Introduction

Epilepsy with myoclonic-atonic seizures (EMAtS) (Doose syndrome) is a rare developmental and epileptic encephalopathy. Seizures remit in 60% but others enter a Stormy Phase (SP) of worsening seizures and recurrent non-convulsive status epilepticus. Our study aimed to distinguish the functional connectivity patterns among electroencephalographs (EEGs) of children diagnosed with a) SP, b) Lennox Gastaut Syndrome (LGS), c) non-SP EMAtS and d) normal EEGs in children without epilepsy.

Methods

We analyzed non-seizure epochs from scalp EEGs of patients from four groups: SP (n=20), non SP EMAtS (n=5), LGS (n=15), and non-epilepsy group (n=12). EEG data was pre-processed through a pipeline including automatic motion artifact rejection and independent component analysis for cleaning. Ten regions of interest were selected: the right and left prefrontal, frontal, center, parietal and occipital regions. Functional connectivity was analyzed using coherence coupling from 1-20 Hz and 3-70 Hz.

Results

Total connectivity strength (CS) was greater among SP (CS 0.37 ± 0.089) and LGS (CS 0.418 ± 0.089) EEGs, indicating greater hyperexcitability in these groups (Figure 1). Normal EEGs demonstrated the lowest CS among all groups. CS in the LGS group was significantly greater than the normal EEG group (p = 0.02).

Conclusion

SP and LGS EEGs demonstrated greater CS, and thus hyperexcitability, which is consistent with the clinical phenotypes of the SP and LGS. Further evaluation will include analysis of connectivity within brain regions and cross-region for each group. This first step will help identify future potential EEG biomarkers and predictors for SP in children with EMAtS.

Purva Choudhari
University of Texas Southwestern
United States

Xiaoxiao Tao
University of North Texas
United States

Lin Li
University of North Texas
United States

Anatol Bragin
University of California, Los Angeles
United States

Katherine Nickels
Mayo Clinic
United States

Michael Ciliberto
University of Iowa
United States

Laurel Reed
University of Michigan Health
United States

Charuta Joshi
University of Texas Southwestern
United States