Management Of Status Epilepticus Research Articles

Covid-19 Related Seizures

Background: The Coronavirus (Sars-CoV-2, COVID-19) has been evolving its viral strains, prevalence, symptomatology, and sequelae of disease for the past three years. Although the most recognized COVID-19 symptoms involve the respiratory tract; neurological symptoms have been documented. Specifically, seizures have specifically been discussed in the literature but remain both under-recognized and under-reported in clinical practice. Aim: To review of the literature of adult patients with COVID-19 and seizures and integrate into clinical practice in the acute care environment; from presentation to the emergency Department to discharge. Methods: A narrative literature review was conducted to identify all reported clinical studies involving adult patients with COVID-19 and de novo seizures from MEDLINE, yielding 108 relevant publication titles and abstracts. Additional three relevant studies were discovered through manual search of reference lists of included studies. After excluding non-related publications, 58 publications underwent full-text review. Reporting of results was guided by the Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR). Results: Data was organized into the following themes in the literature: prevalence of seizure occurrence in patients with COVID-19; pathophysiology discussing possible cause; CSF and EEG findings in these patients, and outcomes both in and out of hospital. Conclusion: Seizures were reported as both the presenting symptom of COVID-19 infection and a sequelae of the disease. Heterogeneity identified in both severity and pathogenesis of disease may partly account for the variability in reporting. Seizures may occur as single incidences, with no further implication to the patient or they may occur in the context of New Onset Refractory Status Epilepticus. Patients may require critical care for management of Status Epilepticus or encephalopathy with accompanying seizures. Clinician vigilance is essential in identifying COVID-19 infection in patients presenting to Emergency Services with seizures. Early recognition impacts patient care both in-hospital and at post-discharge follow-up.

The pharmacologic management of status epilepticus in pregnant patients: a scoping review

AbstractBackgroundStatus epilepticus (SE) is defined as 5 min or more of seizure activity or two recurrent seizures without a return to baseline. Healthcare providers encounter a challenge when a patient with SE is pregnant. SE is not only detrimental to the mother but can also put the baby at risk of severe harm. SE must be treated rapidly and therefore healthcare providers have very little time to thoroughly review the risk and benefits of available antiseizure medication in this population.AimTo evaluate the current available evidence related to the management of SE in pregnancy.DesignA literature search of PubMed, CINAHL, ProQuest Nursing & Allied Health Source, and Web of Science databases was conducted (2012–2022) using the following search terms: ‘pregnancy’, ‘pregnant women’ OR ‘gestation’ AND ‘status epilepticus’, ‘generalized status epilepticus’, ‘generalized convulsive status epilepticus’, ‘non convulsive status epilepticus’ OR ‘non‐convulsive status epilepticus’. Full‐text randomised controlled trials, clinical trials, observational studies, and case reports published in English were included. Data were extracted and the quality of the studies was evaluated using the Mixed Methods Appraisal Tool.ResultsThe literature described 29 pregnancies and 30 total foetuses. Intravenous benzodiazepine use for emergent control was reported in 45% of patients. Phenytoin and levetiracetam were primarily utilised for urgent control, with a variety of agents used for refractory SE. Ninety‐seven percent of maternal outcomes were reported as positive. The most common outcome was the birth of a healthy term infant. There were seven cases of pregnancy loss.ConclusionPublications pertaining to the treatment of SE in pregnancy are limited to case reports and small observational studies. Use of a benzodiazepine followed by levetiracetam or phenytoin is appropriate, whereas valproic acid should be utilised only when necessary due to the risk of major congenital malformation.

In-hospital Management of Status Epilepticus: Does Uniformity Mean Quality?

