Population-based study of rare epilepsy incidence in a US urban population.

OBJECTIVE: This study was undertaken to estimate incidence of rare epilepsies and compare with literature. METHODS: We used electronic health record text search to identify children with 28 rare epilepsies in New York City (2010-2014). We estimated cumulative incidence and compared with literature. RESULTS: Eight of 28 rare epilepsies had five or more prior estimates, and our measurements were within the published range for all. The most common were infantile epileptic spasms syndrome (1 in 2920 live births), Lennox-Gastaut syndrome (1 in 9690), and seizures associated with tuberous sclerosis complex (1 in 14 300). Fifteen of 28 had fewer than five prior estimates, and of these, we provided additional estimates for early infantile developmental and epileptic encephalopathy (1 in 32 700), epilepsy with myoclonic-atonic seizures (1 in 34 100), Sturge-Weber syndrome plus seizures/epilepsy (1 in 40 900), epilepsy in infancy with migrating focal seizures (1 in 54 500), Aicardi syndrome plus seizures/epilepsy (1 in 71 600), hypothalamic hamartoma with seizures (1 in 225 000), and Rasmussen syndrome (1 in 450 000). Five of 28 rare epilepsies had no prior estimates, and of these, we provided a new estimate for developmental/epileptic encephalopathy with spike-and-wave activation in sleep and/or continuous spikes and waves during sleep (1 in 34 100). Data were limited for the remaining 12 rare epilepsies, which were all genetic epilepsies, including PCDH19, CDKL5, Alpers disease, SCN8A, KCNQ2, SCN2A, GLUT1 deficiency, Phelan-McDermid syndrome, myoclonic epilepsy with ragged-red fibers, dup15q syndrome, ring chromosome 14, and ring chromosome 20. SIGNIFICANCE: We estimated the incidence of rare epilepsies using population-based electronic health record data and literature review. More research is needed to better estimate the incidence of genetic epilepsies with nonspecific clinical features. Electronic health records may be a valuable data source for studying rare epilepsies and other rare diseases, particularly as genetic testing becomes more widely adopted.

A multi-center comparison of surgical techniques for corpus Callosotomy in pediatric drug-resistant epilepsy.

OBJECTIVES: Corpus callosotomy (CC) is used to reduce seizures, primarily in patients with generalized drug-resistant epilepsy (DRE). The invasive nature of the procedure contributes to underutilization despite its potential superiority to other palliative procedures. The goal of this study was to use a multi-institutional epilepsy surgery database to characterize the use of CC across participating centers. METHODS: Data were acquired from the Pediatric Epilepsy Research Consortium (PERC) Surgery Database, a prospective observational study collecting data on children 0-18 years referred for surgical evaluation of DRE across 22 U.S. pediatric epilepsy centers. Patient, epilepsy, and surgical characteristics were collected across multiple CC modalities. Outcomes and complications were recorded and analyzed statistically. RESULTS: Eighty-three patients undergoing 85 CC procedures at 14 participating epilepsy centers met inclusion criteria. Mean age at seizure onset was 2.3 years (0-9.4); mean age for Phase I evaluation and surgical intervention were 9.45 (.1-20) and 10.46 (.2-20.6) years, respectively. Generalized seizure types were the most common (59%). Complete CC was performed in 88%. The majority of CC procedures (57%) were via open craniotomy, followed by laser interstitial thermal therapy (LiTT) (20%) and mini-craniotomy/endoscopic (mc/e) (22%). Mean operative times were significantly longer for LiTT, whereas mean estimated blood loss was greater in open cases. Complications occurred in 11 cases (13%) and differed significantly between surgical techniques (p < .001). There was no statistically significant difference in length of postoperative stay across approaches. Mean follow-up was 12.8 months (range 1-39). Favorable Engel outcomes were experienced by 37 (78.7%) of the patients who underwent craniotomy, 10 (58.8%) with LiTT, and 12 (63.2%) with mc/e; these differences were not statistically significant. SIGNIFICANCE: CC is an effective surgical modality for children with DRE. Regardless of surgical modality, complication rates are acceptable and seizure outcomes generally favorable. Newer, less-invasive, surgical approaches may lead to increased adoption of this efficacious therapeutic option for pediatric DRE.

Inequities in Therapy for Infantile Spasms: A Call to Action.

