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POEMS综合征与意义未明的单克隆丙种球蛋白病相关神经病变的电生理特征比较。

Electrophysiologic features of POEMS syndrome compared with MGUS-related neuropathy.

作者信息

Kim Hyunjin, Lim Young-Min, Jin Joo Yea, Yoon Dok Hyun, Suh Cheolwon, Kim Sun-Young, Jo Jae-Cheol, Kim Kwang-Kuk

机构信息

Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.

Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.

出版信息

Muscle Nerve. 2017 Dec;56(6):E73-E77. doi: 10.1002/mus.25684. Epub 2017 May 30.

Abstract

INTRODUCTION

Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome and monoclonal gammopathy of undetermined significance (MGUS) are paraproteinemic disorders that can cause demyelinating polyneuropathy. Herein we assessed the findings of nerve conduction studies (NCS) in patients with POEMS syndrome and MGUS-related neuropathy to determine whether the NCS characteristics can help differentiate between these conditions.

METHODS

We enrolled 24 POEMS and 37 MGUS-related neuropathy patients. NCS parameters, including compound muscle action potential (CMAP), motor conduction velocity (MCV), and terminal latency index (TLI), were evaluated.

RESULTS

Compared with MGUS-related neuropathy patients, POEMS syndrome patients demonstrated a greater reduction in both the upper and lower limb CMAPs and a greater reduction in the median and ulnar MCVs. The TLIs were significantly higher in POEMS patients.

DISCUSSION

NCS can help distinguish POEMS syndrome from MGUS-related neuropathy. Reduced CMAPs, slow MCVs, and high TLIs are indicative of POEMS syndrome rather than MGUS-related neuropathy. Muscle Nerve 56: E73-E77, 2017.

摘要

引言

多神经病、器官肿大、内分泌病、M蛋白和皮肤改变(POEMS)综合征以及意义未明的单克隆丙种球蛋白病(MGUS)是可导致脱髓鞘性多神经病的副蛋白血症性疾病。在此,我们评估了POEMS综合征和MGUS相关神经病患者的神经传导研究(NCS)结果,以确定NCS特征是否有助于区分这些疾病。

方法

我们纳入了24例POEMS综合征患者和37例MGUS相关神经病患者。评估了NCS参数,包括复合肌肉动作电位(CMAP)、运动传导速度(MCV)和终末潜伏期指数(TLI)。

结果

与MGUS相关神经病患者相比,POEMS综合征患者上肢和下肢的CMAP均有更大程度的降低,正中神经和尺神经的MCV也有更大程度的降低。POEMS综合征患者的TLI显著更高。

讨论

NCS有助于将POEMS综合征与MGUS相关神经病区分开来。CMAP降低、MCV减慢和TLI升高提示为POEMS综合征而非MGUS相关神经病。《肌肉与神经》56: E73-E77, 2017年。

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