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.
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Loavenbruck, A.J., Engelstad, J.K., Klein, C.J. (2015). Peripheral Nerve Amyloidosis. 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_31
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