Diagnostic yield of electromyography in children with myopathic disorders

Partha S. Ghosh, Eric J. Sorenson

Research output: Contribution to journalArticle

14 Citations (Scopus)

Abstract

Background Interpretation of pediatric electromyography interpretation in myopathic disorders is technically challenging. We assessed our electromyographic experience with respect to sensitivity and specificity in pediatric myopathy. Methods We did a retrospective chart review of patients ≤18 years between 2009 and 2013. Two hundred twenty-four electromyographic studies were reviewed with the following referral diagnoses: myopathy, muscle weakness, neuromuscular disorders, myositis, myalgia, myoglobinuria, myasthenia, myotonia, cramps, periodic paralysis, hypotonia, and developmental delay. Only children who had an electromyography and muscle biopsy were included for analysis. Patients with neurogenic electromyography and neuromuscular junction disorders were excluded. Myopathic electromyography was defined as short duration, low amplitude, polyphasic motor unit potentials with rapid recruitment. Results Seventy-two patients were included (age range, 6 months-18 years). The following observations were made: group A: myopathic electromyography or pathognomonic of muscle disease and biopsy or genetically confirmed myopathy (32 cases); group B: myopathic electromyography but biopsy normal or nondiagnostic (12 cases); group C: normal electromyography but biopsy or genetically confirmed myopathy (three cases, all with metabolic myopathy); and group D: electromyography normal and biopsy normal or nondiagnostic (25 cases). The most common diagnoses were congenital myopathy (seven cases), metabolic myopathy (six cases), muscular dystrophy (six cases), genetically confirmed myopathy (five cases), myopathy, undefined (five cases), and inflammatory myopathy (four cases). Conclusions Pediatric electromyography was 91% sensitive and 67% specific in myopathic disorders. The metabolic myopathies were commonly missed by electromyography.

Original languageEnglish (US)
Pages (from-to)215-219
Number of pages5
JournalPediatric Neurology
Volume51
Issue number2
DOIs
StatePublished - 2014

Fingerprint

Electromyography
Muscular Diseases
Biopsy
Myositis
Pediatrics
Neuromuscular Junction Diseases
Myoglobinuria
Myotonia Congenita
Myotonia
Muscle Cramp
Muscles
Muscle Hypotonia
Muscular Dystrophies
Myalgia
Muscle Weakness
Paralysis
Referral and Consultation
Sensitivity and Specificity

Keywords

  • children
  • electromyography
  • muscle biopsy
  • myopathy

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Neurology

Cite this

Diagnostic yield of electromyography in children with myopathic disorders. / Ghosh, Partha S.; Sorenson, Eric J.

In: Pediatric Neurology, Vol. 51, No. 2, 2014, p. 215-219.

Research output: Contribution to journalArticle

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abstract = "Background Interpretation of pediatric electromyography interpretation in myopathic disorders is technically challenging. We assessed our electromyographic experience with respect to sensitivity and specificity in pediatric myopathy. Methods We did a retrospective chart review of patients ≤18 years between 2009 and 2013. Two hundred twenty-four electromyographic studies were reviewed with the following referral diagnoses: myopathy, muscle weakness, neuromuscular disorders, myositis, myalgia, myoglobinuria, myasthenia, myotonia, cramps, periodic paralysis, hypotonia, and developmental delay. Only children who had an electromyography and muscle biopsy were included for analysis. Patients with neurogenic electromyography and neuromuscular junction disorders were excluded. Myopathic electromyography was defined as short duration, low amplitude, polyphasic motor unit potentials with rapid recruitment. Results Seventy-two patients were included (age range, 6 months-18 years). The following observations were made: group A: myopathic electromyography or pathognomonic of muscle disease and biopsy or genetically confirmed myopathy (32 cases); group B: myopathic electromyography but biopsy normal or nondiagnostic (12 cases); group C: normal electromyography but biopsy or genetically confirmed myopathy (three cases, all with metabolic myopathy); and group D: electromyography normal and biopsy normal or nondiagnostic (25 cases). The most common diagnoses were congenital myopathy (seven cases), metabolic myopathy (six cases), muscular dystrophy (six cases), genetically confirmed myopathy (five cases), myopathy, undefined (five cases), and inflammatory myopathy (four cases). Conclusions Pediatric electromyography was 91{\%} sensitive and 67{\%} specific in myopathic disorders. The metabolic myopathies were commonly missed by electromyography.",
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