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© 2015

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

Benefits

  • 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

Book

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

Table of contents

  1. Front Matter
    Pages i-xiv
  2. Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 1-5
  3. Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 7-23
  4. Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 25-39
  5. Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 41-53
  6. Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 55-66
  7. Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 83-90
  8. Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 91-100
  9. Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 101-117
  10. Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 119-123
  11. Udo Rüb, Jean Paul G. Vonsattel, Helmut Heinsen, Horst-Werner Korf
    Pages 125-131
  12. Back Matter
    Pages 133-146

About this book

Introduction

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.

Keywords

CAG-repeats Vonsattel HD grading system chromosome 4p16.3 neurological disorder neuronal loss striatal neurodegeneration

Authors and affiliations

  1. 1.Dr. Senckenbergisches Chronomedizinisches InstitutGoethe University FrankfurtFrankfurtGermany
  2. 2.Medical Center Neurological InstituteColumbia UniversityNew YorkUSA
  3. 3.Division Psychiatric clinic Morphological Brain Research UnitJulius Maximilians University WürzburgWürzburgGermany
  4. 4.Dr. Senckenbergisches Chronomedizinisches InstitutGoethe-UniversityFrankfurtGermany

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
  • Series Abbreviated Title Advs Anatomy
  • DOI https://doi.org/10.1007/978-3-319-19285-7
  • Copyright Information Springer International Publishing Switzerland 2015
  • Publisher Name Springer, Cham
  • eBook Packages Biomedical and Life Sciences Biomedical and Life Sciences (R0)
  • Softcover ISBN 978-3-319-19284-0
  • eBook ISBN 978-3-319-19285-7
  • 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
  • Buy this book on publisher's site
Industry Sectors
Biomedicine
Biotechnology
Health & Hospitals
Pharma

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)