The Wide Spectrum of Pathophysiologic Mechanisms of Paraproteinemic Neuropathy

Jean-Michel Vallat; Mathilde Duchesne; Philippe Corcia; Laurence Richard; Karima Ghorab; Laurent Magy; Stéphane Mathis

doi:10.1212/WNL.0000000000011324

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.

Authors

Jean-Michel Vallat¹, Mathilde Duchesne², Philippe Corcia², Laurence Richard², Karima Ghorab², Laurent Magy², Stéphane Mathis²

Affiliations

¹ From the Department of Neurology (J.-M.V., M.D., L.R., K.G., L.M.), National Reference Center for "Rare Peripheral Neuropathies," Dupuytren University Hospital (CHU Limoges), University of Limoges; Department of Pathology (M.D.), Limoges University Hospital (CHU Limoges), University of Limoges; Department of Neurology and ALS Reference Center (P.C.), Bretonneau University Hospital (CHU Tours), University of Tours; and Department of Neurology (S.M.), Nerve-Muscle Unit, 4 Pellegrin University Hospital (CHU Bordeaux), University of Bordeaux, France. [email protected].
² From the Department of Neurology (J.-M.V., M.D., L.R., K.G., L.M.), National Reference Center for "Rare Peripheral Neuropathies," Dupuytren University Hospital (CHU Limoges), University of Limoges; Department of Pathology (M.D.), Limoges University Hospital (CHU Limoges), University of Limoges; Department of Neurology and ALS Reference Center (P.C.), Bretonneau University Hospital (CHU Tours), University of Tours; and Department of Neurology (S.M.), Nerve-Muscle Unit, 4 Pellegrin University Hospital (CHU Bordeaux), University of Bordeaux, France.

PMID: 33277411
DOI: 10.1212/WNL.0000000000011324

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