Paraproteinemic neuropathies

Brain Pathol. 2001 Oct;11(4):399-407. doi: 10.1111/j.1750-3639.2001.tb00407.x.


The occurrence of a peripheral neuropathy (PN) in association with a monoclonal gammopathy is quite common and suggests that monoclonal proteins may play a pathogenetic role in peripheral nervous system damage. In fact, paraproteinemic PN constitute an heterogeneous group of disorders related to various pathogenetic factors, and the histopathologic features in peripheral nerve biopsies differ from one condition to another. In several well defined disorders, the responsibility of the monoclonal component in the development of the PN has been evidenced. This is the case for most of the PN associated with an IgM monoclonal gammopathy, either a monoclonal gammopathy of undetermined significance (MGUS) or Waldenstrom's macroglobulinemia. The responsibility of the monoclonal protein in the occurrence of amyloid neuropathy related to multiple myeloma is also recognized. However, most IgG or IgA MGUS, as well as the monoclonal component in POEMS syndrome, have an uncertain causal relationship with the neuropathy. PN associated with monoclonal cryoglobulin (type 1) are occasional and differ from those associated with mixed cryoglobulins (types 2 or 3).

Publication types

  • Review

MeSH terms

  • Cryoglobulinemia / complications
  • Cryoglobulinemia / immunology
  • Cryoglobulinemia / physiopathology
  • Humans
  • Immunoglobulin M / immunology*
  • Multiple Myeloma / complications
  • Multiple Myeloma / immunology
  • Multiple Myeloma / physiopathology
  • Myelin Sheath / immunology*
  • Myelin Sheath / pathology*
  • POEMS Syndrome / complications
  • POEMS Syndrome / immunology
  • POEMS Syndrome / physiopathology
  • Paraproteinemias / complications*
  • Paraproteinemias / immunology
  • Paraproteinemias / physiopathology
  • Peripheral Nervous System Diseases / immunology*
  • Peripheral Nervous System Diseases / pathology
  • Peripheral Nervous System Diseases / physiopathology
  • Waldenstrom Macroglobulinemia / complications
  • Waldenstrom Macroglobulinemia / immunology
  • Waldenstrom Macroglobulinemia / physiopathology


  • Immunoglobulin M