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Multifocal motor neuropathy: a review of pathogenesis diagnosis and treatment

机译:多灶性运动神经病:发病机理诊断和治疗的综述

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摘要

Multifocal motor neuropathy (MMN) is an uncommon, purely motor neuropathy associated with asymmetric deficits with predilection for upper limb involvement. Even in the early descriptions of MMN, the associations of anti-GM1 antibodies and robust response to immunomodulatory treatment were recognized. These features highlight the likelihood of an underlying autoimmune etiology of MMN. The clinical presentation of MMN can closely mimic several neurological conditions including those with more malignant prognoses such as motor neuron disease. Therefore early and rapid recognition of MMN is critical. Serological evidence of anti GM-1 antibodies and electrodiagnostic findings of conduction block are helpful diagnostic clues for MMN. Importantly, these diagnostic features are not universally present, and patients lacking these characteristic findings can demonstrate similar robust response to immunodulatory treatment. In the current review, recent research in the areas of diagnosis, pathogenesis, and treatment of MMN and needs for the future are discussed. The characteristic findings of MMN and treatment implications are reviewed and contrasted with other mimicking disorders.
机译:多灶性运动神经病(MMN)是一种罕见的,纯粹的运动神经病,伴有不对称的缺陷伴有上肢受累的倾向。即使在MMN的早期描述中,也认识到抗GM1抗体与免疫调节治疗的强烈应答之间的关联。这些特征突出了MMN潜在的自身免疫病因学的可能性。 MMN的临床表现可以紧密模仿几种神经系统疾病,包括那些具有更恶性预后的疾病,例如运动神经元疾病。因此,尽早识别MMN至关重要。抗GM-1抗体的血清学证据和传导阻滞的电诊断结果是MMN的有用诊断线索。重要的是,这些诊断特征并不是普遍存在的,缺乏这些特征性发现的患者可以表现出对免疫调节治疗的类似强劲反应。在当前的审查中,讨论了MMN的诊断,发病机制和治疗领域的最新研究以及对未来的需求。对MMN的特征性发现及其治疗意义进行了综述,并与其他模仿障碍进行了对比。

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