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Challenges of status epilepticus management in a resource-limited setting: A review

Status epilepticus (SE) is a life-threatening neurological condition with significant mortality. Rapid management is essential to minimize the mortality and disability of SE. Two recent trials provided evidence to guide SE management in early and established stages. The Rapid Anticonvulsant Medication Prior To Arrival Trial (RAMPART, 2011) showed that intramuscular midazolam is a better alternative for early convulsive SE in prehospital settings. The Established Status Epilepticus Treatment Trial (ESETT, 2020) supported the use of sodium valproate and levetiracetam as second-line treatment for its efficacy and shorter administration time. However, there are challenges to revising the status epilepticus management in resource-limited settings, in pre-hospital, first- and second-line treatment, as well as management of refractory and super-refractory SE. These challenges included restrictions or lack of training in the administration of benzodiazepine in the prehospital setting, limited availability and accessibility of newer antiseizure medications (ASMs) in emergency departments and smaller hospitals, and low clinicians’ awareness of the latest evidence. A collaborative effort to educate, improve awareness, and make certain ASMs more readily available is recommended to achieve a better clinical outcome in SE.

Status epilepticus management in patients with brain tumors. A cohort study

PurposeStatus epilepticus (SE) represents a neurological emergency with significant morbidity and mortality. SE in patients with primary brain tumors received only limited attention to date; detailed analysis of treatment flow is lacking, especially as compared to other SE causes. This study aims to describe the frequency and treatment flow of tumor-related SE and compare it to other SE etiologies. MethodsRetrospective cohort study based on an institutional SE registry (SERCH) comprising adult SE (excluding post-anoxic causes), treated between January 2013 and December 2022, comparing SE management, frequency of refractory SE, and clinical outcome, among four patients’ groups stratified by SE etiology: Non-neoplastic, Gliomas, Brain metastases, Other brain tumors. ResultsWe analyzed 961 episodes in 831 patients (Non-neoplastic: 649, Gliomas: 85, Metastases: 77, Other brain tumors: 20). Although tumor-patients presented more often with focal episodes and less consciousness impairment than non-neoplastic patients, administration of benzodiazepines as first-line treatment (>75% across all groups), and utilization of second-line ASM were similar across groups. Treatment adequacy was marginally higher in glioma patients compared to the non-neoplastic population (p: 0.049), while refractory SE was comparable in all groups (p: 0.269). No significant differences in clinical outcomes were observed (mortality: non-neoplastic (89/649, 13.7%), glioma (8/85, 9.4%), metastases (14/77, 18.2%), other tumors (5/20, 25.0%), p: 0.198; non-neoplastic vs. glioma, p: 0.271) ConclusionTumor-associated SE represents 1/5 of all SE episodes, and is managed similarly to other SE causes. Treatment responsiveness and short-term clinical outcomes also exhibit comparable results.

Emergent Management of Status Epilepticus.

Status epilepticus is a neurologic emergency that can be life- threatening. The key to effective management is recognition and prompt initiation of treatment. Management of status epilepticus requires a patient-specific-approach framework, consisting of four axes: (1) semiology, (2) etiology, (3) EEG correlate, and (4) age. This article provides a comprehensive overview of status epilepticus, highlighting the current treatment approaches and strategies for management and control. Administering appropriate doses of antiseizure medication in a timely manner is vital for halting seizure activity. Benzodiazepines are the first-line treatment, as demonstrated by three randomized controlled trials in the hospital and prehospital settings. Benzodiazepines can be administered through IV, intramuscular, rectal, or intranasal routes. If seizures persist, second-line treatments such as phenytoin and fosphenytoin, valproate, or levetiracetam are warranted. The recently published Established Status Epilepticus Treatment Trial found that all three of these drugs are similarly effective in achieving seizure cessation in approximately half of patients. For cases of refractory and super-refractory status epilepticus, IV anesthetics, including ketamine and γ-aminobutyric acid-mediated (GABA-ergic) medications, are necessary. There is an increasing body of evidence supporting the use of ketamine, not only in the early phases of stage 3 status epilepticus but also as a second-line treatment option. As with other neurologic emergencies, "time is brain" when treating status epilepticus. Antiseizure medication should be initiated quickly to achieve seizure cessation. There is a need to explore newer generations of antiseizure medications and nonpharmacologic modalities to treat status epilepticus.

