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伴有颅神经受累及声带麻痹的多灶性运动神经病:一例报告

Multifocal Motor Neuropathy With Cranial Nerve Involvement and Vocal Cord Paralysis: A Case Report.

作者信息

Nunez Maria Clarissa, Nepomuceno Belinda Lioba M, Tiongson Ma Luisa Gwenn P

机构信息

Department of Clinical Neurosciences, University of the East Ramon Magsaysay Memorial Medical Center, Quezon City, PHL.

Department of Neuroscience, University of the East Ramon Magsaysay Memorial Medical Center, Quezon City, PHL.

出版信息

Cureus. 2022 May 21;14(5):e25179. doi: 10.7759/cureus.25179. eCollection 2022 May.

DOI:10.7759/cureus.25179
PMID:35746993
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9207705/
Abstract

Multifocal motor neuropathy (MMN) is a progressive, multifocal weakness, which typically begins and predominates in the upper extremities with the absence of a sensory deficit and a hallmark electrophysiologic finding of conduction block. We describe a case of an adult male with MMN who developed both cranial nerve involvement and vocal cord paralysis. The patient presented with left shoulder weakness without sensory loss followed by hoarseness of voice and later developed tongue deviation and wasting of the left sternocleidomastoid and left trapezius muscle. Laryngeal electromyography (EMG) showed findings evident for a focal mononeuropathy involving the left recurrent laryngeal nerve. EMG and nerve conduction studies (EMG NCV) of the upper extremities showed evidence for a multifocal mainly motor neuropathy involving the left spinal accessory and hypoglossal nerves, combined with the presence of median and ulnar proximal conduction blocks bilaterally. Given the clinical presentation and electrophysiologic findings of conduction block, the patient was managed as a case of MMN and received the standard treatment with Intravenous Immunoglobulin (IVIg). Upon follow-up, there was an improvement in symptoms and no recurrence of motor weakness and hoarseness of voice. There are a few case reports about MMN but none with multiple lower cranial nerve involvement.

摘要

多灶性运动神经病(MMN)是一种进行性多灶性肌无力疾病,通常始于上肢并以上肢为主,无感觉障碍,且具有传导阻滞这一典型的电生理表现。我们报告一例成年男性MMN患者,该患者出现了颅神经受累及声带麻痹。患者最初表现为左肩无力且无感觉丧失,随后出现声音嘶哑,之后发展为舌偏斜以及左侧胸锁乳突肌和左侧斜方肌萎缩。喉肌电图(EMG)显示存在累及左侧喉返神经的局灶性单神经病证据。上肢肌电图和神经传导研究(EMG NCV)显示存在累及左侧副神经和舌下神经的多灶性主要为运动性神经病证据,同时双侧正中神经和尺神经近端存在传导阻滞。鉴于临床表现及传导阻滞的电生理表现,该患者被诊断为MMN并接受了静脉注射免疫球蛋白(IVIg)的标准治疗。随访时,症状有所改善,运动无力和声音嘶哑未复发。关于MMN有一些病例报告,但均未涉及多条低位颅神经受累情况。

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