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神经性肌萎缩:诊断、病理生理学及治疗的最新进展

Neuralgic amyotrophy: An update on diagnosis, pathophysiology, and treatment.

作者信息

Van Eijk Jeroen J J, Groothuis Jan T, Van Alfen Nens

机构信息

Department of Neurology, Jeroen Bosch Hospital, 's-Hertogenbosch, The Netherlands.

Department of Neurology and Clinical Neurophysiology, Donders Centre for Neuroscience, Radboud University Medical Center, Nijmegen, The Netherlands.

出版信息

Muscle Nerve. 2016 Mar;53(3):337-50. doi: 10.1002/mus.25008. Epub 2016 Jan 20.

Abstract

In this review we provide a current overview of the clinical features, pathophysiology, epidemiology, and diagnostic and therapeutic strategies in neuralgic amyotrophy (NA). The disorder has several phenotypic variations, with a classic form in 70% of the patients. It is not rare, with an incidence of 1 per 1,000 individuals, but it is still often missed. Recurrences are common, yet the proposed multifactorial etiology, which includes genetic, biomechanical, and immunologic factors, limits our capacity to predict or prevent them. NA is a clinical diagnosis, and ancillary studies serve to exclude infectious or malignant causes or to assess a differential diagnosis. If patients are seen early and are still in pain, a short trial of high-dose oral corticosteroids is advised, and adequate analgesia may require opioids and non-steroidal anti-inflammatory drugs. Persistent complaints are common, and a multidisciplinary rehabilitation approach focusing on scapular coordination, energy distribution strategies, and self-management is indicated.

摘要

在本综述中,我们对神经性肌萎缩(NA)的临床特征、病理生理学、流行病学以及诊断和治疗策略进行了当前概述。该疾病有多种表型变异,70%的患者为经典型。它并不罕见,发病率为每1000人中有1例,但仍常被漏诊。复发很常见,然而所提出的包括遗传、生物力学和免疫因素在内的多因素病因限制了我们预测或预防复发的能力。NA是一种临床诊断,辅助检查用于排除感染性或恶性病因或评估鉴别诊断。如果患者就诊较早且仍有疼痛,建议短期试用高剂量口服皮质类固醇,充分的镇痛可能需要使用阿片类药物和非甾体抗炎药。持续存在的症状很常见,需要采用多学科康复方法,重点关注肩胛协调、能量分配策略和自我管理。

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