Can critical care transport be safely reduced in children intubated during emergency management of status epilepticus in the United Kingdom: a national audit with case–control analysis

ObjectiveThis study describes the baseline clinical characteristics, predictors of successful extubation at referring hospitals and short-term outcomes of children intubated for status epilepticus and referred to United Kingdom (UK) paediatric...

Current practice and safety measures in epilepsy monitoring units in the Gulf Cooperation Council Countries: A cross-sectional study

BackgroundAn increasing number of Epilepsy Monitoring Units (EMU) display various practices and safety protocols. EMU settings should meet clear, standardized safety protocols to avoid seizure adverse events (SAE). We aim to provide the foundational framework facilitating the establishment of unified evidence-based safety regulations to address the practices and safety measures implemented within the Gulf Cooperation Council (GCC). MethodsIn this cross-sectional study, EMU directors in the GCC were contacted directly by phone to personally complete an electronic 37-item questionnaire sent via text messages and email. From January 2021–December 2021. ResultsSeventeen EMUs from six GCC countries participated in the study. All EMU directors responded to the study. Twelve (70.6%) EMUs monitored adults and children, five (29.4%) monitored adults, and none monitored children only. The number of certified epileptologists in the EMUs ranged from one to eight per unit. Fifteen (88.2%) EMUs applied a continuous observation pattern, whereas two (11.8%) performed daytime only. The precautions most commonly used in the video Electroencephalogram (EEG) were seizure pads and bedside oxygen in 15 EMUs (88.2%). For invasive EEG, seizure pads were used in 9 EMUs (52.9%), %) and IV access in 8 EMUs (47.1%). The occurrence of adverse events varied among EMUs. The most common conditions were postictal psychosis 10 (58.8%), injuries 7 (41.2%), and status epilepticus 6 (35.3%). Falls were mainly related to missed seizures or delayed recognition by video monitors in 8 EMUs (47.1%). The extended EMU stay was because of an insufficient number of recorded seizures in 16 EMUs (94.1%), poor seizure lateralization and localization in 10 (58.8%), and re-introduction of AEDs in nine (52.9%). All EMUs had written acute seizure and status epilepticus management protocols. A postictal psychosis management protocol was available for 10 (58.8%). Medications were withdrawn before admission in 6 EMUs (35.3%). The specific medication withdrawal speed protocol upon admission was available in 7 EMUs (41.2%). Pre-admission withdrawal of medication demonstrated a shorter length of stay in both video and invasive EEG, which was statistically significant (ρ (15) = −.529, p =.029; ρ (7) = −.694, p =.038; respectively). ConclusionThe practice and safety regulations of EMUs in the GCC vary widely. Each EMU reported the occurrences of SAE and injuries. Precautions, protective measures, and management protocols must be reassessed to minimize the number of SAEs and increase the safety of the EMU.

Prophylactic antiseizure medications for recurrent status epilepticus in nonsyndromic childhood epilepsy

PurposeThe management of status epilepticus (SE) has mainly focused on the termination of ongoing SE episodes. However, long-term therapeutic strategies for the prevention of SE are lacking. This study aimed to investigate the effectiveness of prophylactic antiseizure medications (ASMs) for SEs in nonsyndromic childhood epilepsy. MethodsThis retrospective study was conducted at Jikei University Hospital. Patients <18 years of age, diagnosed with epilepsy, and experiencing three or more SE episodes within 1 year between April 1, 2017, and October 1, 2021, were included. ASMs introduced for seizure types that developed into SE were evaluated. The effectiveness of ASMs was determined by using the “Rule of Three”: An ASM was determined effective if patients were free of SE for a duration at least three times that of their longest SE interval in 12 months prior to intervention. ResultsThe investigation included a total of 32 ASMs administered to 13 patients. The longest interval between SE episodes before ASM administration was 28–257 d. The first SE interval after ASM administration was 12–797 d. Levetiracetam (LEV) and clobazam (CLB) showed effectiveness in 2/10 and 5/6 patients, respectively. Other ASMs were ineffective. The leading etiology of epilepsy was perinatal brain injury, identified in four patients, and CLB was effective in all of them. ConclusionsThe present study suggests that CLB and LEV may prolong the SE interval in some cases of nonsyndromic childhood epilepsy. CLB may be beneficial, particularly in patients with perinatal brain injury.

