Association of Peri-ictal Brainstem Posturing With Seizure Severity and Breathing Compromise in Patients With Generalized Convulsive Seizures

Vilella, Laura; Lacuey, Nuria; Hampson, Johnson P.; Zhu, Liang; Omidi, Shirin Jamal; Ochoa-Urrea, Manuela; Tao, Shiqiang; Rani, M.R. Sandhya; Sainju, Rup K.; Friedman, Daniel; Nei, Maromi; Scott, Catherine; Allen, Luke; Gehlbach, Brian K.; Hupp, Norma J.; Hampson, Jaison; Shafiabadi, Nassim; Reick-Mitrisin, Victoria; Schuele, Stephan; Ogren, Jennifer A.; Harper, Ronald M.; Diehl, Beate; Bateman, Lisa M.; Devinsky, Orrin; Richerson, George B.; Ryvlin, Philippe; Zhang, Guo Qiang; Lhatoo, Samden D.

doi:10.1212/wnl.0000000000011274

Cited by 18 publications

(29 citation statements)

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“…An increase in age correlates with an increase in tonic phase duration, which in turn correlates with an increase in PGES duration [17]. In adults, the presence of a tonic phase is associated with PGES, and the latter is remarkably longer with decerebrate posturing [26]. Compared to our cohort, adults seem to have more GTCS with symmetrical tonic arm extension [26,27].…”

Section: Pges and Gtcs Semiology In Children Are Relatedsupporting

confidence: 37%

“…Seizure semiology is another potential marker of SUDEP. Studies in adults showed a positive association between GTCS semiology manifesting as decerebrate posturing, with symmetric bilateral arm tonic extension, and PGES duration [26,27]. Other semiologic characteristics, such as those related to the tonic phase of GTCS, may also be related [28,29].…”

Section: Introductionmentioning

confidence: 99%

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Association between semiologic, autonomic, and electrographic seizure characteristics in children with generalized tonic-clonic seizures

Atrache

Tamilia

Amengual‐Gual

et al. 2021

Epilepsy & Behavior

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Introduction: Generalized tonic-clonic seizures (GTCS) are associated with elevated electrodermal activity (EDA) and postictal generalized electroencephalographic suppression (PGES), markers that may indicate sudden unexpected death in epilepsy (SUDEP) risk. This study investigated the association of GTCS semiology, EDA, and PGES in children with epilepsy. Methods: Patients admitted to the Boston Children's Hospital long-term video-EEG monitoring unit wore a sensor that records EDA. We selected patients with at least one GTCS and reviewed video-EEGs for semiology, tonic and clonic phase duration, total clinical seizure duration, electrographic onset, offset, and PGES. We grouped patients into three semiology classes: GTCS 1: bilateral symmetric tonic arm extension, GTCS 2: no specific tonic arm extension or flexion, GTCS 3: unilateral or asymmetrical arm extension, tonic arm flexion or posturing that does not fit into GTCS 1 or 2. We analyzed the correlation between semiology, EDA, and PGES, and measured the area under the curve (AUC) of the ictal EDA (seizure onset to one hour after), subtracting baseline EDA (one-hour seizure-free before seizure onset). Using generalized estimating equation (GEE) and linear regression, we analyzed all seizures and single episodes per patient. Results: We included 30 patients (median age 13.8 ± 3.6 years, 46.7% females) and 53 seizures. With GEE, GTCS 1 was associated with longer PGES duration compared to GTCS 2 (Estimate (b) = À26.32 s, 95% Confidence Interval (CI): À36.46 to À16.18, p < 0.001), and the presence of PGES was associated with greater EDA change (b = 429604 lS, 95% CI: 3550.96 to 855657.04, p = 0.048). With single-episode analysis, GTCS 1 had greater EDA change than GTCS 2 ((b = À601339 lS, 95% CI: À1167016.56 to À35661.44, p = 0.047). EDA increased with PGES presence (b = 637500 lS, 95% CI: 183571.84 to 1091428.16, p = 0.01) and duration (b = 16794 lS, 95% CI: 5729.8 to 27858.2, p = 0.006). Patients with GTCS 1 had longer PGES duration compared to GTCS 2 (b = À30.53 s, 95% CI: À44.6 to À16.46, p < 0.001) and GTCS 3 (b = À22.07 s, 95% CI: À38.95 to À5.19, p = 0.016). Conclusion:In children with epilepsy, PGES correlates with greater ictal EDA. GTCS 1 correlated with longer PGES duration and may indirectly correlate with greater ictal EDA. Our study suggests potential applications in monitoring and preventing SUDEP in these patients.

