Guidelines for Specialized Epilepsy Centers: Executive Summary of the Report of the National Association of Epilepsy Centers Guideline Panel.

The National Association of Epilepsy Centers first published the guidelines for epilepsy centers in 1990, which were last updated in 2010. Since that update, epilepsy care and the science of guideline development have advanced significantly, including the importance of incorporating a diversity of stakeholder perspectives such as those of patients and their caregivers. Currently, despite extensive published data examining the efficacy of treatments and diagnostic testing for epilepsy, there remain significant gaps in data identifying the essential services needed for a comprehensive epilepsy center and the optimal manner for their delivery. The trustworthy consensus-based statements (TCBS) process produces unbiased, scientifically valid guidelines through a transparent process that incorporates available evidence and expert opinion. A systematic literature search returned 5937 relevant studies from which 197 articles were retained for data extraction. A panel of 41 stakeholders with diverse expertise evaluated this evidence and drafted recommendations following the TCBS process. The panel reached consensus on 52 recommendations covering services provided by specialized epilepsy centers in both the inpatient and outpatient settings in major topic areas including epilepsy monitoring unit care, surgery, neuroimaging, neuropsychology, genetics, and outpatient care. Recommendations were informed by the evidence review and reflect the consensus of a broad panel of expert opinions.

Impact of the COVID-19 Pandemic on Epilepsy Center Practice in the United States.

BACKGROUND AND OBJECTIVES: Persons with epilepsy, especially those with drug resistant epilepsy (DRE), may benefit from inpatient services such as admission to the epilepsy monitoring unit (EMU) and epilepsy surgery. The COVID-19 pandemic caused reductions in these services within the US during 2020. This article highlights changes in resources, admissions, and procedures among epilepsy centers accredited by the National Association of Epilepsy Centers (NAEC). METHODS: We compared data reported in 2019, prior to the COVID-19 pandemic, and 2020 from all 260 level 3 and level 4 NAEC accredited epilepsy centers. Data were described using frequency for categorical variables and median for continuous variables and were analyzed by center level, center population category, and geographical location. Qualitative responses from center directors to questions regarding the impact from COVID-19 were summarized utilizing thematic analysis. Responses from the NAEC center annual reports as well as a supplemental COVID-19 survey were included. RESULTS: EMU admissions declined 23% (-21,515) in 2020, with largest median reductions in level 3 centers [-55 admissions (-44%)] and adult centers [-57 admissions (-39%)]. The drop in admissions was more substantial in the East North Central, East South Central, Mid Atlantic, and New England US Census divisions. Survey respondents attributed reduced admissions to re-assigning EMU beds, restrictions on elective admissions, reduced staffing, and patient reluctance for elective admission. Treatment surgeries declined by 371 cases (5.7%), with the largest reduction occurring in VNS implantations [-486 cases (-19%)] and temporal lobectomies [-227 cases (-16%)]. All other procedure volumes increased, including a 35% (54 cases) increase in corpus callosotomies. DISCUSSION: In the US, access to care for persons with epilepsy declined during the COVID-19 pandemic in 2020. Adult patients, those relying on level 3 centers for care, and many persons in the eastern half of the US were most affected.

United States Epilepsy Center Characteristics: A Data Analysis From the National Association of Epilepsy Centers.

