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Paraproteinemic Neuropathy.

Matthew P. Wicklund1, John T. Kissel

  • 1Department of Neurology, Division of Neuromuscular Disease, The Ohio State University, 1654 Upham Drive, Columbus, OH 43210, USA. Kissel@osu.edu

Current Treatment Options in Neurology
|February 17, 2001
PubMed
Summary

Treatment for neuropathies linked to monoclonal gammopathies varies by underlying cause. Management strategies include targeting the primary disease, using specific therapies like interferon-alpha for cryoglobulinemia, or employing plasma exchange and immunomodulatory drugs for certain MGUS-related neuropathies.

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Area of Science:

  • Neurology
  • Hematology
  • Immunology

Background:

  • Neuropathies associated with monoclonal gammopathies (paraproteins) lack extensive prospective, randomized, placebo-controlled trials.
  • Clinical recommendations are largely based on experience and uncontrolled studies.
  • Therapeutic decisions must be tailored to the specific clinical context of the paraprotein.

Purpose of the Study:

  • To provide guidance on managing neuropathies in the setting of monoclonal gammopathies.
  • To review current treatment approaches based on the underlying paraprotein and clinical syndrome.
  • To highlight areas where optimal treatment remains to be established.

Main Methods:

  • Review of clinical experience and existing literature, including uncontrolled and open-label trials.

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  • Categorization of treatment strategies based on associated conditions (e.g., multiple myeloma, POEMS syndrome, MGUS, cryoglobulinemia).
  • Discussion of therapeutic options for specific paraprotein types, including plasma exchange, immunomodulatory drugs, and supportive care.

    • Main Results:

    • Treatment efficacy varies significantly depending on the underlying monoclonal gammopathy.
    • For multiple myeloma, amyloidosis, and Waldenström's macroglobulinemia, treating the underlying disease is paramount.
    • Interferon-alpha is effective for hepatitis C-related cryoglobulinemic neuropathy; radiation therapy is effective for POEMS syndrome.

    Conclusions:

    • Neuropathies associated with monoclonal gammopathies require individualized treatment strategies.
    • Plasma exchange shows efficacy in certain MGUS-related neuropathies (IgG, IgA).
    • Treatment for IgM monoclonal gammopathies, particularly with anti-MAG reactivity, remains challenging, with newer agents like rituximab showing promise.