Jo Sourbron, Renee Proost, Jan Vandenneucker, Valentina Ticcinelli, Filip Roelens, An-Sofie Schoonjans, Els Sercu, Helene Verhelst, Katrien Jansen, Lieven Lagae
{"title":"Seizure quantification in sunflower syndrome by a wrist-worn device.","authors":"Jo Sourbron, Renee Proost, Jan Vandenneucker, Valentina Ticcinelli, Filip Roelens, An-Sofie Schoonjans, Els Sercu, Helene Verhelst, Katrien Jansen, Lieven Lagae","doi":"10.1002/epd2.20318","DOIUrl":"https://doi.org/10.1002/epd2.20318","url":null,"abstract":"<p><strong>Objective: </strong>Sunflower syndrome is a rare photosensitive childhood-onset epilepsy, featuring repetitive handwaving events (HWE) triggered by light. Objective documentation of these HWE can be difficult due to the numerous events occurring daily and/or caregivers who document the seizures but are not always present. Hence, seizure diaries can be underreporting.</p><p><strong>Methods: </strong>We performed a feasibility study in three Belgian Sunflower syndrome individuals to assess the possibility to quantify the HWE by a wrist-worn wearable device (Axivity AX6). We conducted a structured exercise aiming to capture patterns of possible confounders in a controlled environment. Subsequently, patients wore the device for three to six consecutive days and nights at home. Spectral power analyses were performed to characterize the frequency signature of the different movements.</p><p><strong>Results: </strong>The HWE of patient A and B showed homogeneity and narrow-band frequencies. Patient C did not experience any HWE at the start of the study due to proper seizure control. Regarding HWE, there was a higher spectral power for Gyroscope Z (Gz) compared to Gy. The inter-subject variability for HWE frequency peaks was in the 3-6 Hz range. Computer analysis by visual annotation, without checking the seizure diary, detected 71% of the HWE if the HWE lasted for longer than 5 s (sensitivity 64%). For shorter HWE duration, the detection rate was 50% but seemed to be higher if there was a concordant change of eye movement (63%) (sensitivity 36%). The most obvious confounder was toothbrushing (TB). However, TB showed a different pattern: that is, higher or comparable spectral power for Gy compared to Gz. There was also a higher or comparable spectral power for Gy compared to Gz for \"waving hello\".</p><p><strong>Significance: </strong>We show that the wearable movement sensor Axivity AX6 can detect HWE in Sunflower syndrome individuals and distinguish them from confounders in a real-world setting.</p>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2024-12-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142787635","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Marisa Armeno, Eric H Kossoff, Laura Guilhoto, Roberto Caraballo
{"title":"Survey of Latin American ketogenic diet centers: Challenges and success for \"La Terapia Cetogénica\".","authors":"Marisa Armeno, Eric H Kossoff, Laura Guilhoto, Roberto Caraballo","doi":"10.1002/epd2.20319","DOIUrl":"https://doi.org/10.1002/epd2.20319","url":null,"abstract":"<p><strong>Objective: </strong>Despite growing recognition of the efficacy of ketogenic diet therapies (KDT) in reducing seizure frequency and improving the quality of life for individuals with epilepsy, several factors hinder their widespread adoption across Latin America. Specific challenges in the region have been discussed but not formally studied. Currently, no data exist on the availability and operation of KDT teams in the region. To address this gap, we conducted a survey of KDT centers across Latin American countries in the framework of the biannual International Ketogenic Diet Conference in September 2023.</p><p><strong>Methods: </strong>A seven-question survey was distributed via email to neurologists, dietitians, or through indexed publications.</p><p><strong>Results: </strong>Of the 16 centers approached, 14 (87.5%) responded, identifying 98 KDT centers. Nearly half (49%) are public institutions, primarily in Brazil, Chile, and Mexico; 44 are private; and 5 are mixed. Core teams in all centers included a neurologist and dietitian, with some teams in Argentina, Chile, and Paraguay also involving a medical nutritionist. None of the centers reported a protocol for transitioning to adult care, and consistent data on Glut1 patients and protocols were lacking.