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What Is in the Literature.

Mark B Bromberg1

  • 1Department of Neurology, University of Utah, Salt Lake City, UT.

Journal of Clinical Neuromuscular Disease
|August 25, 2020
PubMed
Summary

This review highlights practical insights into diagnosing and managing chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and Guillain-Barré syndrome. It emphasizes adherence to guidelines for accurate diagnosis and effective treatment of these peripheral neuropathies.

Area of Science:

  • Neurology
  • Clinical Neuroscience
  • Peripheral Nervous System Disorders

Background:

  • Diagnostic and treatment guidelines for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) exist but are inconsistently applied, leading to misdiagnoses and delayed treatment.
  • Secondary axonal loss in CIDP contributes to increased connective tissue in muscles.
  • Antibodies targeting the node of Ranvier are identified in a subset of CIDP patients.

Purpose of the Study:

  • To provide new and practical information on the diagnosis, treatment, and management of peripheral neuropathies.
  • To address challenges in the clinical application of CIDP guidelines.
  • To discuss differential diagnoses and diagnostic criteria for CIDP-like conditions.

Main Methods:

  • Literature review focusing on current diagnostic and therapeutic approaches for CIDP and related neuropathies.

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  • Analysis of diagnostic criteria, including cerebrospinal fluid parameters and clinical signs.
  • Discussion of differential diagnoses such as amyloid neuropathy and POEMS syndrome.

    • Main Results:

    • Non-adherence to CIDP guidelines results in diagnostic errors and prolonged treatment.
    • CIDP can lead to secondary axonal loss and increased muscle connective tissue.
    • Cerebrospinal fluid protein and cell count upper limits may require re-evaluation.
    • Hyperactive reflexes do not rule out Guillain-Barré syndrome.
    • A second dose of intravenous immune globulin is unlikely to benefit severely affected Guillain-Barré syndrome patients within 4 weeks of onset.

    Conclusions:

    • Accurate diagnosis and management of CIDP require strict adherence to established guidelines.
    • Understanding the pathophysiology of CIDP, including axonal loss and antibody involvement, is crucial.
    • Differential diagnosis of CIDP-like neuropathies necessitates considering conditions like amyloidosis and POEMS syndrome.
    • Clinical presentation of Guillain-Barré syndrome can be variable, and treatment protocols need refinement.