Rotondo Eleonora, Pellegrino Noemi, Di Battista Caterina, Graziosi Alessandro, Di Stefano Vincenzo, Striano Pasquale
Department of Paediatrics, "G. d'Annunzio" University, Chieti, Italy.
Department of Neuroscience, Imaging and Clinical Sciences, "G. d'Annunzio" University, Via dei Vestini, 66013, Chieti, Italy.
Neurol Sci. 2020 Jul;41(7):1735-1740. doi: 10.1007/s10072-020-04314-8. Epub 2020 Mar 5.
Neuralgic amyotrophy (NA), even known as Personage-Turner's syndrome (PTS), is a neurologic condition, affecting the lower motor neurons of brachial plexus and/or individual nerves or nerve branches, characterized by pain, muscle weakness/atrophy, and sensory symptoms. NA has an acute/subacute onset, after an infection or vaccination; it is more common in male and is rare in the pediatric population. The etiology remains uncertain, being considered heterogeneous and multifactorial. A severe acute neurologic pain around the shoulder girdle is the classic presenting symptom at onset. As the pain subsides, weakness and paresis develop. NA is usually unilateral, but sometimes, a subclinical contralateral limb involvement could be present and bilateral affection has been described. The diagnosis is clinical, through a comprehensive history and neurological examination. However, electrophysiological testing and imaging are critical, because there is no diagnostic test for PTS and it remains a diagnosis of exclusion. Upper brachial plexus peripheral involvement with weakness of periscapular and perihumeral muscles is the classic presentation, associated with electrophysiological evidence of denervation in the affected muscles. Imaging, laboratory, and genetic testing can be useful for the differential diagnosis. NA is in most cases a self-limiting condition, and it is characterized by good recovery. Treatment of NA usually involves a combination of corticosteroids, analgesics, immobilization, and physical therapy, even if limited data are available in children. Physiotherapy is required to maintain muscle strength.
神经性肌萎缩（NA），又称佩尔索纳热特纳综合征（PTS），是一种神经系统疾病，影响臂丛神经的下运动神经元和/或个别神经或神经分支，其特征为疼痛、肌肉无力/萎缩以及感觉症状。NA在感染或接种疫苗后急性/亚急性起病；男性更为常见，在儿科人群中较为罕见。病因仍不确定，被认为具有异质性和多因素性。肩带周围严重的急性神经性疼痛是起病时的典型症状。随着疼痛消退，出现无力和轻瘫。NA通常为单侧，但有时可能存在亚临床对侧肢体受累情况，也有双侧受累的报道。诊断依靠临床，通过全面的病史和神经系统检查。然而，电生理检查和影像学检查至关重要，因为目前尚无针对PTS的诊断性检查，它仍然是一种排除性诊断。上臂丛神经外周受累伴肩胛周和肱骨周围肌肉无力是典型表现，伴有受累肌肉失神经支配的电生理证据。影像学、实验室和基因检测对鉴别诊断可能有用。NA在大多数情况下是一种自限性疾病，其特点是恢复良好。NA的治疗通常包括皮质类固醇、镇痛药、固定和物理治疗的联合应用，尽管儿童相关数据有限。需要进行物理治疗以维持肌肉力量。
