The comparison of MRN, electrophysiology and progression among typical CIDP and atypical CIDP subtypes

Yuan Feng,Xiaoyun Su,Chuansheng Zheng,Yu Zhang,Zuneng Lu

doi:10.1038/s41598-020-73104-1

Abstract

We aimed to compare the electrophysiology and magnetic resonance neurography (MRN) results of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) subtypes and to explore the progression from atypical CIDP to typical CIDP. We collected the medical records of 45 CIDP patients to analyse the rate of progression from atypical CIDP to typical CIDP subtypes. The cerebrospinal fluid (CSF) protein (p = 0.024) and overall disability sum score (ODSS) (p = 0.000) differed among patients with typical CIDP, distal acquired demyelinating symmetric neuropathy (DADS) and Lewis-Sumner syndrome (LSS). The compound motor action potential (CMAP) of typical CIDP was lower than that of the other subtypes (p = 0.016, p = 0.022 and p = 0.012). The cross-sectional area (CSA) of nerve roots in typical CIDP was significantly thicker than that of nerve roots in DADS and LSS. There were fewer DADS and LSS patients who progressed to typical CIDP than those who progressed to pure motor and pure sensory CIDP (p = 0.000), and the progression from pure motor to typical CIDP required a significantly longer time than the progression from pure sensory to typical CIDP (p = 0.007). Typical CIDP was more severe than the other subtypes not only in terms of clinical and electrophysiology factors but also in terms of MRN factors.

Highlights

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare immune-mediated disease that targets the myelin sheaths of peripheral nerves; this disease has a chronic course and often causes disabled sensory-motor neuropathy[1]
We only compared the data of typical chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), DASD and Lewis-Sumner syndrome (LSS) patients because the number of pure motor and sensory CIDP patients was too low for the analysis
We applied the diagnostic criteria for EFNS/PNS and explored the comparison of clinical features, magnetic resonance neurography (MRN), electrophysiology and progression for different CIDP subtypes, as very little research exists in the literature[20,21]

Summary

Introduction

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare immune-mediated disease that targets the myelin sheaths of peripheral nerves; this disease has a chronic course and often causes disabled sensory-motor neuropathy[1]. Some patients may initially present with pure sensory, pure motor, or LSS that evolves over a few months to a typical sensorimotor form[8,9]. Another point that should be noted is that there is no clear boundary with delineated criteria for the diagnosis of atypical CIDP. It is unclear whether CIDP should include patients with clinical manifestations of sensory impairments but both sensory and motor neuro-electromyography abnormalities or those only with clinical and electrophysiology sensory disturbances[4]. We retrospectively researched the features and frequencies of atypical CIDP conversion to typical CIDP

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