BACKGROUND AND OBJECTIVES: Patients with drug-resistant epilepsy (DRE) may benefit from specialized testing and treatments to better control seizures and improve quality of life. Most evaluations and procedures for DRE in the United States are performed at epilepsy centers accredited by the National Association of Epilepsy Centers (NAEC). On an annual basis, the NAEC collects data from accredited epilepsy centers on hospital-based epilepsy monitoring unit (EMU) size and admissions, diagnostic testing, surgeries, and other services. This article highlights trends in epilepsy center services from 2012 through 2019. METHODS: We analyzed data reported in 2012, 2016, and 2019 from all level 3 and level 4 NAEC accredited epilepsy centers. Data were described using frequency for categorical variables and median for continuous variables and were analyzed by center level and center population category. EMU beds, EMU admissions, epileptologists, and aggregate procedure volumes were also described using rates per population per year. RESULTS: During the period studied, the number of NAEC accredited centers increased from 161 to 256, with the largest increases in adult- and pediatric-only centers. Growth in EMU admissions (41%), EMU beds (26%), and epileptologists (109%) per population occurred. Access to specialized testing and services broadly expanded. The largest growth in procedure volumes occurred in laser interstitial thermal therapy (LiTT) (61%), responsive neurostimulation (RNS) implantations (114%), and intracranial monitoring without resection (152%) over the study period. Corpus callosotomies and vagus nerve stimulator (VNS) implantations decreased (-12.8% and -2.4%, respectively), while growth in temporal lobectomies (5.9%), extratemporal resections (11.9%), and hemispherectomies/otomies (13.1%) lagged center growth (59%), leading to a decrease in median volumes of these procedures per center. DISCUSSION: During the study period, the availability of specialty epilepsy care in the United States improved as the NAEC implemented its accreditation program. Surgical case complexity increased while aggregate surgical volume remained stable or declined across most procedure types, with a corresponding decline in cases per center. This article describes recent data trends and current state of resources and practice across NAEC member centers and identifies several future directions for driving systematic improvements in epilepsy care.

Highlights From AES2020, a Virtual American Epilepsy Society Experience.

Due to COVID-19 a live, in-person meeting was not possible for the American Epilepsy Society in 2020. An alternative, virtual event, the AES2020, was held instead. AES2020 was a great success with 4679 attendees from 70 countries. The educational content was outstanding and spanned the causes, treatments, and outcomes from epileptic encephalopathy to the iatrogenicity of epilepsy interventions to neurocognitive disabilities to the approach to neocortical epilepsies. New gene therapy approaches such as antisense oligonucleotide treatment for Dravet syndrome were introduced and neuromodulation devices were discussed. There were many other topics discussed in special interest groups and investigators' workshops. A highlight was having a Nobel prize winner speak about memory processing. Human intracranial electrophysiology contributes insights into memory processing and complements animal work. In a special COVID symposium, the impact of COVID on patients with epilepsy was reviewed. Telehealth has been expanded rapidly and may be well suited for some parts of epilepsy care. In summary, the epilepsy community was alive and engaged despite being limited to a virtual platform.

Nonepileptic Episodic Events.

PURPOSE OF REVIEW: This review addresses the scope, evaluation, treatments, and outcomes of patients with nonepileptic episodic events with a focus on psychogenic nonepileptic seizures. Differentiation of the types of events, including a review of terminology, is included, as well as a brief review of special patient populations with these disorders. RECENT FINDINGS: There are continued efforts to develop tools to improve the diagnosis of these disorders. A thorough evaluation with trained personnel and physicians knowledgeable in the assessment and treatment of these disorders is important. Although inpatient video-EEG monitoring in an epilepsy monitoring unit remains the gold standard for diagnosis, the assessment of clinical and historical factors is critical and can be useful in expediting the process and improving diagnostic certainty. International efforts have recently assisted in providing guidelines for the evaluation of the psychogenic disorders and may help target educational and other resources to underserved areas. SUMMARY: The prompt and accurate diagnosis of nonepileptic episodic events and psychogenic nonepileptic seizures is possible with current technology, and the appropriate and targeted use of evidence-based treatments may help improve patient quality of life and avoid unnecessary disability in patients with these disorders.

Interictal Epileptiform Discharge Detection in EEG in Different Practice Settings.

OBJECTIVE: The goal of the study was to measure the performance of academic and private practice (PP) neurologists in detecting interictal epileptiform discharges in routine scalp EEG recordings. METHODS: Thirty-five EEG scorers (EEGers) participated (19 academic and 16 PP) and marked the location of ETs in 200 30-second EEG segments using a web-based EEG annotation system. All participants provided board certification status, years of Epilepsy Fellowship Training (EFT), and years in practice. The Persyst P13 automated IED detection algorithm was also run on the EEG segments for comparison. RESULTS: Academic EEGers had an average of 1.66 years of EFT versus 0.50 years of EFT for PP EEGers (P < 0.0001) and had higher rates of board certification. Inter-rater agreement for the 35 EEGers was fair. There was higher performance for EEGers in academics, with at least 1.5 years of EFT, and with American Board of Clinical Neurophysiology and American Board of Psychiatry and Neurology-E specialty board certification. The Persyst P13 algorithm at its default setting (perception value = 0.4) did not perform as well at the EEGers, but at substantially higher perception value settings, the algorithm performed almost as well human experts. CONCLUSIONS: Inter-rater agreement among EEGers in both academic and PP settings varies considerably. Practice location, years of EFT, and board certification are associated with significantly higher performance for IED detection in routine scalp EEG. Continued medical education of PP neurologists and neurologists without EFT is needed to improve routine scalp EEG interpretation skills. The performance of automated detection algorithms is approaching that of human experts.