Management of status epilepticus in pregnancy: a clinician survey

BackgroundStatus epilepticus in pregnancy (SEP) is rare and life-threatening for both mother and fetus. There are well-established guidelines for the management of women with epilepsy during pregnancy; however, there is little evidence guiding the management of SEP, leading to uncertainty among treating physicians. Therefore, this survey aims to investigate the real-world practices of physicians treating SEP to explore management approaches for improvements in care.MethodsAn anonymous, electronic survey was created and distributed to neurointensivists and neurologists between September and December 2021.ResultsOne hundred physicians initiated the survey and 95 completed it in full: 87 (87%, 87/100) identified neurology as their primary specialty, 31 had subspecialty training in neurocritical care, and 48 had subspecialty training in epilepsy and/or clinical neurophysiology. Over half of the survey respondents (67%, 67/100) reported having participated in the management of SEP, with 48.9% (49/98) having done so in the past year. Most survey respondents (73%, 73/100) reported that their management approach to SEP is different than that of non-pregnant patients. Survey respondents were more likely to involve epilepsy consultants when treating SEP (58.5%, 58/99) and the vast majority involved Obstetrics/Maternal Fetal Medicine consultants (90.8%, 89/98). Survey respondents showed a clear preference for levetiracetam (89.7%, 87/97) in the treatment of benzodiazepine refractory status epilepticus followed by lacosamide (61%, 60/98) if an additional second line agent was needed. Valproate and phenobarbital were unlikely to be used. There was less agreement for the management of refractory and super-refractory SEP.ConclusionsLevetiracetam is the most frequently used anti-seizure medication (ASM) for benzodiazepine-refractory SEP. Survey participants tended to manage SEP differently than in non-pregnant patients including greater involvement of interdisciplinary teams as well as avoidance of ASMs associated with known teratogenicity.

ACVIM Consensus Statement on the management of status epilepticus and cluster seizures in dogs and cats.

Seizure emergencies (ie, status epilepticus [SE] and cluster seizures [CS]), are common challenging disorders with complex pathophysiology, rapidly progressive drug-resistant and self-sustaining character, and high morbidity and mortality. Current treatment approaches are characterized by considerable variations, but official guidelines are lacking. To establish evidence-based guidelines and an agreement among board-certified specialists for the appropriate management of SE and CS in dogs and cats. None. A panel of 5 specialists was formed to assess and summarize evidence in the peer-reviewed literature with the aim to establish consensus clinical recommendations. Evidence from veterinary pharmacokinetic studies, basic research, and human medicine also was used to support the panel's recommendations, especially for the interventions where veterinary clinical evidence was lacking. The majority of the evidence was on the first-line management (ie, benzodiazepines and their various administration routes) in both species. Overall, there was less evidence available on the management of emergency seizure disorders in cats in contrast to dogs. Most recommendations made by the panel were supported by a combination of a moderate level of veterinary clinical evidence and pharmacokinetic data as well as studies in humans and basic research studies. Successful management of seizure emergencies should include an early, rapid, and stage-based treatment approach consisting of interventions with moderate to preferably high ACVIM recommendations; management of complications and underlying causes related to seizure emergencies should accompany antiseizure medications.

Management of status epilepticus in dogs

Background: Status epilepticus (SE) is a common neurological emergency with high mortality, and is often the first manifestation of a seizure disorder. When faced with a patient in SE (or severe cluster seizures) our priority should always be to stop the seizure activity; however, this should not distract us from assessing our seizuring patient as a whole. Neglecting to do so could lead to severe complications and could dramatically affect the patient's short‐term outcome. Management of dogs in SE requires a swift, coordinated response from all members of the veterinary team to improve chances of survival.Aim of the article: This article aims to provide a step‐by‐step guide for the management of dogs in SE with a focus on strategies to best monitor, stabilise and treat this neurological emergency.

