ABNORMAL SPONTANEOUS ACTIVITY IN PRIMARY MYOPATHIC DISORDERS

作者：Nojszewska Monika; Gawel Malgorzata^*; Szmidt Salkowska Elzbieta; Kostera Pruszczyk Anna; Potulska Chromik Anna; Lusakowska Anna; Kierdaszuk Biruta; Lipowska Marta; Macias Anna; Gawel Damian; Seroka Andrzej; Kaminska Anna M

来源：Muscle & Nerve, 2017, 56(3): 427-432.

DOI：10.1002/mus.25521

• CRD
• electromyography
• fibrillation potentials
• hereditary muscle diseases
• PSW

Introduction: Reproducible non-insertional spontaneous activity (SA), with the exception of endplate activity, is an unequivocal sign of abnormality and is one of the most useful findings obtained on electromyography. Methods: In this retrospective study we analyzed occurrence and distribution of abnormal SA in 151 patients with genetically confirmed myopathies. Results: Complex repetitive discharges (CRDs) occurred more frequently than fibrillation potentials (fibs) and positive sharp waves (PSWs) in centronuclear myopathy (CNM) and limb-girdle muscular dystrophy type 2A (LGMD-2A), whereas fibs/PSWs were observed more often in desminopathy and facioscapulohumeral dystrophy (FSHD). Abnormal SA was commonly found in CNM (66.7%) and desminopathy (61.5%), occasionally in Duchenne (DMD) and Becker muscular dystrophy (BMD) (45.2% and 27.6%, respectively), but rarely in FSHD (14.9%) and LGMD-2A (12.0%). Conclusions: Abnormal SA probably occurs more frequently in disorders associated with structural changes in muscle fibers. Screening for SA may be a valuable tool for diagnosis of non-myotonic myopathies.

• 出版日期2017-9