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The Neuropathology of Huntington’s Disease

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Behavioral Neurobiology of Huntington's Disease and Parkinson's Disease

Part of the book series: Current Topics in Behavioral Neurosciences ((CTBN,volume 22))

Abstract

The basal ganglia are a highly interconnected set of subcortical nuclei and major atrophy in one or more regions may have major effects on other regions of the brain. Therefore, the striatum which is preferentially degenerated and receives projections from the entire cortex also affects the regions to which it targets, especially the globus pallidus and substantia nigra pars reticulata. Additionally, the cerebral cortex is itself severely affected as are many other regions of the brain, especially in more advanced cases. The cell loss in the basal ganglia and the cerebral cortex is extensive. The most important new findings in Huntington’s disease pathology is the highly variable nature of the degeneration in the brain. Most interestingly, this variable pattern of pathology appears to reflect the highly variable symptomatology of cases with Huntington’s disease even among cases possessing the same number of CAG repeats.

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Authors and Affiliations

  1. Centre for Brain Research, Department of Anatomy with Radiology, University of Auckland, Auckland, New Zealand

    Henry J. Waldvogel, Eric H. Kim & Richard LM Faull

  2. Department of Pathology, Presbyterian Hospital, Columbia University, New York, NY10032, USA

    Jean-Paul G. Vonsattel

  3. Centre for Brain Research, Department of Psychology, University of Auckland, Auckland, New Zealand

    Lynette J. Tippett

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  1. Henry J. Waldvogel
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  2. Eric H. Kim
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  3. Lynette J. Tippett
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  4. Jean-Paul G. Vonsattel
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  5. Richard LM Faull
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Correspondence to Henry J. Waldvogel .

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  1. Institute of Medical Genetics and Applied Genomics, University of Tuebingen, Tübingen, Germany

    Hoa Huu Phuc Nguyen

  2. Dept of Experimental Medical Science, Lund University, Lund, Sweden

    M. Angela Cenci

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Waldvogel, H.J., Kim, E.H., Tippett, L.J., Vonsattel, JP.G., Faull, R.L. (2014). The Neuropathology of Huntington’s Disease. In: Nguyen, H., Cenci, M. (eds) Behavioral Neurobiology of Huntington's Disease and Parkinson's Disease. Current Topics in Behavioral Neurosciences, vol 22. Springer, Berlin, Heidelberg. https://doi.org/10.1007/7854_2014_354

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