</p><p><strong>Significance: </strong>Over the past two decades, the KDT landscape in Latin America has improved, with established centers expanding and new ones emerging. However, continued efforts are needed to promote KDT adoption in countries where it is not yet practiced and in regions with limited resources and expertise.</p>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2024-12-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142787649","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Elissa G. Yozawitz, Maria Roberta Cilio, Eli M. Mizrahi, Jee-Young Moon, Solomon L. Moshé, Magda L. Nunes, Perrine Plouin, Sameer Zuberi, Ronit M. Pressler
{"title":"ILAE neonatal seizure framework to aide in determining etiology","authors":"Elissa G. Yozawitz, Maria Roberta Cilio, Eli M. Mizrahi, Jee-Young Moon, Solomon L. Moshé, Magda L. Nunes, Perrine Plouin, Sameer Zuberi, Ronit M. Pressler","doi":"10.1002/epd2.20312","DOIUrl":"10.1002/epd2.20312","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>To employ the neonatal seizure framework developed by the International League Against Epilepsy (ILAE) Neonatal Task force to assess its usefulness in determining the etiology of neonatal seizures.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>The members of the ILAE Neonatal Task Force evaluated 157 seizures from 146 neonates to determine internal validity and associations between semiology and a specific etiology.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Provoked neonatal electrographic and electroclinical seizures were due to multiple etiologies. For electroclinical seizures, unilateral clonic seizures were typically seen with vascular etiologies, focal tonic seizures and sequential seizures with genetic etiologies, and myoclonic seizures with inborn errors of metabolism. Electrographic seizures were often seen in hypoxic–ischemic encephalopathy or vascular etiologies.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Significance</h3>\u0000 \u0000 <p>These data suggest that the ILAE neonatal seizure classification may be used as a bedside tool to aid and guide workup to determine the etiology of seizures.</p>\u0000 </section>\u0000 </div>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":"27 1","pages":"64-70"},"PeriodicalIF":1.9,"publicationDate":"2024-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142734370","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kaley J. Marcinski Nascimento, Fábio A. Nascimento, Sándor Beniczky
{"title":"Surface electromyography patterns of epileptic seizures","authors":"Kaley J. Marcinski Nascimento, Fábio A. Nascimento, Sándor Beniczky","doi":"10.1002/epd2.20314","DOIUrl":"10.1002/epd2.20314","url":null,"abstract":"","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":"27 1","pages":"130-134"},"PeriodicalIF":1.9,"publicationDate":"2024-11-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142689439","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Genetic neonatal seizures in the neonatal intensive care unit: Diagnostic and prognostic implications for three families.","authors":"Rongrong Chen, Huiming Wu, Yi Lu, Honggang Yin, Xueqian Wang, Xiaohua Zhang","doi":"10.1002/epd2.20315","DOIUrl":"https://doi.org/10.1002/epd2.20315","url":null,"abstract":"<p><strong>Objective: </strong>We investigated neonatal seizures in three probands admitted to the neonatal intensive care units and their affected family members.</p><p><strong>Methods: </strong>Whole exome sequencing (WES) was performed along with confirmation by Sanger sequencing and segregation analysis. Copy number variant (CNV) analysis was also conducted. Neuroimaging, electroencephalography, and metabolic analysis revealed clinical phenotypes.</p><p><strong>Results: </strong>Bi-allelic variants c.1025T>C and c.1150G>A in MOCS1 were found in twin girls with molybdenum cofactor deficiency. The c.1025T>C variant was novel. A c.877C>T variant in KCNQ2 co-segregated with seizures in a family. A de novo 6.25 Mb duplication on 2q24.3 encompassing SCN1A, SCN2A, and SCN3A was identified in a proband who demonstrated normal development without seizures on follow-up.