神经性肌萎缩症:评估、诊断和治疗方法的最新进展。

IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY
Muscle & Nerve Pub Date : 2024-10-14 DOI:10.1002/mus.28274
Joelle M Gabet, Noriko Anderson, Jan T Groothuis, Evan R Zeldin, John W Norbury, Andrew S Jack, Line Jacques, Darryl B Sneag, Ann Poncelet
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引用次数: 0

摘要

神经性肌萎缩症(NA)是一种未得到充分认识的周围神经疾病,表现为剧烈疼痛,随后一条或多条神经分布无力,最常见于上肢。虽然传统上认为该病的预后良好,但最新的研究表明,患者常常被延误诊断,甚至在确诊后数年仍会经历长期的疼痛、瘫痪和疲劳。本综述建议并描述了治疗方法的转变,强调必须针对代偿性异常运动控制和疲劳,重点关注运动协调、节能策略和行为改变,而不是可能会加重症状的力量训练。影像学检查中出现结构性沙漏样收缩(HGCs)有助于确诊疑似临床诊断,如果伴有持续性乏力和电诊断测试恢复受限,可考虑进行手术会诊。鉴于治疗的复杂性,本文介绍了一种多学科方法,该方法可为NA持续症状患者提供最佳护理和支持,并为康复和手术转诊提供更统一的指导。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Neuralgic amyotrophy: An update in evaluation, diagnosis, and treatment approaches.

Neuralgic amyotrophy (NA) is an underrecognized peripheral nerve disorder distinguished by severe pain followed by weakness in the distribution of one or more nerves, most commonly in the upper extremity. While classically felt to carry a favorable prognosis, updates in research have demonstrated that patients frequently endure delay in diagnosis and continue to experience long term pain, paresis, and fatigue even years after the diagnosis is made. A transition in therapeutic approach is recommended and described by this review, which emphasizes the necessity to target compensatory abnormal motor control and fatigue by focusing on motor coordination, energy conservation strategies, and behavioral change, rather than strength training which may worsen the symptoms. The development of structural hourglass-like constrictions (HGCs) on imaging can help confirm the suspected clinical diagnosis, and in association with persistent weakness and limited recovery on electrodiagnostic testing may be considered for surgical consultation. Given the complex nature of management, a multidisciplinary approach is described, which can provide an optimal level of care and support for patients with persistent symptoms from NA and allow more unified guidance of rehabilitation and surgical referrals.

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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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