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CIDP mimics: a case series.

Orly Moshe-Lilie1, Erik Ensrud1, Thomas Ragole1

  • 1Department of Neurology, Oregon Health & Science University, Portland, OR, USA.

BMC Neurology
|February 28, 2021
PubMed
Summary

Many patients diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP) actually have other conditions. Recognizing CIDP mimics is crucial for accurate diagnosis and effective treatment of neuropathies.

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Area of Science:

  • Neurology
  • Clinical Electrophysiology
  • Immunology

Background:

  • Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare neurological disorder.
  • Patients with refractory CIDP may have alternative diagnoses.
  • Diagnostic criteria for CIDP can sometimes be met by non-CIDP conditions.

Purpose of the Study:

  • To identify and characterize patients initially diagnosed with CIDP who were found to have other conditions.
  • To highlight the importance of considering differential diagnoses in refractory CIDP cases.
  • To improve diagnostic accuracy for demyelinating neuropathies.

Main Methods:

  • Retrospective review of patients seen between 2017-2019 with refractory CIDP.
  • Inclusion criteria: fulfilled "definite" electrodiagnostic EFNS criteria for CIDP but had an alternate diagnosis.
Keywords:
CANOMADCIDPCIDP mimicsNeurolymphomatosisPOEMS

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  • Exclusion criteria: confirmed CIDP, anti-MAG neuropathy, or MMN with conduction block.
  • Data collected: demographics, clinical and electrophysiological findings, workup, final diagnoses, and outcomes.

    • Main Results:

    • Seven patients were identified with alternate diagnoses: POEMS syndrome (n=5), CANOMAD (n=1), and neurolymphomatosis (n=1).
    • Common symptoms included neuropathic pain, leg swelling, and weight loss.
    • All patients had a monoclonal protein; elevated VEGF and CSF cyto-albuminologic dissociation were frequent.
    • Electrophysiology showed demyelination and axonal loss, particularly in the lower extremities.
    • Variable response to steroids or IVIG was observed, with some benefit noted early in the disease.

    Conclusions:

    • Systemic symptoms, monoclonal proteins, and specific electrophysiological findings can mimic CIDP.
    • Over-reliance on CSF analysis and electrophysiology can be misleading.
    • Early recognition of CIDP mimics is essential for appropriate management and improved patient outcomes.