OBJECTIVE: The aim of this study was to determine whether selection of treatment for children with infantile spasms (IS) varies by race/ethnicity. METHODS: The prospective US National Infantile Spasms Consortium database includes children with IS treated from 2012 to 2018. We examined the relationship between race/ethnicity and receipt of standard IS therapy (prednisolone, adrenocorticotropic hormone, vigabatrin), adjusting for demographic and clinical variables using logistic regression. Our primary outcome was treatment course, which considered therapy prescribed for the first and, when needed, the second IS treatment together. RESULTS: Of 555 children, 324 (58%) were non-Hispanic white, 55 (10%) non-Hispanic Black, 24 (4%) non-Hispanic Asian, 80 (14%) Hispanic, and 72 (13%) other/unknown. Most (398, 72%) received a standard treatment course. Insurance type, geographic location, history of prematurity, prior seizures, developmental delay or regression, abnormal head circumference, hypsarrhythmia, and IS etiologies were associated with standard therapy. In adjusted models, non-Hispanic Black children had lower odds of receiving a standard treatment course compared with non-Hispanic white children (odds ratio [OR], 0.42; 95% confidence interval [CI], 0.20-0.89; p = 0.02). Adjusted models also showed that children with public (vs. private) insurance had lower odds of receiving standard therapy for treatment 1 (OR, 0.42; CI, 0.21-0.84; p = 0.01). INTERPRETATION: Non-Hispanic Black children were more often treated with non-standard IS therapies than non-Hispanic white children. Likewise, children with public (vs. private) insurance were less likely to receive standard therapies. Investigating drivers of inequities, and understanding the impact of racism on treatment decisions, are critical next steps to improve care for patients with IS. ANN NEUROL 2022;92:32-44.

Creating rare epilepsy cohorts using keyword search in electronic health records.

OBJECTIVE: Administrative codes to identify people with rare epilepsies in electronic health records are limited. The current study evaluated the use of keyword search as an alternative method for rare epilepsy cohort creation using electronic health records data. METHODS: Data included clinical notes from encounters with International Classification of Diseases, Ninth Revision (ICD-9) codes for seizures, epilepsy, and/or convulsions during 2010-2014, across six health care systems in New York City. We identified cases with rare epilepsies by searching clinical notes for keywords associated with 33 rare epilepsies. We validated cases via manual chart review. We compared the performance of keyword search to manual chart review using positive predictive value (PPV), sensitivity, and F-score. We selected an initial combination of keywords using the highest F-scores. RESULTS: Data included clinical notes from 77 924 cases with ICD-9 codes for seizures, epilepsy, and/or convulsions. The all-keyword search method identified 6095 candidates, and manual chart review confirmed that 2068 (34%) had a rare epilepsy. The initial combination method identified 1862 cases with a rare epilepsy, and this method performed as follows: PPV median = .64 (interquartile range [IQR] = .50-.81, range = .20-1.00), sensitivity median = .93 (IQR = .76-1.00, range = .10-1.00), and F-score median = .71 (IQR = .63-.85, range = .18-1.00). Using this method, we identified four cohorts of rare epilepsies with over 100 individuals, including infantile spasms, Lennox-Gastaut syndrome, Rett syndrome, and tuberous sclerosis complex. We identified over 50 individuals with two rare epilepsies that do not have specific ICD-10 codes for cohort creation (epilepsy with myoclonic atonic seizures, Sturge-Weber syndrome). SIGNIFICANCE: Keyword search is an effective method for cohort creation. These findings can improve identification and surveillance of individuals with rare epilepsies and promote their referral to specialty clinics, clinical research, and support groups.

COVID-19 is Observed in Older Children During the Omicron Wave in New York City.

BACKGROUND: The Omicron variant of SARS-CoV-2 has a predilection for the upper airways, causing symptoms such as sore throat, hoarse voice, and stridor. OBJECTIVE: We describe a series of children with COVID-19-associated croup in an urban multicenter hospital system. METHODS: We conducted a cross-sectional study of children ≤18 years of age presenting to the emergency department during the COVID-19 pandemic. Data were extracted from an institutional data repository comprised of all patients who were tested for SARS-CoV-2. We included patients with a croup diagnosis by International Classification of Diseases, 10th revision code and a positive SARS-CoV-2 test within 3 days of presentation. We compared demographics, clinical characteristics, and outcomes for patients presenting during a pre-Omicron period (March 1, 2020-December 1, 2021) to the Omicron wave (December 2, 2021-February 15, 2022). RESULTS: We identified 67 children with croup, 10 (15%) pre-Omicron and 57 (85%) during the Omicron wave. The prevalence of croup among SARS-CoV-2-positive children increased by a factor of 5.8 (95% confidence interval 3.0-11.4) during the Omicron wave compared to prior. More patients were ≥6 years of age in the Omicron wave than prior (19% vs. 0%). The majority were not hospitalized (77%). More patients ≥6 years of age received epinephrine therapy for croup during the Omicron wave (73% vs. 35%). Most patients ≥6 years of age had no croup history (64%) and only 45% were vaccinated against SARS-CoV-2. CONCLUSION: Croup was prevalent during the Omicron wave, atypically affecting patients ≥6 years of age. COVID-19-associated croup should be added to the differential diagnosis of children with stridor, regardless of age. © 2022 Elsevier Inc.