</p><p><strong>Significance: </strong>WES facilitated the molecular diagnosis of neonatal seizures in the study participants. Variants in the KCNQ2 and MOCS1 genes were classified as likely pathogenic based on our findings. The individual with a duplication of the sodium channel gene cluster on 2q24.3 exhibited additional phenotypes. Our investigation expanded the genotype-phenotype spectrum.</p>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2024-11-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142683410","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Nucleus accumbens shell electrical lesion attenuates seizures and gliosis in chronic temporal lobe epilepsy rats.","authors":"Shuaishuai Xue, Peiyao Yi, Yangqi Mao, Zhengming Zhan, Yonghua Cai, Zibin Song, Kewan Wang, Kaijun Yang, Ye Song, Xingqin Wang, Hao Long","doi":"10.1002/epd2.20316","DOIUrl":"https://doi.org/10.1002/epd2.20316","url":null,"abstract":"<p><strong>Objective: </strong>Temporal lobe epilepsy (TLE) is the most prevalent form of epilepsy. Prior research has indicated the involvement of the nucleus accumbens shell (NAcSh) in the process of epileptogenesis, thereby implying its potential as a therapeutic target for TLE. In the present study, we investigated the antiepileptic effect of the NAcSh electrical lesion.</p><p><strong>Methods: </strong>Chronic TLE was induced by stereotactic injection of kainic acid (KA) into the hippocampus 3 weeks after KA administration, and NAcSh electrical lesions were performed. Seizures in rats were monitored by video electroencephalogram (EEG) 1 week following the NAcSh electrical lesion. Besides, the spatial memory function assessment in rats was conducted using the Morris water maze (MWM) test in the final week of the experiment. Later, hippocampal glial cell activation and neuron loss in rats were evaluated through immunohistochemistry.</p><p><strong>Results: </strong>TLE rats subjected to NAcSh electrical lesion exhibited a significant reduction in the frequency of seizures compared to untreated TLE rats. Furthermore, NAcSh electrical lesion led to less activation of hippocampal glial cells and fewer neuronal loss in TLE rats. It is worth noting that the NAcSh electrical lesion did not cause additional memory impairment.</p><p><strong>Significance: </strong>In the present study, the NAcSh electrical lesion exhibited a definitive therapeutic effect on the chronic TLE rat model, potentially due to decreased hippocampal TLE-induced activation of glial cells and neuron loss. In conclusion, our results indicated that the NAcSh is a promising therapeutic target for TLE and possesses high potential for clinical application.</p>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2024-11-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142683412","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kaley J. Marcinski Nascimento, Sándor Beniczky, Fábio A. Nascimento
{"title":"Slow alpha variant: A normal EEG pattern","authors":"Kaley J. Marcinski Nascimento, Sándor Beniczky, Fábio A. Nascimento","doi":"10.1002/epd2.20313","DOIUrl":"10.1002/epd2.20313","url":null,"abstract":"","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":"27 1","pages":"127-129"},"PeriodicalIF":1.9,"publicationDate":"2024-11-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142669650","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
J. Proietti, C. Nanyunja, S. R. Mathieson, E. Duckworth, S. Sadoo, I. Mambule, A. Nakimuli, C. J. Tann, G. B. Boylan
{"title":"Retrospective characterization of seizure semiology and treatment using continuous video-EEG monitoring in neonatal encephalopathy in Uganda","authors":"J. Proietti, C. Nanyunja, S. R. Mathieson, E. Duckworth, S. Sadoo, I. Mambule, A. Nakimuli, C. J. Tann, G. B. Boylan","doi":"10.1002/epd2.20299","DOIUrl":"10.1002/epd2.20299","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Neonatal encephalopathy (NE) is a leading cause of childhood death and disability, particularly in sub-Saharan Africa. Detection of NE-related seizures is challenging. We explored NE seizure semiology and management in Uganda.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Video-EEG was recorded (days 1–5), seizure semiology reviewed according to ILAE classification and administration of antiseizure medication (ASM) evaluated. Clinicians treated seizures based on the clinical presentation alone.