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Section: Pges and Gtcs Semiology In Children Are Relatedsupporting

confidence: 37%

Section: Introductionmentioning

confidence: 99%

Association between semiologic, autonomic, and electrographic seizure characteristics in children with generalized tonic-clonic seizures

Atrache

Tamilia

Amengual‐Gual

et al. 2021

Epilepsy & Behavior

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“…Interestingly, in grouped analysis, we found some seizure features to worsen, namely duration of decerebrate posturing and ICA duration. Both decerebrate posturing duration and ICA duration are putative markers of seizure severity (39,40). Decerebrate posturing has been associated with PGES, a proposed SUDEP biomarker (40).…”

Section: Discussionmentioning

confidence: 99%

“…Both decerebrate posturing duration and ICA duration are putative markers of seizure severity (39,40). Decerebrate posturing has been associated with PGES, a proposed SUDEP biomarker (40). Similarly, prolonged ICA (>60 s) has been associated with severe hypoxemia (39).…”

Section: Discussionmentioning

confidence: 99%

Seizure Clusters, Seizure Severity Markers, and SUDEP Risk

et al. 2021

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Rationale: Seizure clusters may be related to Sudden Unexpected Death in Epilepsy (SUDEP). Two or more generalized convulsive seizures (GCS) were captured during video electroencephalography in 7/11 (64%) patients with monitored SUDEP in the MORTEMUS study. It follows that seizure clusters may be associated with epilepsy severity and possibly with SUDEP risk. We aimed to determine if electroclinical seizure features worsen from seizure to seizure within a cluster and possible associations between GCS clusters, markers of seizure severity, and SUDEP risk.Methods: Patients were consecutive, prospectively consented participants with drug-resistant epilepsy from a multi-center study. Seizure clusters were defined as two or more GCS in a 24-h period during the recording of prolonged video-electroencephalography in the Epilepsy monitoring unit (EMU). We measured heart rate variability (HRV), pulse oximetry, plethysmography, postictal generalized electroencephalographic suppression (PGES), and electroencephalography (EEG) recovery duration. A linear mixed effects model was used to study the difference between the first and subsequent seizures, with a level of significance set at p < 0.05.Results: We identified 112 GCS clusters in 105 patients with 285 seizures. GCS lasted on average 48.7 ± 19 s (mean 49, range 2–137). PGES emerged in 184 (64.6%) seizures and postconvulsive central apnea (PCCA) was present in 38 (13.3%) seizures. Changes in seizure features from seizure to seizure such as seizure and convulsive phase durations appeared random. In grouped analysis, some seizure features underwent significant deterioration, whereas others improved. Clonic phase and postconvulsive central apnea (PCCA) were significantly shorter in the fourth seizure compared to the first. By contrast, duration of decerebrate posturing and ictal central apnea were longer. Four SUDEP cases in the cluster cohort were reported on follow-up.Conclusion: Seizure clusters show variable changes from seizure to seizure. Although clusters may reflect epilepsy severity, they alone may be unrelated to SUDEP risk. We suggest a stochastic nature to SUDEP occurrence, where seizure clusters may be more likely to contribute to SUDEP if an underlying progressive tendency toward SUDEP has matured toward a critical SUDEP threshold.

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“…In the highlighted study by Vilella and colleagues, the authors sought to indirectly relate presumed brainstem involvement during generalized seizures with clinical factors that might predispose to SUDEP in adult epilepsy patients. 2 Data were collected from 295 generalized seizures in 180 patients undergoing video-electroencephalogram (EEG) monitoring at multiple epilepsy monitoring units (EMUs), with approximately half of seizures captured during wakefulness and half during sleep. Approximately 16% of patients had a generalized epilepsy subtype, while most had presumed focal or multifocal onset, with temporal lobe localization being most common.…”

Section: Commentarymentioning

confidence: 99%

The Underappreciated But Potentially Lethal Role of Brainstem Dysfunction in Epilepsy

Englot¹

2021

Epilepsy Curr

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Association of Peri-ictal Brainstem Posturing With Seizure Severity and Breathing Compromise in Patients With Generalized Convulsive Seizures

Cited by 18 publications

References 47 publications

Association between semiologic, autonomic, and electrographic seizure characteristics in children with generalized tonic-clonic seizures

Association between semiologic, autonomic, and electrographic seizure characteristics in children with generalized tonic-clonic seizures

Seizure Clusters, Seizure Severity Markers, and SUDEP Risk

The Underappreciated But Potentially Lethal Role of Brainstem Dysfunction in Epilepsy

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