Manuela Ochoa-Urrea1,2, Nuria Lacuey1,2, Laura Vilella1,2, Liang Zhu3, Shirin Jamal-Omidi1,2, M R Sandhya Rani1,2, Johnson P Hampson1,2, Mojtaba Dayyani1,2, Jaison Hampson1,2, Norma J Hupp1,2, Shiqiang Tao1,2, Rup K Sainju1,4, Daniel Friedman1,5, Maromi Nei1,6, Catherine Scott1,7, Luke Allen1,7, Brian K Gehlbach1,4, Victoria Reick-Mitrisin8, Stephan Schuele1,9, Jennifer Ogren1,10, Ronald M Harper1,10, Beate Diehl1,7, Lisa M Bateman1,11, Orrin Devinsky1,5, George B Richerson1,4, Guo-Qiang Zhang1,2, Samden D Lhatoo1,2. 1. National Institute of Neurological Disorders and Stroke Center for Sudden Unexpected Death in Epilepsy Research (CSR), McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, United States. 2. Department of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, United States. 3. Biostatistics & Epidemiology Research Design Core, Division of Clinical and Translational Sciences, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, United States. 4. University of Iowa Carver College of Medicine, Iowa City, IA, United States. 5. New York University Langone School of Medicine, New York, NY, United States. 6. Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, United States. 7. Department of Clinical and Experimental Epilepsy, Institute of Neurology, University College London, London, United Kingdom. 8. Case Western Reserve University, Cleveland, OH, United States. 9. Feinberg School of Medicine, Northwestern University, Chicago, IL, United States. 10. Department of Neurobiology and the Brain Research Institute, University of California, Los Angeles (UCLA), Los Angeles, CA, United States. 11. Department of Neurology, Cedars-Sinai Medical Center, Los Angeles, CA, United States.
Abstract
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.
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.
Authors: Ingrid E Scheffer; Samuel Berkovic; Giuseppe Capovilla; Mary B Connolly; Jacqueline French; Laura Guilhoto; Edouard Hirsch; Satish Jain; Gary W Mathern; Solomon L Moshé; Douglas R Nordli; Emilio Perucca; Torbjörn Tomson; Samuel Wiebe; Yue-Hua Zhang; Sameer M Zuberi Journal: Epilepsia Date: 2017-03-08 Impact factor: 5.864
Authors: Julia Scholly; Fabrice Bartolomei; Maria Paola Valenti-Hirsch; Clotilde Boulay; Anne De Saint Martin; Alexander Timofeev; Pierre Kehrli; Edouard Hirsch Journal: Epileptic Disord Date: 2017-09-01 Impact factor: 1.819
Authors: Laura Vilella; Nuria Lacuey; Johnson P Hampson; Liang Zhu; Shirin Omidi; Manuela Ochoa-Urrea; Shiqiang Tao; M R Sandhya Rani; Rup K Sainju; Daniel Friedman; Maromi Nei; Kingman Strohl; Catherine Scott; Luke Allen; Brian K Gehlbach; Norma J Hupp; Jaison S Hampson; Nassim Shafiabadi; Xiuhe Zhao; Victoria Reick-Mitrisin; Stephan Schuele; Jennifer Ogren; Ronald M Harper; Beate Diehl; Lisa M Bateman; Orrin Devinsky; George B Richerson; Philippe Ryvlin; Guo-Qiang Zhang; Samden D Lhatoo Journal: Neurology Date: 2020-12-02 Impact factor: 9.910
Authors: Cory A Massey; Samantha J Thompson; Ryan W Ostrom; Janice Drabek; Olafur A Sveinsson; Torbjörn Tomson; Elisabeth A Haas; Othon J Mena; Alica M Goldman; Jeffrey L Noebels Journal: Brain Commun Date: 2021-07-09