多灶性运动神经病传导阻滞延迟表现1例报告

Q4 Medicine US endocrinology Pub Date : 2017-01-01 DOI:10.17925/USE.2017.13.02.99
L. Darki, S. Beydoun
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引用次数: 0

摘要

多灶性运动神经病(MMN)是一种罕见的、可治疗的、免疫介导的神经病变,常伴有多灶性传导阻滞(CB)。标志性的电诊断特征是在非夹闭部位出现CB。然而,没有CB的MMN也被描述过,即使在没有CB的情况下也可以被诊断出来。因此,诊断和识别没有结核杆菌的MMN病例是至关重要的,因为结核杆菌是一种可治疗的疾病。病例介绍:我们提出一个病例进行性症状不对称远端上肢和下肢无力,没有感觉缺陷。静脉注射免疫球蛋白(IVIG)治疗开始,因为患者满足可能MMN的标准,尽管没有CB。患者对IVIG的反应表现出相对平稳期。尽管患者失去了随访,但在首次就诊后11年进行的反复电诊断研究显示,在神经节段中出现了新的CB,而以前没有任何CB的证据。结论:本病例强调MMN早期诊断和早期分别进行IVIG治疗的重要性,以维持临床功能。对临床疑似MMN的诊断不足,基于缺乏CB,将导致拒绝对潜在的IVIG应答者进行治疗。
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Delayed Appearance of Conduction Block in Multifocal Motor Neuropathy—A Case Report
I ntroduction: Multifocal motor neuropathy (MMN) is a rare, treatable, immune-mediated neuropathy often associated with multifocal conduction block (CB). The hallmark electrodiagnostic feature is the presence of CB occurring at non-entrapment sites. However, MMN without CB has also been described and can be diagnosed, even in the absence of CB. Therefore, it is crucial to diagnose and identify MMN cases without CB, as it is a treatable disorder. Case presentation: We present a case with progressive symptoms of asymmetric distal upper and lower extremity weakness with no sensory deficits. Intravenous immunoglobulin (IVIG) therapy was initiated, as the patient fulfilled the criteria for probable MMN, despite the absence of CB. The patient’s symptoms demonstrated a relative plateau phase in response to IVIG. Although the patient lost follow-up visits, repeated electrodiagnostic study, conducted 11 years after initial presentation, revealed new CB in nerve segments that previously did not show any evidence of CB. Conclusion: This case emphasizes the importance of early diagnosis and respectively initiating early IVIG treatment in MMN, in order to maintain the clinical function. Underdiagnosis of clinically suspected MMN, based on absence of CB, will result in denial of treatment to potential IVIG responders.
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来源期刊
US endocrinology
US endocrinology Medicine-Endocrinology, Diabetes and Metabolism
CiteScore
1.90
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