Zimmermann Maximilian, Wollsching-Strobel Maximilian, Majorski Daniel Sebastian, Kroppen Doreen, Schwarz Sarah Bettina, Berger Melanie, Windisch Wolfram, Holle Johannes Fabian
Pneumologie, Kliniken der Stadt Köln gGmbH, Köln, Deutschland.
Lehrstuhl für Pneumologie, Universität Witten/Herdecke Fakultät für Gesundheit, Köln, Deutschland.
Pneumologie. 2023 Oct;77(10):814-824. doi: 10.1055/a-2113-0385. Epub 2023 Aug 30.
There are several causes for unilateral or bilateral diaphragmatic paresis. The most common cause is an (intraoperative) injury to the phrenic nerve.However, in up to 20% of cases, no explanation can be found despite extensive workup. Neuralgic amyotrophy (NA, also known as Parsonage-Turner syndrome) is a common underdiagnosed multifocal autoimmune-inflammatory disease that predominantly affects proximal nerve segments of the upper extremities. Classic symptoms include acute onset of severe pain in the shoulder girdle with delayed onset of paresis of the shoulder and arm muscles. In at least 7% of cases, the phrenic nerve is also affected. Based on the annual incidence of NA of 1:1000, the entity as a cause of diaphragmatic dysfunction is probably not as uncommon as previously thought. However, clinical experience shows that this diagnosis is often not considered, and diaphragmatic paresis gets wrongly classified as idiopathic.This is particularly disastrous because in the early stage of NA, medical therapy with corticosteroids is mostly not considered and the possibility that surgical repair of the diaphragm may be performed prematurely, given that the condition may resolve spontaneously many months after symptom onset.The aim of the present article is to raise awareness of the entity of NA as a cause of diaphragmatic paresis and to establish a standardized approach to diagnosis and treatment.
单侧或双侧膈肌麻痹有多种病因。最常见的病因是(术中)膈神经损伤。然而,在高达20%的病例中,尽管进行了广泛检查,仍无法找到病因。神经性肌萎缩(NA,也称为Parsonage-Turner综合征)是一种常见的未被充分诊断的多灶性自身免疫性炎症性疾病,主要影响上肢近端神经节段。典型症状包括肩胛带突发剧痛,随后肩部和手臂肌肉麻痹延迟出现。至少7%的病例中,膈神经也会受到影响。基于NA的年发病率为1:1000,该疾病作为膈肌功能障碍的病因可能不像之前认为的那么罕见。然而,临床经验表明,这种诊断往往未被考虑,膈肌麻痹常被错误归类为特发性。这尤其具有灾难性,因为在NA的早期阶段,大多不会考虑使用皮质类固醇进行药物治疗,而且鉴于症状出现数月后病情可能会自行缓解,可能会过早地进行膈肌手术修复。本文的目的是提高对NA作为膈肌麻痹病因的认识,并建立标准化的诊断和治疗方法。