Pediatric Epilepsy Learning Healthcare System Quality of Life (PELHS-QOL-2): A novel health-related quality of life prompt for children with epilepsy.

OBJECTIVE: Pediatric epilepsy is often associated with diminished health-related quality of life (HRQOL). Our aim was to establish the validity of the Pediatric Epilepsy Learning Healthcare System Quality of Life (PELHS-QOL-2) questions, a novel two-item HRQOL prompt for children with epilepsy, primarily for use in clinical care. METHODS: We performed a multicenter cross-sectional study to validate the PELHS-QOL-2. Construct validity was established through bivariate comparisons with four comparator measures and known drivers of quality of life in children with epilepsy, as well as by creating an a priori multivariable model to predict the Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-55). Validity generalization was established through bivariate comparisons with demographic and clinical information. Content validity and clinical utility were established by assessing how well the PELHS-QOL-2 met eight design criteria for an HRQOL prompt established by a multistakeholder group of experts. RESULTS: The final participant sample included 154 English-speaking caregivers of children with epilepsy (mean age = 9.7 years, range = .5-18, 49% female, 70% White). The PELHS-QOL-2 correlated with the four comparator instruments (ρ = .44-.56), was significantly associated with several known drivers of quality of life in children with epilepsy (p < .05), and predicted QOLCE-55 scores in the multivariate model (adjusted R2 = .54). The PELHS-QOL-2 item was not associated with the age, sex, and ethnicity of the children nor with the setting and location of data collection, although PELHS-QOL-Medications was significantly associated with race (worse for White race). Following both quantitative and qualitative analysis, the PELHS-QOL-2 met seven of eight design criteria. SIGNIFICANCE: The PELHS-QOL-2 is a valid HRQOL prompt and is well suited for use in clinical care as a mechanism to routinely initiate conversations with caregivers about quality of life in children with epilepsy. The association of PELHS-QOL-Medications with race merits further study.

Surgical evaluation in children <3 years of age with drug-resistant epilepsy: Patient characteristics, diagnostic utilization, and potential for treatment delays.

OBJECTIVE: Drug-resistant epilepsy (DRE) occurs at higher rates in children <3 years old. Epilepsy surgery is effective, but rarely utilized in young children despite developmental benefits of early seizure freedom. The present study aims to identify unique patient characteristics and evaluation strategies in children <3 years old who undergo epilepsy surgery evaluation as a means to assess contributors and potential solutions to health care disparities in this group. METHODS: The Pediatric Epilepsy Research Consortium Epilepsy Surgery Database, a multicentered, cross-sectional collaboration of 21 US pediatric epilepsy centers, collects prospective data on children <18 years of age referred for epilepsy surgery evaluation. We compared patient characteristics, diagnostic utilization, and surgical treatment between children <3 years old and those older undergoing initial presurgical evaluation. We evaluated patient characteristics leading to delayed referral (>1 year) after DRE diagnosis in the very young. RESULTS: The cohort included 437 children, of whom 71 (16%) were <3 years of age at referral. Children evaluated before the age of 3 years more commonly had abnormal neurological examinations (p = .002) and daily seizures (p = .001). At least one ancillary test was used in 44% of evaluations. Fifty-nine percent were seizure-free following surgery (n = 34), with 35% undergoing limited focal resections. Children with delayed referrals more often had focal aware (p < .001) seizures and recommendation for palliative surgeries (p < .001). SIGNIFICANCE: There are relatively few studies of epilepsy surgery in the very young. Surgery is effective, but may be disproportionally offered to those with severe presentations. Relatively low utilization of ancillary testing may contribute to reduced surgical therapy for those without evident lesions on magnetic resonance imaging. Despite this, a sizeable portion of patients have favorable outcome after focal epilepsy surgery resections.

Automated identification and quality measurement for pediatric convulsive status epilepticus.

