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Muscle Nerve. 2017 Sep;56(3):427-432. doi: 10.1002/mus.25521. Epub 2017 Jun 17.

Abnormal spontaneous activity in primary myopathic disorders.

Author information

1
Department of Neurology, Medical University of Warsaw, Warsaw, Poland.
2
Department of Biochemistry and Molecular Biology, Centre of Postgraduate Medical Education, Marymoncka Strasse 99/103, 01-813, Warsaw, Poland.

Abstract

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. Muscle Nerve 56: 427-432, 2017.

KEYWORDS:

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

PMID:
28000226
DOI:
10.1002/mus.25521
[Indexed for MEDLINE]

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