CIDP mimics: a case series

BMC Neurology

Chafic Karam¹  Chahin Nizar²  Erik Ensrud²  Diana Dimitrova²  Orly Moshe-Lilie²  Thomas Ragole² 

[1] Department of Neurology, Hospital of the University of Pennsylvania, 3400 Spruce St., 3 West Gates, 19104, Philadelphia, PA, USA;Department of Neurology, Oregon Health & Science University, Portland, OR, USA;

关键词: CIDP;    CIDP mimics;    POEMS;    CANOMAD;    Neurolymphomatosis;   

DOI  :  10.1186/s12883-021-02118-7

来源: Springer

【 摘 要 】

BackgroundTo 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.MethodsPatients 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.ResultsSeven 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.ConclusionPain, 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.

【 授权许可】

CC BY