多发性神经病伴 POEMS 运动传导阻滞。

IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY
Muscle & Nerve Pub Date : 2024-11-22 DOI:10.1002/mus.28302
Handan Uzunçakmak Uyanık, Fatma Gökçem Yıldız, Bahar Gülmez, Ersin Tan, Çağrı Mesut Temuçin
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引用次数: 0

摘要

导言/目的:多发性神经病、器官肥大、内分泌病、单克隆丙种球蛋白病和皮肤改变(POEMS)综合征是一种由潜在浆细胞异常引起的副肿瘤综合征。POEMS 综合征中的多发性神经病可表现为亚急性或慢性对称性感觉运动性多发性神经病,其电生理特征提示存在脱髓鞘。运动传导阻滞(CB)主要见于慢性炎症性脱髓鞘性多发性神经病(CIDP),被认为是 POEMS 综合征的非典型电生理特征。我们对 POEMS 综合征中运动 CB 的频率进行了研究:本研究纳入了我科数据库中2017年8月至2022年12月期间接受检查的POEMS综合征患者。对所有患者的临床和电生理数据进行回顾性收集和分析:我们提供了 7 名 POEMS 综合征患者的数据。对这些患者进行了 28 次上肢运动神经传导检查,其中 6 名患者的 7 条上肢运动神经(25%)检测到部分 CB。其中一名患者的正中神经和尺神经都出现了运动神经CB:讨论:区分 POEMS 综合征和 CIDP 非常重要,因为这两种疾病需要不同的治疗方法。POEMS 运动性 CB 可能比一般认为的更为常见。临床医生在评估脱髓鞘性多发性神经病患者时应考虑到这一点,并在识别 CB 时做到一丝不苟。我们需要更多患者的数据。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Polyneuropathy With Motor Conduction Block in POEMS.

Introduction/aims: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell dyscrasia. Polyneuropathy in POEMS syndrome may present as a subacute or chronic symmetric sensorimotor polyneuropathy, with electrophysiological features suggesting demyelination. Motor conduction block (CB), which is mostly seen in chronic inflammatory demyelinating polyneuropathy (CIDP), is considered an atypical electrophysiological feature in POEMS syndrome. We examined the frequency of motor CB in POEMS syndrome.

Methods: Patients with POEMS syndrome from the database of our department who had been examined between August 2017 and December 2022 were included in this study. All of the patients' clinical and electrophysiological data were retrospectively collected and analyzed.

Results: We present the data of seven POEMS syndrome patients. Twenty-eight upper extremity motor nerve conduction studies were performed on these patients, and partial CB was detected in seven upper extremity motor nerves (25%) of six of the patients. One patient had motor CB in both the median and ulnar nerves.

Discussion: The distinction between POEMS syndrome and CIDP is important since these conditions require different treatments. Motor CB in POEMS may be more common than has been generally believed. Clinicians should consider this when evaluating patients with demyelinating polyneuropathies and be meticulous in identifying CB. Data from much larger numbers of patients are needed.

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来源期刊
Muscle & Nerve
Muscle & Nerve 医学-临床神经学
CiteScore
6.40
自引率
5.90%
发文量
287
审稿时长
3-6 weeks
期刊介绍: Muscle & Nerve is an international and interdisciplinary publication of original contributions, in both health and disease, concerning studies of the muscle, the neuromuscular junction, the peripheral motor, sensory and autonomic neurons, and the central nervous system where the behavior of the peripheral nervous system is clarified. Appearing monthly, Muscle & Nerve publishes clinical studies and clinically relevant research reports in the fields of anatomy, biochemistry, cell biology, electrophysiology and electrodiagnosis, epidemiology, genetics, immunology, pathology, pharmacology, physiology, toxicology, and virology. The Journal welcomes articles and reports on basic clinical electrophysiology and electrodiagnosis. We expedite some papers dealing with timely topics to keep up with the fast-moving pace of science, based on the referees'' recommendation.
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