OBJECTIVE: Treatment delays for refractory convulsive status epilepticus (RCSE) are associated with worse outcomes. In the United States, treatment for pediatric RCSE is slower than guidelines recommend. To address this gap, the American Academy of Neurology and Child Neurology Society (AAN/CNS) developed a quality measure: the percentage of RCSE patients that receive third-line treatment within 60 minutes. We aimed to develop computable phenotypes for convulsive status epilepticus (CSE) and RCSE to automate calculation of the quality measure. METHODS: From an observational cohort of children presenting to the emergency department for seizures or epilepsy, we identified presentations of RCSE and its precursors: CSE and benzodiazepine-resistant status epilepticus (BRSE). These served as a gold standard for computable phenotype development. Using multivariate analyses, we constructed and evaluated statistical models for case identification. We then evaluated adherence to the AAN/CNS RCSE quality measure. RESULTS: From 664 charts, we identified 56 patients with CSE, 36 with BRSE, and 18 with RCSE. Four predictors were used: International Classification of Diseases (ICD) codes, and receiving first-, second-, or third-line agents shortly after presentation to the emergency department (ED). Combinations of these predictors identified CSE with 84% sensitivity and 81% positive predictive value (PPV), BRSE with 67% sensitivity and 89% PPV, and RCSE with 94% sensitivity and 85% PPV. Median (interquartile range [IQR]) time to treatment for first-line agent was 13 (5-27) minutes for CSE, second-line for BRSE was 24 (9.5-43.5) minutes, and third-line for RCSE was 52 (27-87) minutes. Sixty percent of RCSE patients received a third-line agent within 60 minutes of ED arrival. SIGNIFICANCE: RCSE and its precursors can be identified automatically with high fidelity allowing automated calculation of time to treatment and the RCSE quality measure. This has the potential to facilitate quality improvement work and improve care for RCSE.

Design and implementation of electronic health record common data elements for pediatric epilepsy: Foundations for a learning health care system.

OBJECTIVE: Common data elements (CDEs) are standardized questions and answer choices that allow aggregation, analysis, and comparison of observations from multiple sources. Clinical CDEs are foundational for learning health care systems, a data-driven approach to health care focused on continuous improvement of outcomes. We aimed to create clinical CDEs for pediatric epilepsy. METHODS: A multiple stakeholder group (clinicians, researchers, parents, caregivers, advocates, and electronic health record [EHR] vendors) developed clinical CDEs for routine care of children with epilepsy. Initial drafts drew from clinical epilepsy note templates, CDEs created for clinical research, items in existing registries, consensus documents and guidelines, quality metrics, and outcomes needed for demonstration projects. The CDEs were refined through discussion and field testing. We describe the development process, rationale for CDE selection, findings from piloting, and the CDEs themselves. We also describe early implementation, including experience with EHR systems and compatibility with the International League Against Epilepsy classification of seizure types. RESULTS: Common data elements were drafted in August 2017 and finalized in January 2020. Prioritized outcomes included seizure control, seizure freedom, American Academy of Neurology quality measures, presence of common comorbidities, and quality of life. The CDEs were piloted at 224 visits at 10 centers. The final CDEs included 36 questions in nine sections (number of questions): diagnosis (1), seizure frequency (9), quality of life (2), epilepsy history (6), etiology (8), comorbidities (2), treatment (2), process measures (5), and longitudinal history notes (1). Seizures are categorized as generalized tonic-clonic (regardless of onset), motor, nonmotor, and epileptic spasms. Focality is collected as epilepsy type rather than seizure type. Seizure frequency is measured in nine levels (all used during piloting). The CDEs were implemented in three vendor systems. Early clinical adoption included 1294 encounters at one center. SIGNIFICANCE: We created, piloted, refined, finalized, and implemented a novel set of clinical CDEs for pediatric epilepsy.

Predictors of SUDEP counseling and implications for designing interventions.

