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Abnormal spontaneous activity in primary myopathic disorders
Author(s) -
Nojszewska Monika,
Gawel Malgorzata,
SzmidtSalkowska Elzbieta,
KosteraPruszczyk Anna,
PotulskaChromik Anna,
Lusakowska Anna,
Kierdaszuk Biruta,
Lipowska Marta,
Macias Anna,
Gawel Damian,
Seroka Andrzej,
Kaminska Anna M.
Publication year - 2017
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.25521
Subject(s) - facioscapulohumeral muscular dystrophy , myopathy , medicine , abnormality , muscular dystrophy , myotonic dystrophy , electromyography , limb girdle muscular dystrophy , pathology , physical medicine and rehabilitation , mutation , genetics , psychiatry , biology , gene
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. Muscle Nerve 56 : 427–432, 2017