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Peripheral Nerve Amyloidosis

  • Adam J. LoavenbruckEmail author
  • JaNean K. Engelstad
  • Christopher J. Klein
Part of the Current Clinical Pathology book series (CCPATH)

Abstract

In this chapter, we provide an overview of peripheral nerve amyloidosis, focusing on both universal and subtype-specific pathologic features. In the great majority of cases of amyloidosis involving peripheral nerve, amyloid fibrils are either formed by immunoglobulin light chains stemming from systemic plasma cell dyscrasia (primary or AL amyloidosis) or else mutant transthyretin (TTR). Peripheral neural involvement is a common feature and hallmark of amyloidosis—reported to occur in 17–35 % of AL amyloidosis cases, and in a majority of cases of TTR amyloidosis. Other than AL and TTR amyloidosis, only rare kindreds with a short list of amyloidogenic mutations, such as gelsolin and apolipoprotein A1, are known to develop amyloid neuropathy. Pathologic features are outlined in detail in the context of these amyloid subtypes, and clinical details and issues are reported briefly.

Keywords

Amyloidosis Peripheral neuropathy Amyloid neuropathy Peripheral nerve amyloidosis Autonomic neuropathy Nerve pathology Nerve biopsy 

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Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  • Adam J. Loavenbruck
    • 1
    Email author
  • JaNean K. Engelstad
    • 2
  • Christopher J. Klein
    • 2
  1. 1.Kennedy Laboratory, Department of NeurologyUniversity of MinnesotaMinneapolisUSA
  2. 2.Peripheral Nerve LaboratoryMayo ClinicRochesterUSA

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