OBJECTIVE: We aimed to describe how often and why clinicians counsel people with epilepsy about sudden unexpected death in epilepsy (SUDEP). Understanding counseling gaps can help design interventions. METHODS: We searched clinical notes of 77,924 patients from 2010 to 2014 from six hospitals to find examples of SUDEP counseling and seizure safety counseling. Visits were coded for patient, clinician, and visit factors, and documented reasons for counseling. We evaluated factors associated with SUDEP vs. seizure safety counseling, and reasons for counseling using bivariate and multivariable statistics. Reasons for counseling included: poor medication adherence, lifestyle factors (e.g., poor sleep, drinking alcohol), patient/family reluctance to make recommended medication adjustment, epilepsy surgery considerations, and patient education only. RESULTS: Analysis was restricted to two of six hospitals where 91% of counseling occurred. Documentation of SUDEP counseling was rare (332 of 33,821 patients, 1.0%), almost exclusively by epileptologists (98.5% of counseling), and stable over time, X2 (4, n = 996) = 3.81, p = 0.43. Adult neurologists were more likely to document SUDEP counseling than pediatric (OR = 1.65, 95% CI = 1.12-2.44). Most SUDEP counseling was documented with a goal of seizure reduction (214 of 332, 64.5%), though some was for patient education only (118 of 332, 35.5%). By the time SUDEP counseling was documented, the majority of patients had refractory epilepsy (187 of 332, 56.3%) and/or a potentially modifiable risk factor (214 of 332, 64.5%). Neurologists with more years of clinical experience (OR = 2.18, 95% CI = 1.12-4.25) and more senior academic titles (OR = 2.25, 95% CI = 1.27-3.99) were more likely to document SUDEP counseling for patient education only. People with ≥2 anti-seizure medications (ASM) were more likely to receive counseling for patient education (OR = 2.72, 95% CI = 1.49-4.97). CONCLUSIONS: Documentation of SUDEP is rare, and varies by clinician, hospital, and patient factors. Efforts to increase SUDEP counseling should focus on junior clinicians, and emphasize starting the conversation soon after onset of epilepsy.

Health and Financial Burdens Associated With Venous Thrombosis in Hospitalized Children With Inflammatory Bowel Disease.

ABSTRACT: Venous thromboembolism (VTE) is a known complication in children with inflammatory bowel disease (IBD). Despite awareness of the increased thrombosis risk in this population, prophylaxis is not standardly used and there is limited published guidance for thrombosis prevention. To better appreciate the impact of thrombosis in this population, we compared children with IBD who did or did not have a VTE, using the Pediatric Health Information System inpatient database from 2009 to 2017. In hospitalized children with IBD, VTE was associated with longer median hospital stays (11 vs 5 days), need for intensive care unit admission (30.2% vs 4.8%), higher median adjusted costs ($32.8k vs $12.3k) and hospital charges ($96.6k vs $36k), and in-hospital death (1.5% vs 0.2%) (P  < 0.001 in all comparisons). These findings highlight the need to determine and implement appropriate strategies to reduce VTE rates in children with IBD, given its association with high morbidity, mortality, and cost.

Early Use of Antiseizure Medication in Mechanically Ventilated Traumatic Brain Injury Cases: A Retrospective Pediatric Health Information System Database Study.

OBJECTIVES: Traumatic brain injury is a leading cause of morbidity and mortality in children. Post-traumatic seizures occur in 25% of children with severe traumatic brain injury and may worsen outcomes. Our objective was to use a retrospective cohort study to examine the association between the early seizure occurrence and the choice of early antiseizure medication in children with traumatic brain injury. DESIGN: Retrospective cohort study using the Pediatric Health Information Systems database, 2010-2017. SETTING: Fifty-one U.S. children's hospitals. PATIENTS: Children (< 18 yr old at admission) with diagnostic codes for traumatic brain injury who were mechanically ventilated at the time of admission and with hospital length of stay greater than 24 hours. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 3,479 children were identified via coding and including in the analysis. Patients receiving antiseizure medication starting day 0 with levetiracetam were compared with those receiving phenytoin. The outcome was seizure occurrence, identified using validated International Classification of Diseases, 9th Revision, Clinical Modification and International Classification of Diseases, 10th Revision, Clinical Modification diagnosis codes. The median (interquartile range) age of patients was 4 (1-11) years, and the most common mechanism of injury was motor vehicle accident, occurring in 960 of patients (27%). A total of 2,342 patients (67%) received levetiracetam on day 0 and 1,137 patients (33%) received phenytoin on day 0. Totally 875 patients (37%) receiving levetiracetam on day 0 developed seizures, compared with 471 patients (41%) receiving phenytoin on day 0 (p = 0.02). Upon multivariable analysis adjusting for age, injury by child abuse, subdural hemorrhage, ethnicity, and admission year, children receiving phenytoin on day 0 were 1.26 (95% CI, 1.07-1.48) times more likely to be associated with post-traumatic seizure occurrence, compared with children receiving levetiracetam on day 0 (p = 0.01). CONCLUSIONS: Early administration of levetiracetam was associated with less-frequent seizure occurrence than early administration of phenytoin in mechanically ventilated children with traumatic brain injury. Additional studies are necessary to determine if the association is causal or due to unmeasured confounders and/or selection bias.

Risk for infantile spasms after acute symptomatic neonatal seizures.

