Diaphragm ‘atonia’ during ictal and post-ictal apnoea: A case report

Objective Ictal and post ictal central apnoea (ICA and PCA) are driven by suppression of brainstem respiratory drive and represent an electroclinical biomarker of sudden unexpected death in epilepsy (SUDEP). While animal models have demonstrated a true ‘atonic diaphragm’ underlying seizure-related apnoea, no human polygraphic study has yet confirmed respiratory muscle ‘atonia’ with direct EMG recording. Methods In this case report, we present a 26-year-old female with meningoencephalocele-associated temporal lobe epilepsy who underwent a video-EEG polygraphy integrated with surface EMG from intercostals muscles and the diaphragm. Results We recorded a left frontotemporal seizure with ICA and four consecutives PCA events, all accompanied by surface electromyographic silence of the diaphragm and the intercostal muscles. Conclusions This case provide a human polygraphic evidence of diaphragm and intercostal muscles silence during ICA and PCA, confirming the cascade of respiratory inhibition through brainstem respiratory centres down to respiratory muscles themselves. Notably, muscles relaxation occurred despite the patient was awake and able to follow commands. Significance These findings support the importance of cardiorespiratory polygraphy during presurgical video-EEG evaluation, especially for temporal lobe seizures, to identify patients at higher SUDEP risk.