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神经性肌萎缩:评估、诊断及治疗方法的最新进展

Neuralgic amyotrophy: An update in evaluation, diagnosis, and treatment approaches.

作者信息

Gabet Joelle M, Anderson Noriko, Groothuis Jan T, Zeldin Evan R, Norbury John W, Jack Andrew S, Jacques Line, Sneag Darryl B, Poncelet Ann

机构信息

Department of Physical Medicine and Rehabilitation, MetroHealth Rehabilitation Institute, The MetroHealth System and Case Western Reserve University, Cleveland, Ohio, USA.

Department of Neurology, University of California San Francisco Medical Center and Weill Institute for Neurosciences, San Francisco, California, USA.

出版信息

Muscle Nerve. 2025 May;71(5):846-856. doi: 10.1002/mus.28274. Epub 2024 Oct 14.

DOI:10.1002/mus.28274
PMID:39402917
Abstract

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.

摘要

神经性肌萎缩(NA)是一种未得到充分认识的周围神经疾病,其特征是严重疼痛,随后在一条或多条神经分布区域出现无力,最常见于上肢。虽然传统上认为其预后良好,但研究进展表明,患者常常诊断延迟,即使在确诊多年后仍持续遭受长期疼痛、轻瘫和疲劳。本综述建议并描述了治疗方法的转变,强调有必要通过关注运动协调、能量节约策略和行为改变来针对代偿性异常运动控制和疲劳,而不是进行可能会加重症状的力量训练。影像学上出现结构类似沙漏样的缩窄(HGCs)有助于确诊疑似临床诊断,并且结合电诊断测试中持续存在的无力和恢复有限的情况,可考虑进行手术咨询。鉴于管理的复杂性,本文描述了一种多学科方法,可为患有持续性NA症状的患者提供最佳水平的护理和支持,并为康复和手术转诊提供更统一的指导。

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