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. 2021 Feb 28;21(1):94.
doi: 10.1186/s12883-021-02118-7.

CIDP mimics: a case series

Orly Moshe-Lilie  1 , Erik Ensrud  1 , Thomas Ragole  1 , Chahin Nizar  1 , Diana Dimitrova  1 , Chafic Karam  2
Affiliations

CIDP mimics: a case series

Orly Moshe-Lilie et al. BMC Neurol. .

Abstract

Background: To report our experience with a group of patients referred for refractory CIDP who fulfilled "definite" electrodiagnostic EFNS criteria for CIDP but were found to have an alternate diagnosis.

Methods: Patients who were seen between 2017 and 2019 for refractory CIDP that fulfilled "definite" electrodiagnostic ENFS criteria for CIDP, but had an alternate diagnosis, were included. Patients who correctly had CIDP, anti MAG neuropathy, or MMN with conduction block, were excluded from the study. Demographics, clinical and electrophysiological characteristics, pertinent workup, final alternate diagnoses, and outcomes were collected.

Results: Seven patients were included: POEMS (n = 5), CANOMAD (n = 1), and neurolymphomatosis (n = 1). Most patients reported neuropathic pain and leg swelling (n = 6) or significant weight loss (n = 4). All patients had a monoclonal protein, and most patients who were tested had an elevated VEGF and CSF cyto-albuminologic dissociation. Electrophysiology showed pronounced intermediate more than distal demyelination, and axonal loss in the lower extremities. Response to steroids or IVIG varied, but some patients did respond to these treatments, especially early in the disease.

Conclusion: Pain, systemic symptoms, suggestive electrophysiological findings, and/or a serum monoclonal protein should raise suspicion for CIDP mimics. Initial response to steroids or IVIG, over reliance on CSF, and electrophysiology findings can all be misleading.

Keywords: CANOMAD; CIDP; CIDP mimics; Neurolymphomatosis; POEMS.

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Conflict of interest statement

Dr. Chafic Karam is Associate Editor of BMC Neurology.

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