Electrodiagnostic characteristics of neuromuscular disease in paediatric intensive care.
PMID 40273615 | DOI 10.1016/j.clinph.2025.04.005 · Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology · 2025
OBJECTIVE: Assessing peripheral electrodiagnostic (EDX) tests in paediatric intensive care. METHODS: Data from patients who had undergone EDX test/s between 2010 and 2019 at a tertiary centre were retrospectively analysed, including final neuromuscular diagnoses, EDX results and demographic information. EDX data included motor and sensory nerve conduction study, needle electromyography (EMG), repetitive nerve stimulation and stimulated single fiber EMG. Final clinical diagnosis was based on several investigations including muscle biopsy, MR imaging, gene testing, EDX-tests and clinical phenotype. RESULTS: 351 patients were identified (56 % male, average age 42.5 months), with diagnoses categorised into the following groups: no identifiable neuromuscular disorders (45 %), neuropathy (13 %), motor neuron disease (9 %), isolated bulbar palsy (6 %), myopathy (14 %), neuromuscular junction disorders (5 %), and critical illness neuromyopathy (8 %). EDX data was stratified into 7 electrodiagnostic categories: normal, neuropathy, motor neuron disease, isolated bulbar palsy, myopathy, neuromuscular junction disorders, and critical illness neuromyopathy. With this stratification we were able to predict the final diagnosis with acceptable accuracy. CONCLUSION: The prevalence of neuromuscular disease groups in paediatric ICU was defined together with their corresponding EDX characteristics. SIGNIFICANCE: The study confirms the utility of electrophysiology as a valuable tool for diagnosing and managing neuromuscular conditions in paediatric ICU.
Validated evidence
| Type | Entity | Source evidence | Confidence | Extractor |
|---|---|---|---|---|
| phenotype | congenital myopathy | “RESULTS: 351 patients were identified (56 % male, average age 42.5 months), with diagnoses categorised into the following groups: no identifiable neuromuscular disorders (45 %), neuropathy (13 %), motor neuron disease (9 %), isolated bulbar palsy (6 %), myopathy (14 %), neuromuscular junction disorders (5 %), and critical illness neuromyopathy (8 %).” | 0.93 | phenotype_alias_lexicon_v2 |
| population | Population | “RESULTS: 351 patients were identified (56 % male, average age 42.5 months), with diagnoses categorised into the following groups: no identifiable neuromuscular disorders (45 %), neuropathy (13 %), motor neuron disease (9 %), isolated bulbar palsy (6 %), myopathy (14 %), neuromuscular junction disorders (5 %), and critical illness neuromyopathy (8 %).” | 0.80 | saudi_context_rules_v1 |