PDF(Pubmed)
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，多灶性弱点，通常在上肢开始并占主导地位，没有感觉缺陷和传导阻滞的标志性电生理发现。我们描述了一例患有MMN的成年男性，他同时出现了颅神经受累和声带麻痹。患者表现为左肩无力，无感觉丧失，随后声音嘶哑，后来出现舌头偏斜和左胸锁乳突和左斜方肌消瘦。喉肌电图（EMG）显示了累及左喉返神经的局灶性单神经病。上肢的EMG和神经传导研究（EMGNCV）显示了多灶性主要运动神经病的证据，涉及左脊柱附件和舌下神经。结合双侧正中和尺骨近端传导阻滞的存在。鉴于传导阻滞的临床表现和电生理发现，该患者作为MMN病例进行治疗,并接受静脉免疫球蛋白(IVIg)标准治疗.在后续行动中，症状有所改善，没有运动无力和声音嘶哑的复发。有一些关于MMN的病例报告，但没有多个下颅神经受累。