OBJECTIVE: Infantile spasms (IS) is a severe epilepsy in early childhood. Early treatment of IS provides the best chance of seizure remission and favorable developmental outcome. We aimed to develop a prediction rule to accurately predict which neonates with acute symptomatic seizures will develop IS. METHODS: We used data from the Neonatal Seizure Registry, a prospective, multicenter cohort of infants with acute symptomatic neonatal seizures born from July 2015 to March 2018. Neonates with acute symptomatic seizures who received clinical electroencephalography (EEG) and magnetic resonance imaging (MRI) and were younger than 2 years of age at the time of enrollment were included. We evaluated the association of neonatal EEG, MRI, and clinical factors with subsequent IS using bivariate analysis and best subsets logistic regression. We selected a final model through a consensus process that balanced statistical significance with clinical relevance. RESULTS: IS developed in 12 of 204 infants (6%). Multiple potential predictors were associated with IS, including Apgar scores, EEG features, seizure characteristics, MRI abnormalities, and clinical status at hospital discharge. The final model included three risk factors: (a) severely abnormal EEG or ≥3 days with seizures recorded on EEG, (b) deep gray or brainstem injury on MRI, and (c) abnormal tone on discharge exam. The stratified risk of IS was the following: no factors 0% (0/82, 95% confidence interval [CI] 0%-4%), one or two factors 4% (4/108, 95% CI 1%-9%), and all three factors 57% (8/14, 95% CI 29%-83%). SIGNIFICANCE: IS risk after acute symptomatic neonatal seizures can be stratified using commonly available clinical data. No child without risk factors, vs >50% of those with all three factors, developed IS. This risk prediction rule may be valuable for clinical counseling as well as for selecting participants for clinical trials to prevent post-neonatal epilepsy. This tailored approach may lead to earlier diagnosis and treatment and improve outcomes for a devastating early life epilepsy.

Depression and anxiety in children with epilepsy and other chronic health conditions: National estimates of prevalence and risk factors.

OBJECTIVE: This study estimates the national prevalence of depression and anxiety among children with epilepsy and determines which demographic variables and comorbidities increase the risk of these psychopathologies. We also compare the rates of depression and anxiety in pediatric epilepsy with those of other chronic health conditions in childhood. METHODS: We used the 2009-2010 National Survey of Children with Special Health Care Needs to identify children with epilepsy with and without depression and anxiety. We assessed demographic factors and comorbidities associated with depression and anxiety using weighted multivariable logistic regressions. The rates of psychiatric comorbidity in children with chronic conditions other than epilepsy were also determined. RESULTS: The final sample included 1042 children over the age of five with epilepsy. After applying the sampling weights, we estimated that 283,000 children between 5 and 17 years of age have epilepsy in the United States (U.S.). Among these children, 25% have depression and/or anxiety. This figure was not significantly different from the rates seen among children with asthma (16.5%) or allergies (21.6%) but was significantly lower than the rate seen among children with migraines (43.2%). In our analyses of children with epilepsy, low-income children (regardless of race) and children whose needs for specialist care were unmet (relative to those whose needs were met) were more likely to have depression. Low-income black children were less likely to have anxiety than high-income white children. Gender, age, and epilepsy severity were unrelated to depression or anxiety. CONCLUSIONS: One in four U.S. children with epilepsy has depression and/or anxiety. Therefore, physicians should consider the various factors that are related to depression and anxiety in children with epilepsy so that at-risk children can be screened and managed appropriately.

An accelerated shift in the use of remote systems in epilepsy due to the COVID-19 pandemic.

PURPOSE: The purpose of the study was to describe epileptologists' opinion on the increased use of remote systems implemented during the COVID-19 pandemic across clinics, education, and scientific meetings activities. METHODS: Between April and May 2020, we conducted a cross-sectional, electronic survey on remote systems use before and during the COVID-19 pandemic through the European reference center for rare and complex epilepsies (EpiCARE) network, the International and the French Leagues Against Epilepsy, and the International and the French Child Neurology Associations. After descriptive statistical analysis, we compared the results of France, China, and Italy. RESULTS: One hundred and seventy-two respondents from 35 countries completed the survey. Prior to the COVID-19 pandemic, 63.4% had experienced remote systems for clinical care. During the pandemic, the use of remote clinics, either institutional or personal, significantly increased (p < 10-4). Eighty-three percent used remote systems with video, either institutional (75%) or personal (25%). During the pandemic, 84.6% of respondents involved in academic activities transformed their courses to online teaching. From February to July 2020, few scientific meetings relevant to epileptologists and routinely attended was adapted to virtual meeting (median: 1 [25th-75th percentile: 0-2]). Responders were quite satisfied with remote systems in all three activity domains. Interestingly, before the COVID-19 pandemic, remote systems were significantly more frequently used in China for clinical activity compared with France or Italy. This difference became less marked during the pandemic. CONCLUSION: The COVID-19 pandemic has dramatically altered how academic epileptologists carry out their core missions of clinical care, medical education, and scientific discovery and dissemination. Close attention to the impact of these changes is merited.