Management of Status Epilepticus by Different Pediatric Departments: Neurology, Intensive Care, and Emergency Medicine

Introduction: The aim of this study was to explore the differences in status epilepticus (SE) management among pediatric neurology, emergency medicine, and intensive care specialists in Turkey. Methods: A 22-item questionnaire regarding first-, second-, and third-line management strategies of SE including demographic characteristics and common etiologies according to the specialty of participants was mailed to 370 specialists working in Turkey. Results: A total of 334 participants (response rate 90%) comprising 136 pediatric neurologists, 102 pediatric emergency medicine specialists, and 96 pediatric intensive care specialists completed the survey. While intensive care specialists frequently managed SE due to metabolic and autoimmune reasons, the most common etiologies encountered by emergency medicine specialists were epilepsy and infections. More than half of the intensive care specialists (64.6%) reported using non-BZD antiseizure medications in the 5th minute of the seizure. Most of the neurologists (76.4%) preferred to administer intravenous (IV) levetiracetam infusion as a second-line agent. About half of intensive care specialists and neurologists tried immunomodulatory therapies in super-refractory SE. Intensive care and emergency medicine specialists were less likely to favor ketogenic diet and pyridoxine therapy for the treatment of super-refractory SE. The rate of requesting EEG monitoring to recognize nonconvulsive SE (NCSE) was found to be very low except for neurologists. Conclusion: There was no consensus among neurologists, intensive care specialists, and emergency medicine specialists in the management of SE in Turkey. Familiarity with particular antiseizure medications and the etiologies they manage seem to be the most important factors influencing the attitudes.

Management of Status Epilepticus in Adults for the Acute Physician

Generalized convulsive status epilepticus is a neurological emergency and defined as a generalized tonic/clonic motor activity (seizure) which continues for more than five minutes or series of seizures which occur without regaining function in between. Common causes for Generalized convulsive status epilepticus are structural brain injury like head injury, stroke, brain tumor, or cerebral anoxia. Noncompliance with antiepileptic medications or rapid withdrawal due to poor absorption from acute illness like diarrhea or vomiting is another common cause, withdrawal from alcohol or benzodiazepines in patients who consume these chemicals daily is a well-known cause for GCSE.

Pediatric status epilepticus management by Emergency Medical Services (the pSERG cohort)

PurposeDelayed treatment in status epilepticus (SE) is independently associated with increased treatment resistance, morbidity, and mortality. We describe the prehospital management pathway and Emergency Medical Services (EMS) timeliness in children who developed refractory convulsive status epilepticus (RCSE). MethodsRetrospective multicenter study in the United States using prospectively collected observational data from June 2011 to March 2020. We selected pediatric patients (one month–21 years) with RCSE initiated outside the hospital and transported to the hospital by EMS. ResultsWe included 91 patients with a median (percentile25-percentile75) age of 3.0 (1.5–7.3) years. The median time from seizure onset to hospital arrival was 45 (30–67) minutes, with a median time cared for by EMS of 24 (15–36) minutes. Considering treatment by caregivers and EMS before hospital arrival, 20 (22%) patients did not receive any anti-seizure medications (ASM) and 71 (78%) received one to five doses of benzodiazepines (BZD), without non-BZD ASM. We provided the prehospital treatment flow path of these patients through caregivers and EMS including relevant time points. Patients with a history of SE were more likely to receive the first BZD in the prehospital setting compared to patients without a history of SE (adjusted HR 3.25, 95% CI 1.72–6.12, p<0.001). ConclusionIn this multicenter study of pediatric RCSE, prehospital treatment may be streamlined further. Patients with a history of SE were more likely to receive prehospital rescue medication.

