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  4. Horst-Werner Korf

    1. Dr. Senckenbergisches Chronomedizinisches Institut, Goethe-University, Frankfurt, Germany

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  • Describes the progress made during more than hundred years of neuropathological HD research, the neuropathological hallmarks of HD and their pathogenic and clinical relevance
  • Explains the potential pathophysiological role of axonal transport deficits and impairments of the neuronal protein quality control machinery in the disease process
  • Illustrates the potential pathophysiological role of neuronal huntingtin aggregations and nerve cell alterations, as well as the possible significance of transneuronal mechanisms for the spread of the neurodegenerative process of HD

Part of the book series: Advances in Anatomy, Embryology and Cell Biology (ADVSANAT, volume 217)

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Table of contents (11 chapters)

  1. Front Matter

    Pages i-xiv
    Download chapter PDF

  2. Introduction

    • Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 1-5

  3. The Neuropathological Grading of Huntington’s Disease (HD)

    • Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 7-23

  4. The Cerebral Cortex in Huntington’s Disease (HD)

    • Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 25-39

  5. Degeneration of Select Motor and Limbic Nuclei of the Thalamus in Huntington’s Disease (HD)

    • Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 41-53

  6. Consistent and Widespread Degeneration of the Cerebellum in Huntington’s Disease (HD)

    • Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 55-66

  7. Elucidation of the Role of the Premotor Oculomotor Brainstem Nuclei in the Pathogenesis of Oculomotor Dysfunctions in Huntington’s Disease (HD)

    • Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 67-82

  8. Widespread Brainstem Neurodegeneration in Huntington’s Disease (HD)

    • Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 83-90

  9. Intraneuronal Transport and Defense Mechanisms with Possible Pathogenetic Relevance in Huntington’s Disease (HD)

    • Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 91-100

  10. The Disease Protein Huntingtin and Neuronal Protein Aggregations in Huntington’s Disease (HD)

    • Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 101-117

  11. Pathological Nerve Cell Alterations in Huntington’s Disease (HD) and Their Possible Role for the Demise of Nerve Cells

    • Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 119-123

  12. Conclusions and Outlook

    • Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 125-131

  13. Back Matter

    Pages 133-146
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Keywords

About this book

This monograph describes the progress in neuropathological HD research made during the last century, the neuropathological hallmarks of HD and their pathogenic relevance. Starting with the initial descriptions of the progressive degeneration of the striatum as one of the key events in HD, the worldwide practiced Vonsattel HD grading system of striatal neurodegeneration will be outlined. Correlating neuropathological data with results on the functional neuroanatomy of the human brain, subsequent chapters will highlight recent HD findings: the neuronal loss in the cerebral neo-and allocortex, the neurodegeneration of select thalamic nuclei, the affection of the cerebellar cortex and nuclei, the involvement of select brainstem nuclei, as well as the pathophysiological relevance of these pathologies for the clinical picture of HD. Finally, the potential pathophysiological role of neuronal huntingtin aggregations and the most important and enduring challenges of neuropathological HD research are discussed.

Reviews

“This multiauthored monograph is a comprehensive overview of Huntington’s disease by experts in this field. … The photographs are of consistently high quality. The captions are complete and accurate. … For a basic scientist, neurologist, neuropathologist, or anyone with an interest in neurodegenerative disease, this book provides a thorough review and authoritative update on Huntington’s disease. It is a reliable resource and a complete academic delight.” (Sherry Krawitz, Doody’s Book Reviews, March, 2016)

Authors and Affiliations

  • Dr. Senckenbergisches Chronomedizinisches Institut, Goethe University Frankfurt, Frankfurt, Germany

    Udo Rüb

  • Medical Center Neurological Institute, Columbia University, New York, USA

    Jean Paul G. Vonsattel

  • Division Psychiatric clinic Morphological Brain Research Unit, Julius Maximilians University Würzburg, Würzburg, Germany

    Helmut Heinsen

  • Dr. Senckenbergisches Chronomedizinisches Institut, Goethe-University, Frankfurt, Germany

    Horst-Werner Korf

Bibliographic Information

  • Book Title: The Neuropathology of Huntington’s Disease: Classical Findings, Recent Developments and Correlation to Functional Neuroanatomy

  • Authors: Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf

  • Series Title: Advances in Anatomy, Embryology and Cell Biology

  • DOI: https://doi.org/10.1007/978-3-319-19285-7

  • Publisher: Springer Cham

  • eBook Packages: Biomedical and Life Sciences, Biomedical and Life Sciences (R0)

  • Copyright Information: The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Switzerland AG 2015

  • Softcover ISBN: 978-3-319-19284-0Published: 08 October 2015

  • eBook ISBN: 978-3-319-19285-7Published: 29 September 2015

  • Series ISSN: 0301-5556

  • Series E-ISSN: 2192-7065

  • Edition Number: 1

  • Number of Pages: XIV, 146

  • Number of Illustrations: 3 b/w illustrations, 47 illustrations in colour

  • Topics: Neurosciences, Human Genetics, Pathology

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