Automated detection of sudden unexpected death in epilepsy risk factors in electronic medical records using natural language processing.

OBJECTIVE: Sudden unexpected death in epilepsy (SUDEP) is an important cause of mortality in epilepsy. However, there is a gap in how often providers counsel patients about SUDEP. One potential solution is to electronically prompt clinicians to provide counseling via automated detection of risk factors in electronic medical records (EMRs). We evaluated (1) the feasibility and generalizability of using regular expressions to identify risk factors in EMRs and (2) barriers to generalizability. METHODS: Data included physician notes for 3000 patients from one medical center (home) and 1000 from five additional centers (away). Through chart review, we identified three SUDEP risk factors: (1) generalized tonic-clonic seizures, (2) refractory epilepsy, and (3) epilepsy surgery candidacy. Regular expressions of risk factors were manually created with home training data, and performance was evaluated with home test and away test data. Performance was evaluated by sensitivity, positive predictive value, and F-measure. Generalizability was defined as an absolute decrease in performance by <0.10 for away versus home test data. To evaluate underlying barriers to generalizability, we identified causes of errors seen more often in away data than home data. To demonstrate how small revisions can improve generalizability, we removed three "boilerplate" standard text phrases from away notes and repeated performance. RESULTS: We observed high performance in home test data (F-measure range = 0.86-0.90), and low to high performance in away test data (F-measure range = 0.53-0.81). After removing three boilerplate phrases, away performance improved (F-measure range = 0.79-0.89) and generalizability was achieved for nearly all measures. The only significant barrier to generalizability was use of boilerplate phrases, causing 104 of 171 errors (61%) in away data. SIGNIFICANCE: Regular expressions are a feasible and probably a generalizable method to identify variables related to SUDEP risk. Our methods may be implemented to create large patient cohorts for research and to generate electronic prompts for SUDEP counseling.

Expanding Health Information Exchange Improves Identification of Frequent Emergency Department Users.

STUDY OBJECTIVE: Frequent emergency department (ED) users are of interest to policymakers and hospitals. The objective of this study is to examine the effect of health information exchange size on the identification of frequent ED users. METHODS: We retrospectively analyzed data from Healthix, a health information exchange in New York that previously included 10 hospitals and then grew to 31 hospitals. We divided patients into 3 cohorts: high-frequency ED users with 4 or more visits in any 30-day period, medium-frequency ED users with 4 or more visits in any year, and infrequent ED users with fewer than 4 visits in any year. For both the smaller (10-hospital) and larger (31-hospital) health information exchanges, we compared the identification rate of frequent ED users that was based on hospital-specific data with the corresponding rates that were based on health information exchange data. RESULTS: The smaller health information exchange (n=1,696,279 unique ED patients) identified 11.4% more high-frequency users (33,467 versus 30,057) and 9.5% more medium-frequency users (109,497 versus 100,014) than the hospital-specific data. The larger health information exchange (n=3,684,999) identified 19.6% more high-frequency patients (52,727 versus 44,079) and 18.2% more medium-frequency patients (222,574 versus 192,541) than the hospital-specific data. Expanding from the smaller health information exchange to the larger one, we found an absolute increase of 8.2% and 8.7% identified high- and medium-frequency users, respectively. CONCLUSION: Increasing health information exchange size more accurately reflects how patients access EDs and ultimately improves not only the total number of identified frequent ED users but also their identification rate.

The evaluation and costs of transition programs for youth with epilepsy.

