Holle Johannes Fabian, Limmroth Volker, Windisch Wolfram, Zimmerman Maximilian
Department of Neurology, Cologne-Merheim, Hospitals of the City of Cologne, Cologne; Cologne-Merheim Lung Clinic, Hospitals of the City of Cologne, Cologne; Health Faculty/Department for Human Medicine, University of Witten/Herdecke.
Dtsch Arztebl Int. 2024 Jul 26;121(15):483-489. doi: 10.3238/arztebl.m2024.0077.
Neuralgic amyotrophy (NA) is a multifactorial, monophasic neuritis that mainly affects the nerves of the shoulder girdle. It is characterized by very severe pain and by weakness that arises some time after the pain. Its reported incidence is high (100 cases per 100 000 persons per year), but our data suggest that many or most cases are diagnosed late or not at all.
This review of the epidemiology, pathophysiology, diagnosis, and treatment of NA is based on pertinent publications retrieved by a selective literature search, and on data provided by the scientific institute of AOK, a German statutory health-insurance carrier.
It is currently thought that the combination of a genetic predisposition, an immunological trigger factor, and mechanical stress on the affected nerve segment(s) is pathophysiologically determinative. The prognosis of untreated NA is poor, with 25% of patients remaining unable to work at three years. The main form of treatment is with corticosteroids that are administered as early as possible. If there is evidence of nerve constriction or torsion, surgery may also help. There have only been six controlled cohort studies on the treatment of NA, and no randomized trials. It is not uncommon for the acute phase to develop into a chronic pain syndrome requiring multidimensional treatment.
Particularly in view of the high incidence and improved therapeutic options, NA should be included in the differential diagnosis of all patients with suggestive symptoms.
神经性肌萎缩（NA）是一种多因素的单相神经炎，主要影响肩胛带神经。其特征是疼痛非常剧烈，且疼痛一段时间后会出现无力症状。据报道，其发病率较高（每年每10万人中有100例），但我们的数据表明，许多或大多数病例诊断较晚或根本未被诊断。
本对NA的流行病学、病理生理学、诊断和治疗的综述基于通过选择性文献检索获得的相关出版物，以及德国法定健康保险公司AOK科学研究所提供的数据。
目前认为，遗传易感性、免疫触发因素以及受影响神经节段的机械应力共同作用在病理生理上起决定性作用。未经治疗的NA预后较差，25%的患者在三年后仍无法工作。主要治疗方式是尽早使用皮质类固醇。如果有神经受压或扭转的证据，手术可能也有帮助。关于NA治疗的对照队列研究仅有六项，尚无随机试验。急性期发展为需要多维治疗的慢性疼痛综合征的情况并不少见。
特别是鉴于其高发病率和治疗选择的改善，NA应纳入所有有提示性症状患者的鉴别诊断中。
