The Wide Spectrum of Pathophysiologic Mechanisms of Paraproteinemic Neuropathy

Neurology. 2021 Feb 2;96(5):214-225. doi: 10.1212/WNL.0000000000011324. Epub 2020 Dec 4.

Abstract

Monoclonal gammopathy is encountered quite frequently in the general population. This type of hematologic abnormality may be mild, referred to as monoclonal gammopathy of undetermined significance or related to different types of hematologic malignancies. The association of a peripheral neuropathy with monoclonal gammopathy is also fairly common, and hemopathy may be discovered in an investigation of peripheral neuropathy. In such a situation, it is essential to determine the exact nature of the hematologic process in order not to miss a malignant disease and thus initiate the appropriate treatment (in conjunction with hematologists and oncologists). In this respect, nerve biopsy (discussed on a case-by-case basis) is of great value in the management of such patients. We therefore propose to present the objectives and main interests of nerve biopsy in this situation.

Publication types

  • Review

MeSH terms

  • Anemia, Hemolytic, Autoimmune / diagnosis
  • Anemia, Hemolytic, Autoimmune / etiology
  • Anemia, Hemolytic, Autoimmune / pathology
  • Anemia, Hemolytic, Autoimmune / physiopathology
  • Ataxia / diagnosis
  • Ataxia / etiology
  • Ataxia / pathology
  • Ataxia / physiopathology
  • Autoantibodies / immunology
  • Biopsy
  • Decision Trees
  • Electrodiagnosis
  • Humans
  • Immunoglobulin A
  • Immunoglobulin G
  • Immunoglobulin Light-chain Amyloidosis / complications
  • Immunoglobulin Light-chain Amyloidosis / diagnosis
  • Immunoglobulin Light-chain Amyloidosis / physiopathology
  • Immunoglobulin M
  • Monoclonal Gammopathy of Undetermined Significance
  • Myelin-Associated Glycoprotein / immunology
  • Neural Conduction / physiology
  • Ophthalmoplegia / diagnosis
  • Ophthalmoplegia / etiology
  • Ophthalmoplegia / pathology
  • Ophthalmoplegia / physiopathology
  • POEMS Syndrome / diagnosis
  • POEMS Syndrome / etiology
  • POEMS Syndrome / pathology
  • POEMS Syndrome / physiopathology
  • Paraproteinemias / complications
  • Paraproteinemias / diagnosis
  • Paraproteinemias / physiopathology*
  • Peripheral Nerves / pathology
  • Peripheral Nerves / ultrastructure
  • Peripheral Nervous System Diseases / diagnosis
  • Peripheral Nervous System Diseases / etiology
  • Peripheral Nervous System Diseases / pathology
  • Peripheral Nervous System Diseases / physiopathology*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / diagnosis
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / etiology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / pathology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / physiopathology
  • Primary Dysautonomias / diagnosis
  • Primary Dysautonomias / etiology
  • Primary Dysautonomias / pathology
  • Primary Dysautonomias / physiopathology
  • Small Fiber Neuropathy / diagnosis
  • Small Fiber Neuropathy / etiology
  • Small Fiber Neuropathy / pathology
  • Small Fiber Neuropathy / physiopathology
  • Waldenstrom Macroglobulinemia

Substances

  • Autoantibodies
  • Immunoglobulin A
  • Immunoglobulin G
  • Immunoglobulin M
  • Myelin-Associated Glycoprotein

Supplementary concepts

  • CANOMAD syndrome