There is limited information about the effectiveness of transition programs for youth moving from pediatric to adult care with any chronic disease. Two Delphi studies and National Institute for Health and Care Excellence (NICE) guidelines about transition for epilepsy have suggested few critical outcome measures for transition. A single large prospective study found that the most important transition program elements were appropriate parent involvement, promotion of health self-efficacy, and meeting the adult team before transfer. Two Cochrane reviews of the value of transition for epilepsy found insufficient evidence to establish or refute the value of various programs, although evaluation of a few programs suggested a great deal of family/patient satisfaction. The cost of transition programs and their cost effectiveness have also not been established except for renal transplantation where transition programs were associated with fewer losses of the transplanted kidneys, a cost-effective outcome. Published data on the overall cost of care for children and adults with epilepsy may be helpful to establish a business plan for a transition program, and are briefly reviewed. Establishing cost effectiveness of transition programs for epilepsy would promote their establishment and viability. However, a number of studies will be needed based on the nature of the program, the healthcare system where it is carried out, and the type of epilepsy. In fee-for-service health systems, the reevaluation of patients with epilepsy prior to transfer may be sufficient to cover the costs of the transition program, whereas in single payer systems, there may be positive downstream health or societal benefits that justify the costs. A theoretical framework for comprehensive evaluation of epilepsy transition programs is needed. The Triple Aim Framework seems applicable with focus on population health, patient experiences, and cost and has the potential to assess transition interventions in the context of system-wide improvements in healthcare. Transition programs in general have not been well evaluated, and very little evaluation data exist regarding transition programs for epilepsy. We recommend more evaluative research using rigorous methodology to comprehensively assess these programs.

Immediate outcomes in early life epilepsy: A contemporary account.

RATIONALE: Early-life epilepsies (ELEs) include some of the most challenging forms of epilepsy to manage. Given recent diagnostic and therapeutic advances, a contemporary assessment of the immediate short-term outcomes can provide a valuable framework for identifying priorities and benchmarks for evaluating quality improvement efforts. METHODS: Children with newly diagnosed epilepsy and onset <3 years were prospectively recruited through 17 US hospitals, from 2012 to 2015 and followed for 1 year after diagnosis. Short-term outcome included mortality, drug resistance, evolution of nonsyndromic epilepsy to infantile spasms (IS) and from IS to other epilepsies, and developmental decline. Multivariable analyses assessed the risk of each outcome. RESULTS: Seven hundred seventy-five children were recruited, including 408 (53%) boys. Median age at onset was 7.5 months (interquartile range (IQR): 4.2-16.5), and 509 (66%) had onset in the first year of life. Of 22 deaths that occurred within one year of epilepsy diagnosis, 21 were children with epilepsy onset in infancy (<12 months). Of 680 children followed ≥6 months, 239 (35%) developed drug-resistant seizures; 34/227 (15%) infants with nonsyndromic epilepsy developed IS, and 48/210 (23%) initially presenting with IS developed additional seizure types. One hundred of 435 (23%) with initially typical development or only mild/equivocal delays at seizure onset, had clear developmental impairment within one year after initial diagnosis. Each outcome had a different set of predictors; however, younger age and impaired development at seizure onset were broadly indicative of poorer outcomes. Type of epilepsy and early identification of underlying cause were not reliable predictors of these outcomes. CONCLUSION: Early-life epilepsies carry a high risk of poor outcome which is evident shortly after epilepsy diagnosis. Onset in infancy and developmental delay is associated with an especially high risk, regardless of epilepsy type. The likelihood of poor outcomes is worrisome regardless of specific clinical profiles.

Do Children with Special Health Care Needs with Anxiety have Unmet Health Care Needs? An Analysis of a National Survey.

OBJECTIVE: To describe differences in health care needs between Children with Special Health Care Needs (CSHCN) with and without anxiety and examine the association between anxiety and unmet health care needs. METHODS: We analyzed data from the 2009/2010 national survey of CSHCN. The independent variable was anxiety. The main outcomes were health care needs and unmet needs. Covariates included demographics, other co-morbid conditions, and the presence and quality of a medical home. We used bivariate analyses and multivariable logistic regression to assess the relationships among anxiety, covariates, and the outcomes. We stratified our analysis by age (6-11 years, 12-17 years). Propensity score matched paired analysis was used as a sensitivity analysis. RESULTS: Our final sample included 14,713 6-11 year-olds and 15,842 12-17-year-olds. Anxiety was present in 16% of 6-11 year-olds and 23% or 12-17 year-olds. In bivariate analyses, CSHCN with anxiety had increased health care needs and unmet needs, compared to CSHCN without anxiety. In multivariable analyses, only children 12-17 years old with anxiety had increased odds of having an unmet health care need compared to those children without anxiety (OR 1.44 [95% CI 1.17-1.78]). This was confirmed in the propensity score matching analysis (OR 1.12, [95% CI 1.02-1.22]). The specific unmet needs for older CSHCN with anxiety were mental health care (OR 1.54 [95% CI 1.09-2.17]) and well child checkups (OR 2.01 [95% CI 1.18-3.44]). CONCLUSION: Better integration of the care for mental and physical health is needed to ensure CSHCN with anxiety have all of their health care needs met.