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Among 50 participants, 52% (26) had EEG-confirmed seizures; 70% (18) combined electroclinical/electrographic; 4% (1) exclusively electroclinical; 22% (6) electrographic. Of those with electroclinical seizures (19), 42% displayed >1 semiology. Distribution of seizure semiology was; clonic 34% (11); autonomic 24% (8, of which 6 had prolonged ictal apnea); automatisms 18% (6); behavioral arrest 12% (4); and sequential 12% (4). ASM was administered to 64% (32/50). Of those with EEG-confirmed seizures, only 62% (16/26) received ASM. In the non-seizure group, 38% (9/24) received ASM during monitoring. ASM was administered 42 times, of which 45% (19) were considered appropriate.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Significance</h3>\u0000 \u0000 <p>In this Ugandan NE population, incidence of seizures was high and clinical manifestations frequent. Clonic, autonomic and automatisms were most common. Clinical management was challenging, with both under and overtreatment evident. Respiratory impairment due to autonomic seizures frequently went unrecognized and is a prominent concern, particularly in settings without neonatal intensive care.</p>\u0000 </section>\u0000 </div>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":"27 1","pages":"31-43"},"PeriodicalIF":1.9,"publicationDate":"2024-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epd2.20299","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142649521","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pamela Engelhart, Caleb Marcin, Jason Lerner, David Dill, Gil L'Italien, Vlad Coric, Joyce Matsumoto, Michele H. Potashman
{"title":"Determinants of health-related quality of life of patients with focal epilepsy: A systematic literature review","authors":"Pamela Engelhart, Caleb Marcin, Jason Lerner, David Dill, Gil L'Italien, Vlad Coric, Joyce Matsumoto, Michele H. Potashman","doi":"10.1002/epd2.20292","DOIUrl":"10.1002/epd2.20292","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Focal epilepsy can have significant negative impacts on a person's health-related quality of life (HRQoL). Although studies have been published on HRQoL in persons with focal epilepsy (PWFE), determinants of HRQoL have not been comprehensively examined. This systematic literature review (SLR) queried existing literature to identify aspects associated with HRQoL in PWFE without focus on resective epilepsy surgery, with an interest in identifying modifiable determinants for medical/nonmedical interventions.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This SLR was conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Searches were conducted in PubMed and Google Scholar for articles published from January 1, 1900, to February 19, 2023, reporting on the association between HRQoL or employability and a range of demographic, psychosocial, or epilepsy-related factors and comorbidities in PWFE.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 879 abstracts were identified, with 126 manuscripts reviewed and 37 studies selected for inclusion that quantified the relationship between HRQoL and the variable of interest by multivariate (<i>N</i> = 21) or univariate only (<i>N</i> = 15) methods; 10 multivariate models also included univariate data. In adjusted models, the most commonly examined determinants of HRQoL included depression (<i>n</i> = 15/21), number of antiseizure medications (ASMs; <i>n</i> = 13/21), seizure frequency (continuous seizure count, <i>n</i> = 11/21; seizure freedom, <i>n</i> = 5/21), anxiety (<i>n</i> = 10/21), duration of disease (<i>n</i> = 9/21), and cognition (n = 9/21). Depression, anxiety, and cognition were frequently seen as significant contributors to HRQoL when studied (14/15 [93%], 9/10 [90%], and 7/9 [78%], respectively). Among concepts studied less frequently, ASM severity/adverse event burden was significant each time examined (in 5/19 studies). Attainment of seizure freedom and employability was significant 75% (<i>n</i> = 3/4) and 72% (<i>n</i> = 5/7) of the time, respectively.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Significance</h3>\u0000 \u0000 <p>Poor HRQoL in PWFE can be attributed to a multitude of factors, including depression, anxiety, factors in disease management, and employability. An unmet need remains in addressing elements associated with poor HRQoL in this population.</p>\u0000 </section>\u0000 </div>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":"27 1","pages":"9-30"},"PeriodicalIF":1.9,"publicationDate":"2024-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epd2.20292","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142640205","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Joseph Ta, Christopher Smith, Nyasia Mayaudon Finley, Martin Gallagher, Andre LaGrange, Kelly Lowen, Trevor Meyer, Vivek Ganesh, Jay V. Shah, William Nobis
