Self-Assessment: Management of CMT Disease in Children & Adolescents | Pediatric EMG Pearls-Session recording- Pediatric EMG Pearls

Video Transcription

Video Summary

This comprehensive pediatric neurology session focused on setting up and running pediatric EMG labs, interpreting pediatric electrodiagnostic studies, and integrating EMG with genetic testing and novel therapies. Dr. Isaac Kanju discussed the challenges of performing EMG in children, emphasizing that pediatric EMG is more art than protocol-driven medicine and requires individualized approaches, often under sedation. She covered technical aspects, such as electrode size, stabilization, sedation considerations, and developmental maturation effects on nerve conduction velocities and amplitudes. Key uses of pediatric EMG include diagnosing disorders like CIDP, Guillain-Barré syndrome, neuromuscular junction disorders (e.g., myasthenia, botulism), and certain myopathies and neuropathies. Dr. Alex Fayez presented detailed neonatal cases illustrating the value of EMG alongside genetic testing. Despite rapid genomic diagnostics, electrodiagnostics remain crucial for localization, diagnosis confirmation, and guiding treatment for neonatal neuromuscular disorders, including immune, genetic, and mitochondrial neuropathies, congenital myopathies, SCN4A channelopathies, and congenital myasthenic syndromes. He emphasized careful interpretation of sensory responses and maturational norms in neonates. Dr. Sumit Verma highlighted the evolving role of EMG in the era of newborn screening and targeted therapies for conditions like spinal muscular atrophy (SMA) and Duchenne muscular dystrophy. He advocated using serial EMG and advanced techniques such as Motor Unit Number Index (MUNIX), stimulated jitter analysis, and muscle ultrasound to provide objective biomarkers to guide therapy decisions, monitor disease progression, and support clinical trials. The session concluded with practical tips on sedation, EMG techniques, and the importance of electrophysiology alongside genetic and clinical data, underscoring the indispensable role of pediatric EMG in diagnosis, treatment guidance, and multidisciplinary care of neuromuscular disorders in children.

Keywords

pediatric neurology

pediatric EMG labs

electrodiagnostic studies

EMG and genetic testing

novel therapies

EMG in children

sedation in pediatric EMG

nerve conduction velocities

CIDP diagnosis

Guillain-Barré syndrome

neuromuscular junction disorders

neonatal neuromuscular disorders

Motor Unit Number Index (MUNIX)

muscle ultrasound