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Determining jitter values in the very young by use of the e-norms methodology
1Department of Clinical Neurophysiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N 3JH, UK.
Insights
Diagnosing myasthenia gravis in infants is difficult. This study derived reference values for jitter mean consecutive difference (MCD) using the extrapolated norms (e-norms) method, providing crucial diagnostic data for young children.
Area of Science:
- Neurology
- Pediatric Electromyography
Background:
- Diagnosing myasthenia gravis in infants presents significant challenges.
- Stimulated single-fiber electromyography (StimSFEMG) is valuable but lacks reference values for infants.
- This necessitates the development of normative data for pediatric populations.
Purpose of the Study:
- To derive a reference range for jitter mean consecutive difference (MCD) in infants under 3 years of age.
- To establish normative data for StimSFEMG in a pediatric cohort.
- To address the diagnostic limitations in very young infants with suspected myasthenia gravis.
Main Methods:
- Utilized the extrapolated norms (e-norms) technique to analyze data.
- Calculated jitter MCD descriptive statistics for children under 3 years old.
- Applied established statistical methods to derive reference values from existing cohorts.
Main Results:
- Established upper limits for jitter MCD: 45 μs (<1 year), 33 μs (<2 years), and 26 μs (<3 years).
- Provided specific reference ranges tailored to different age groups within the first three years of life.
- Demonstrated the feasibility of the e-norms method for generating pediatric reference data.
Conclusions:
- The e-norms method offers a viable alternative for deriving jitter reference values in infants.
- These derived values can aid in the diagnosis of neuromuscular disorders in young children.
- Addresses a critical gap in diagnostic tools for pediatric myasthenia gravis.
Introduction:
The diagnosis of myasthenia gravis in very young infants is a challenging one. In young infants, stimulated single-fiber electromyography (StimSFEMG) is the most appropriate technique, but it has serious limitations due to the absence of reference values in this subpopulation. Here we present our efforts to derive a reference range of jitter in a patient cohort of infants <3 years of age using the extrapolated norms, or e-norms, technique.
Methods:
The e-norms method was used to calculate jitter mean consecutive difference (MCD) descriptive statistics for children <3 years of age.
Results:
The e-norms derived jitter upper MCD limit was 45 μs in children <1 year, 33 μs in those <2 years, and 26 in those <3 years of age.
Conclusion:
In the absence of jitter reference values for the very young, the e-norms method can be used as an alternative to derive these values from laboratory cohorts. Muscle Nerve 55: 51-54, 2017.
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