Abstract
We retrospectively reviewed 2030 childhood electromyograms performed over an 11-year period (2004-2014). Twenty children (1%) with myotonic discharges were identified and placed into 2 groups. Group A (electrical and clinical myotonia) comprised 9 children (8 with myotonia congenita and 1 with paramyotonia congenita); all of them had diffuse myotonic discharges without clinical weakness or elevated creatine kinase. Group B (electrical myotonia without clinical myotonia) comprised 11 children (4 with inflammatory myopathy; 3, congenital myopathy, 3, muscular dystrophy; and 1, congenital muscular dystrophy). Clinical weakness was demonstrated in all of them and elevated creatine kinase in 6; all had a myopathic electromyogram and scattered myotonic discharges. We conclude that myotonic discharges are a rare but characteristic spontaneous discharge identified during electrodiagnostic studies in children. The presence of electrical and clinical myotonia provides helpful clues to differentiate between various muscle disorders in children.
Original language | English (US) |
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Pages (from-to) | 1300-1306 |
Number of pages | 7 |
Journal | Journal of child neurology |
Volume | 30 |
Issue number | 10 |
DOIs | |
State | Published - Sep 18 2015 |
Keywords
- children
- electromyography
- muscle disorders
- myotonia
- myotonic discharges
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Clinical Neurology