Regulating drivers with epilepsy in Maryland: Results of the application of a United States consensus guideline.

OBJECTIVE: Driving regulations for people with seizures vary widely throughout the United States and the world. Maryland updated their guidelines in 2003 to reflect those of a U.S. consensus guideline requiring a minimum 3-month seizure-free period as well as an individual risk assessment by a Medical Advisory Board (MAB). This retrospective study provides the first analysis of outcomes after the implementation of the consensus guidelines and an assessment of their predictive validity through longitudinal outcome data. METHODS: MAB reviews and licensing records for Maryland driver applicants with seizures between 2004 and 2005 were reviewed, during which 254 first-time applicants were processed. The initial licensing decisions were assessed and the subsequent seizure recurrence and crash rates over the following 7 years were evaluated. RESULTS: The MAB approved driving for 74.8% of initial applicants; most had been seizure-free for over 6 months. Approved drivers had a longer median seizure-free period (563 days) compared to those who were denied (104.5 days, p < 0.01), and 22.7% of approved drivers had seizures recur during monitoring over the next year, although none resulted in crashes or deaths. Of applicants initially denied (n = 50), 89.3% were eventually licensed. Treating physicians recommended driving for 84.4% of applicants rejected by the MAB. SIGNIFICANCE: Maryland's individualized system for assessing driving applicants with seizures resulted in a dynamic process of approvals and denials based on favorable and unfavorable risk factors and lengths of seizure freedom. Seizure recurrences were comparable to internationally accepted rates. Over the course of monitoring, most applicants were eventually licensed. Treating physicians recommended that nearly all their patient applicants be permitted to drive, which raises safety concerns for the 10 states that rely solely on physician recommendations. Further assessment is needed of the risk factors deemed favorable and unfavorable by the U.S. consensus guidelines.

Which patients with epilepsy are at risk for psychogenic nonepileptic seizures (PNES)? A multicenter case-control study.

OBJECTIVE: We sought to examine the clinical and electrographic differences between patients with combined epileptic (ES) and psychogenic nonepileptic seizures (PNES) and age- and gender-matched patients with ES-only and PNES-only. METHODS: Data from 138 patients (105 women [77%]), including 46 with PNES/ES (39±12years), 46 with PNES-only (39±11years), and 46 with ES-only (39±11years), were compared using logistic regression analysis after adjusting for clustering effect. RESULTS: In the cohort with PNES/ES, ES antedated PNES in 28 patients (70%) and occurred simultaneously in 11 (27.5%), while PNES were the initial presentation in only 1 case (2.5%); disease duration was undetermined in 6. Compared with those with ES-only, patients with PNES/ES had higher depression and anxiety scores, shorter-duration electrographic seizures, less ES absence/staring semiology (all p≤0.01), and more ES arising in the right hemisphere, both in isolation and in combination with contralateral brain regions (61% vs. 41%; p=0.024, adjusted for anxiety and depression) and tended to have less ES arising in the left temporal lobe (13% vs. 28%; p=0.054). Compared with those with PNES-only, patients with PNES/ES tended to show fewer right-hemibody PNES events (7% vs. 23%; p=0.054) and more myoclonic semiology (10% vs. 2%; p=0.073). CONCLUSIONS: Right-hemispheric electrographic seizures may be more common among patients with ES who develop comorbid PNES, in agreement with prior neurobiological studies on functional neurological disorders.

Counseling Epilepsy Patients on Driving and Employment.