Management of status epilepticus in Malaysia: A national survey of current practice and treatment gap

IntroductionEarly and effective treatment is fundamental in status epilepticus (SE) management. At the initiative of the Epilepsy Council of Malaysia, this study aimed to determine the treatment gap in SE across different healthcare settings in Malaysia. MethodsA web-based survey was sent to clinicians involved in the management of SE, across all states and at all levels of healthcare services. ResultsA total of 158 responses were received from 104 health facilities, including 23 tertiary government hospitals (95.8% of all government tertiary hospitals in Malaysia), 4 (80.0%) universities, 14 (6.7%) private, 15 (11.5%) district hospitals and 21 clinics. Intravenous (IV) diazepam was available in 14 (93.3%) district and 33 (80.5%) tertiary hospitals for prehospital management. Non-IV benzodiazepine (rectal diazepam and intramuscular midazolam) was not widely available in prehospital services (75.8% and 51.5%). Intramuscular midazolam was underutilised (60.0% in district and 65.9% in tertiary hospitals). IV sodium valproate and levetiracetam were only available in 66.7% and 53.3% of the district hospitals, respectively. Electroencephalogram (EEG) services were available in only 26.7% of the district hospitals. Non-pharmacological therapies such as ketogenic diet, electroconvulsive therapy, and therapeutic hypothermia were not available in most district and tertiary hospitals for refractory and super-refractory SE. ConclusionsWe identified several gaps in the current practice of SE management, including limited availability and underutilization of non-IV midazolam in prehospital services, underutilization of non-IV midazolam and other second-line ASMs, and lack of EEG monitoring in district hospitals and limited treatment options for refractory and super-refractory SE in tertiary hospitals.

Evidence-based guideline on management of status epilepticus in adult intensive care unit in resource-limited settings: a review article.

This review was presented under the Protocol for Systematic Reviews and Meta-Analyses (PRISMA). A literature search was performed in PubMed, Google Scholar, Cochrane, and Medline databases from 2007 to 2021. The keywords for the literature search were (SE or controlled clinical trial) AND (SE or randomized controlled trial), (SE or multicenter trial) AND (SE or meta-analysis) AND (SE or crossover study). SE is an urgent medical emergency that requires early recognition and aggressive treatment. Medical treatment is initiated when seizures continue for more than 5min after all stabilization measures have been taken. Based on the available evidence, diazepam can be used as a substitute for lorazepam in the treatment of SE. Ketamine is effective when given before other anesthetics as a third-line treatment in refractory and very refractory epilepsy. Propofol reduced the number of days of mechanical ventilation in the treatment of SE and has better seizure control than thiopental. Music has been recommended as an adjunctive therapy for epilepsy medication.

Management of status epilepticus in childhood: a survey conducted at pediatric hospitals in the City of Buenos Aires.

Introduction. Status epilepticus is the most common neurological emergency. Although mortality in children is low, morbidity may exceed 20%. Objective. To evaluate the management of status epilepticus by pediatricians who usually treat this condition. Population and methods. Descriptive, cross-sectional study based on a survey administered to physicians from 3 pediatric hospitals in the City of Buenos Aires. Results. A total of 292 surveys were administered (complete response rate as high as 86%); 77% were administered to pediatricians and 16% to intensive care specialists. Forty-seven percent of the participants reported that they administer the first dose of a benzodiazepine within the correct timeframe; 56% use intrarectal diazepam when intravenous access is not available; 95% choose lorazepam as the initial benzodiazepine if an intravenous line is available; 58% initiate the administration of a second-line drug within the correct timeframe; 84% administer phenytoin as the first-choice, second-line drug; and 33% do not measure treatment time. Overall adherence to international recommendations was 17%. Conclusions. Our study highlights poor adherence of pediatricians to international guidelines, particularly in time-dependent decisions. Greater heterogeneity was observed in treatment approaches as the treatment algorithm progressed.

Clinical Features and Management of Status Epilepticus in the Pediatric Intensive Care Unit.

