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Paraproteinemia and neuropathy.

Haruki Koike1, Masahisa Katsuno2

  • 1Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, 466-8550, Japan. koike-haruki@med.nagoya-u.ac.jp.

Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
|September 16, 2021
PubMed
Summary

Paraproteinemia causes peripheral neuropathies like anti-MAG neuropathy, AL amyloidosis, and POEMS syndrome. Early diagnosis is crucial as new treatments emerge for these complex conditions.

Keywords:
Monoclonal gammopathyPOEMS syndromeParaproteinPlasma cell dyscrasiaUncompacted myelin lamellaeWidely spaced myelin

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Area of Science:

  • Neurology
  • Immunology
  • Oncology

Background:

  • Paraproteinemia is linked to diverse peripheral neuropathies.
  • Key types include anti-MAG neuropathy, AL amyloidosis, and POEMS syndrome.
  • These neuropathies involve immunoglobulin deposition or paraneoplastic mechanisms.

Purpose of the Study:

  • To review the mechanisms and diagnostic criteria of paraproteinemic neuropathies.
  • To discuss current and emerging therapeutic strategies.
  • To emphasize the importance of early and accurate diagnosis.

Main Methods:

  • Literature review of paraproteinemic neuropathies.
  • Analysis of diagnostic criteria, including electrodiagnostic findings.
  • Summary of treatment options for different subtypes.

Main Results:

  • Anti-MAG neuropathy and POEMS syndrome show nerve conduction slowing, meeting CIDP criteria.
  • AL amyloidosis can also present with CIDP-like features.
  • Conventional CIDP therapies are often ineffective; targeted treatments are emerging.

Conclusions:

  • Paraproteinemic neuropathies require specific diagnostic approaches.
  • Emerging targeted therapies offer new hope for patients.
  • Early diagnosis is essential for effective management and improved outcomes.