Abstract
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.
Highlights
To report our experience with a group of patients referred for refractory chronic inflammatory demyelinating polyneuropathy (CIDP) who fulfilled “definite” electrodiagnostic EFNS criteria for CIDP but were found to have an alternate diagnosis
Over a dozen guidelines and expert consensus exist to aid with the adequate diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP)
While following the EFNS/PNS CIDP guidelines for CIDP would dramatically reduce the rate of misdiagnosis, there remains a small number of patients who fullfill these criteria for CIDP but may have an alternative diagnosis (“true CIDP mimics”) [5,6,7,8]
Summary
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. While following the EFNS/PNS CIDP guidelines for CIDP would dramatically reduce the rate of misdiagnosis, there remains a small number of patients who fullfill these criteria for CIDP but may have an alternative diagnosis (“true CIDP mimics”) [5,6,7,8]. These patients may experience delay in appropriate diagnosis and initiation of adequate therapy resulting in significant disability and morbidity [6]. Whereas most studies have focused on “over” diagnosis of CIDP, mainly in patients not fulfilling ENFS/PNS criteria, we aimed to discuss a group of patients who were referred for “refractory CIDP” and fulfilled “definite” electrophysiological ENFS/PNS criteria for CIDP, but had an alternate diagnosis
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