{"title":"Near SUDEP in a patient with craniosynostosis syndrome and temporal lobe encephaloceles","authors":"Joseph Ta, Christopher Smith, Nyasia Mayaudon Finley, Martin Gallagher, Andre LaGrange, Kelly Lowen, Trevor Meyer, Vivek Ganesh, Jay V. Shah, William Nobis","doi":"10.1002/epd2.20304","DOIUrl":"10.1002/epd2.20304","url":null,"abstract":"<p>Crouzon and Apert syndromes are rare genetic disorders characterized by craniosynostosis, a condition with premature fusion of sutures of the skull bones, which is occasionally associated with epilepsy. We report a case of a patient with Crouzon syndrome and medically refractory temporal lobe epilepsy with temporal encephaloceles that experienced a near sudden unexpected death in epilepsy (SUDEP) event. This case illuminates both the need to consider encephaloceles as a treatable lesional etiology of epilepsy in craniosynostosis syndromes and that postictal apnea may be an important mechanism of cardiorespiratory collapse seen in SUDEP, emphasizing the importance of cardiorespiratory monitoring.</p><p>The patient has a history of medically refractory focal presumed non-lesional epilepsy (onset age: 20 years old) and Crouzon syndrome. Informed consent was obtained from the patient. The patient suffers from focal seizures with impaired awareness (FIAS) and focal to bilateral tonic–clonic seizures (FBTCS). FIAS occur two to four times per month and have an aura of blurred vision, a sense of fear, and tachycardia at onset followed by dialepsis and oral automatisms. FBTCS occurs one to two times a year.</p><p>While in the EMU, the patient suffered a near SUDEP event (Figure 1). At the clinical onset, tachycardia (120 bpm) and oral automatisms were followed by versive left head and body turn before tonic stiffening, left lower extremity clonus, and generalized tonic–clonic convulsion. Electrographically there was rhythmic theta in the right frontotemporal region, evolving bilaterally. Postictally, he was apneic with a heart rate of 110 bpm and had diffuse postictal generalized EEG suppression (PGES). Within 12 s, he became bradycardic, and within another 40 s, he became asystolic for 17 s before a heart rate returned and quickly evolved into tachycardia (> 140 bpm). Stertorous breaths are observed for the first time 2 mins and 15 s after the seizure ended. Immediately following the return of respiratory effort, the rapid tachycardia and diffuse PGES resolve. An arm wearable device captured generalized seizure onset via sEMG and motion sensors. A rapid response was called, and the patient was intubated. While no cardiorespiratory resuscitation was performed (CPR, rescue breathing, etc.), the patient had a cardiorespiratory arrest with no other cause, consistent with a near SUDEP.<span><sup>1</sup></span></p><p>Temporal encephaloceles are increasingly recognized as a cause of drug-resistant temporal lobe epilepsy as surgical intervention renders patients seizure-free.<span><sup>2</sup></span> Because Apert and Crouzon<span><sup>3</sup></span> syndromes have encephaloceles, this may be a prominent etiology of epilepsy in patients with these syndromes. As they frequently have medically refractory epilepsy, they are at higher risk for SUDEP.<span><sup>2, 3</sup></span> Airway obstruction that is often associated with these syndromes may also put these","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":"27 1","pages":"111-113"},"PeriodicalIF":1.9,"publicationDate":"2024-11-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epd2.20304","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142631866","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}