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Amyloid and Related Disorders pp 121–131Cite as

Amyloid Deposition in the Central Nervous System

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Part of the book series: Current Clinical Pathology ((CCPATH))

Abstract

Amyloidoses are protein conformational disorders that lead to aggregation and formation of insoluble fibrils with a β-pleated sheet structure. Amyloid deposition in the central nervous system (CNS) is associated with several distinct clinicopathologic disorders. The predominant form of amyloid deposition in the CNS is derived from the amyloid β precursor protein (AβPP), an intrinsic transmembrane protein. Aβ amyloid forms the core of extracellular neuritic plaques in Alzheimer disease and is also deposited in vessel walls in Alzheimer disease and in the most common form of amyloid angiopathy. PrP forms amyloid deposits in several forms of prion disease, while AL amyloid may take the form of larger, tumefactive deposits known as amyloidomas associated with localized clonal plasma cell proliferations. Infrequent forms of amyloid deposition, sometimes associated with genetic syndromes, include transthyretin, BR12, gelsolin, and Cystatin C in several inherited forms of amyloid angiopathy. Systemic amyloidosis may also involve the brain secondarily, where it usually is limited to regions lacking a blood brain barrier. In this review, we discuss the pathologic features of the various amyloid deposits involving the CNS as well as current concepts regarding their pathogenesis.

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Authors and Affiliations

  1. Department of Pathology, Division of Neuropathology, Johns Hopkins Hospital, Sheikh Zayed Tower, Room M2101, 1800 Orleans Street, Baltimore, MD, 21231, USA

    Fausto J. Rodriguez MD

  2. Department of Pathology, Loyola University Medical Center, Loyola University Chicago, Building 110, Room 2242, 2160 South First Avenue, Maywood, IL, 60153, USA

    Maria M. Picken MD, PhD

  3. Department of Pathology and Laboratory Medicine, NorthShore University Health System, Room 1914, 2650 Ridge Avenue, Evanston, IL, 60201, USA

    John M. Lee MD, PhD

Authors
  1. Fausto J. Rodriguez MD
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  2. Maria M. Picken MD, PhD
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  3. John M. Lee MD, PhD
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Correspondence to Fausto J. Rodriguez MD .

Editor information

Editors and Affiliations

  1. Department of Pathology, Loyola University Medical Center, Maywood, Illinois, USA

    Maria M. Picken

  2. Department of Pathology, LSU Health Shreveport, Shreveport, Louisiana, USA

    Guillermo A. Herrera

  3. Department of Pathology and Laboratory Medicine, Department of Pathology and Laboratory Medicine, New York, New York, USA

    Ahmet Dogan

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Rodriguez, F.J., Picken, M.M., Lee, J.M. (2015). Amyloid Deposition in the Central Nervous System. In: Picken, M., Herrera, G., Dogan, A. (eds) Amyloid and Related Disorders. Current Clinical Pathology. Humana Press, Cham. https://doi.org/10.1007/978-3-319-19294-9_8

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  • DOI: https://doi.org/10.1007/978-3-319-19294-9_8

  • Publisher Name: Humana Press, Cham

  • Print ISBN: 978-3-319-19293-2

  • Online ISBN: 978-3-319-19294-9

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