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Amyloid Deposition in the Central Nervous System

  • Fausto J. RodriguezEmail author
  • Maria M. Picken
  • John M. Lee
Part of the Current Clinical Pathology book series (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.

Keywords

Amyloid β AβPP Cerebral amyloid angiopathy Lobar hemorrhages Dementia Pathology Amyloidoma 

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Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  • Fausto J. Rodriguez
    • 1
    Email author
  • Maria M. Picken
    • 2
  • John M. Lee
    • 3
  1. 1.Department of Pathology, Division of NeuropathologyJohns Hopkins HospitalBaltimoreUSA
  2. 2.Department of PathologyLoyola University Medical Center, Loyola University ChicagoMaywoodUSA
  3. 3.Department of Pathology and Laboratory MedicineNorthShore University Health SystemEvanstonUSA

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