Status epilepticus (SE) is associated with significant morbidity and mortality in children. SE in the pediatric intensive care unit (PICU) are not well characterized. The aim of this study is to retrospectively investigate the clinical features and treatment of seizures in children admitted to the PICU of our hospital. We retrospectively examined the clinical characteristics of patients aged between 1 month and 18 years who were admitted to our hospital with SE or who were diagnosed with SE after hospitalization and were followed up with continuous electroencephalographic monitoring between January 2015 and December 2019. A total of 88 patients with SE, 50 (56.8%) boys and 38 (43.2%) girls, were included. The median age was 24 months (interquartile range, 12-80 months). When we evaluate the continuous electroencephalographic monitoring data, 27 (30.7%) were lateralized, 20 (22.7%) were multifocal, 30 (34.1%) were generalized, and 11 (12.5%) were bilateral independent epileptic activity. Seventy nine patients (89.8%) were evaluated as convulsive status epilepticus (CSE) and 9 (10.2%) as nonconvulsive status epilepticus (NCSE). Pediatric Risk of Mortality (PRISM III) score and mortality of patients with NCSE were higher ( P = 0.004 and P = 0.046, respectively). Thirteen eight patients (43.1%) were diagnosed as SE, 38 patients (43.1%) as refractory SE, and 12 patients (13.6%) as super-refractory SE. The overall mortality rate was 10.2%. Status epilepticus is a neurological emergency that causes mortality and morbidity. Electroencephalographic monitoring is important for the recognition of seizures and rapid intervention. No superiority of second-line treatments or combined treatments was demonstrated in patients with SE.

Rates of Status Epilepticus and Sudden Unexplained Death in Epilepsy in People With Genetic Developmental and Epileptic Encephalopathies.

The genetic developmental and epileptic encephalopathies (DEEs) comprise a large group of severe epilepsy syndromes, with a wide phenotypic spectrum. Currently, the rates of convulsive status epilepticus (CSE), nonconvulsive status epilepticus (NCSE), and sudden unexplained death in epilepsy (SUDEP) in these diseases are not well understood. We aimed to describe the proportions of patients with frequently observed genetic DEEs who developed CSE, NCSE, mortality, and SUDEP. Understanding the risks of these serious presentations in each genetic DEE will enable earlier diagnosis and appropriate management. In this retrospective analysis of patients with a genetic DEE, we estimated the proportions with CSE, NCSE, and SUDEP and the overall and SUDEP-specific mortality rates for each genetic diagnosis. We included patients with a pathogenic variant in the genes SCN1A, SCN2A, SCN8A, SYNGAP1, NEXMIF, CHD2, PCDH19, STXBP1, GRIN2A, KCNT1, and KCNQ2 and with Angelman syndrome (AS). The cohort comprised 510 individuals with a genetic DEE, in whom we observed CSE in 47% and NCSE in 19%. The highest proportion of CSE occurred in patients with SCN1A-associated DEEs, including 181/203 (89%; 95% CI 84-93) patients with Dravet syndrome and 8/15 (53%; 95% CI 27-79) non-Dravet SCN1A-DEEs. CSE was also notable in patients with pathogenic variants in KCNT1 (6/10; 60%; 95% CI 26-88) and SCN2A (8/15; 53%; 95% CI 27-79). NCSE was common in patients with non-Dravet SCN1A-DEEs (8/15; 53%; 95% CI 27-79) and was notable in patients with CHD2-DEEs (6/14; 43%; 95% CI 18-71) and AS (6/19; 32%; 95% CI 13-57). There were 42/510 (8%) deaths among the cohort, producing a mortality rate of 6.1 per 1,000 person-years (95% CI 4.4-8.3). Cases of SUDEP accounted for 19/42 (48%) deaths. Four genes were associated with SUDEP: SCN1A, SCN2A, SCN8A, and STXBP1. The estimated SUDEP rate was 2.8 per 1,000 person-years (95% CI 1.6-4.3). We showed that proportions of patients with CSE, NCSE, and SUDEP differ for commonly encountered genetic DEEs. The estimates for each genetic DEE studied will inform early diagnosis and management of status epilepticus and SUDEP and inform disease-specific counseling for patients and families in this high-risk group of conditions.