Clinico-diagnostic features of neuralgic amyotrophy in childhood
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Clinico-diagnostic features of neuralgic amyotrophy in childhood Eleonora Rotondo 1 & Noemi Pellegrino 1 & Caterina Di Battista 1 & Alessandro Graziosi 1 & Vincenzo Di Stefano 2 & Pasquale Striano 3,4 Received: 4 November 2019 / Accepted: 21 February 2020 # Fondazione Società Italiana di Neurologia 2020
Abstract 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. Keywords Neuralgic amyotrophy . Pediatric . Neuralgic pain . Electromyography . Personage-Turner’s syndrome
Definition and epidemiology Neuralgic amyotrophy (NA) was first described by Feinberg in 1897 and is characterized by the clinical triad of pain, muscle weakness/atrophy, and sensory symptoms [1]. NA affects the lower motor neurons of brachial plexus and/or individual nerves or nerve branches. The onset of symptoms is usually explosive, and infections and vaccinations may occur in children before the NA occurs [2]. In 1948, Personage and Turner
* Vincenzo Di Stefano [email protected] 1
Department of Paediatrics, “G. d’Annunzio” University, Chieti, Italy
2
Department of Neuroscience, Imaging and Clinical Sciences, “G. d’Annunzio” University, Via dei Vestini, 66013 Chieti, Italy
3
Paediatric Neurology and Muscular Diseases Unit, “G. Gaslini” Institute, Genoa, Italy
4
Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, Genoa, Italy
described 136 subjects with brachial plexitis [3]. The authors described
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