Update on Pediatric NMJ Disorders-Dr. Verma Presentation

Dr. Verma Presentation

Pdf Summary

The document is a comprehensive overview focused on neurophysiological testing in children with neuromuscular junction (NMJ) disorders, presented by Dr. Sumit Verma. It emphasizes techniques like Stimulated Jitter Analysis (Stim-JA) and Repetitive Nerve Stimulation (RNS), which assist in diagnosing NMJ disorders. The learning objectives highlight understanding the methodology, clinical applications, and means to interpret results effectively.<br /><br /><strong>Stimulated Jitter Analysis (Stim-JA):</strong> This technique uses single fiber electromyography with concentric needle electrodes to record action potentials from different motor units. It examines the time variability between these potentials, known as jitter, to diagnose NMJ abnormalities. Such analysis aids in discerning blocking and identifying neuromuscular transmission failure.<br /><br /><strong>Repetitive Nerve Stimulation (RNS):</strong> RNS assesses neuromuscular transmission by observing compound muscle action potentials. The document outlines RNS's use in diagnosing slow-channel congenital myasthenic syndromes (CMS) or acetylcholinesterase deficiency.<br /><br /><strong>Case Studies:</strong> The document also details two pediatric case studies involving infant botulism, highlighting EMG findings like increased jitter and blocking, and their successful diagnosis and treatment with BabyBIG.<br /><br /><strong>Tips for Pediatric Electromyography:</strong> Emphasizing the importance of quality recording, the text provides practical advice for recognizing abnormalities and managing the patient experience, ensuring procedural comfort and accuracy, especially in young patients.<br /><br /><strong>Parental Involvement:</strong> The report outlines strategies for involving and reassuring parents during testing, recognizing the importance of their support in the EMG lab.<br /><br /><strong>Conclusion:</strong> The document points out the diagnostic value of neurophysiological tests like Stim-JA as biomarkers for NMJ disorders and encourages medical professionals to provide feedback through the AANEM app, which is integral for improving educational offerings in this field.

Keywords

neurophysiological testing

neuromuscular junction disorders

Stimulated Jitter Analysis

Repetitive Nerve Stimulation

Dr. Sumit Verma

pediatric case studies

electromyography tips

parental involvement

diagnostic value

AANEM app