People with epilepsy identify driving and employment among their major concerns. People with controlled seizures may be permitted to drive in every state in the United States, but people with uncontrolled seizures are restricted from licensure. Unemployment and underemployment for people with epilepsy are serious problems that depend on the frequency and type of seizure disorder and associated medical and psychological problems. Most jobs, with reasonable accommodation by employers, are suitable for people with epilepsy. Federal protections through the Americans with Disabilities Act confer civil rights protection by law on people with disabilities such as epilepsy.

Development and Feasibility Testing of a Critical Care EEG Monitoring Database for Standardized Clinical Reporting and Multicenter Collaborative Research.

PURPOSE: The rapid expansion of the use of continuous critical care electroencephalogram (cEEG) monitoring and resulting multicenter research studies through the Critical Care EEG Monitoring Research Consortium has created the need for a collaborative data sharing mechanism and repository. The authors describe the development of a research database incorporating the American Clinical Neurophysiology Society standardized terminology for critical care EEG monitoring. The database includes flexible report generation tools that allow for daily clinical use. METHODS: Key clinical and research variables were incorporated into a Microsoft Access database. To assess its utility for multicenter research data collection, the authors performed a 21-center feasibility study in which each center entered data from 12 consecutive intensive care unit monitoring patients. To assess its utility as a clinical report generating tool, three large volume centers used it to generate daily clinical critical care EEG reports. RESULTS: A total of 280 subjects were enrolled in the multicenter feasibility study. The duration of recording (median, 25.5 hours) varied significantly between the centers. The incidence of seizure (17.6%), periodic/rhythmic discharges (35.7%), and interictal epileptiform discharges (11.8%) was similar to previous studies. The database was used as a clinical reporting tool by 3 centers that entered a total of 3,144 unique patients covering 6,665 recording days. CONCLUSIONS: The Critical Care EEG Monitoring Research Consortium database has been successfully developed and implemented with a dual role as a collaborative research platform and a clinical reporting tool. It is now available for public download to be used as a clinical data repository and report generating tool.

Evidence-based guideline: Management of an unprovoked first seizure in adults: Report of the Guideline Development Subcommittee of the American Academy of Neurology and the American Epilepsy Society.

OBJECTIVE: To provide evidence-based recommendations for treatment of adults with an unprovoked first seizure. METHODS: We defined relevant questions and systematically reviewed published studies according to the American Academy of Neurology's classification of evidence criteria; we based recommendations on evidence level. RESULTS AND RECOMMENDATIONS: Adults with an unprovoked first seizure should be informed that their seizure recurrence risk is greatest early within the first 2 years (21%-45%) (Level A), and clinical variables associated with increased risk may include a prior brain insult (Level A), an EEG with epileptiform abnormalities (Level A), a significant brain-imaging abnormality (Level B), and a nocturnal seizure (Level B). Immediate antiepileptic drug (AED) therapy, as compared with delay of treatment pending a second seizure, is likely to reduce recurrence risk within the first 2 years (Level B) but may not improve quality of life (Level C). Over a longer term (>3 years), immediate AED treatment is unlikely to improve prognosis as measured by sustained seizure remission (Level B). Patients should be advised that risk of AED adverse events (AEs) may range from 7% to 31% (Level B) and that these AEs are likely predominantly mild and reversible. Clinicians' recommendations whether to initiate immediate AED treatment after a first seizure should be based on individualized assessments that weigh the risk of recurrence against the AEs of AED therapy, consider educated patient preferences, and advise that immediate treatment will not improve the long-term prognosis for seizure remission but will reduce seizure risk over the subsequent 2 years.

Overlap between dissociation and other psychological characteristics in patients with psychogenic nonepileptic seizures.

Patients with psychogenic nonepileptic seizures (PNES) often report symptoms of dissociation. However, it is unclear how these symptoms relate to psychotherapeutic treatment, for example, with cognitive-behavioral therapy (CBT). Here, we investigated the degree of overlap between symptoms of dissociation and other psychiatric features that are more traditional targets for CBT. We used a hierarchical linear regression to measure the variance associated with dissociative symptoms (as assessed by the Dissociative Experiences Scale - DES) among 46 individuals with PNESs. The regression predictor variables are indices of participants' self-rated mood, self-efficacy, quality of life, locus of control, and life outlook (e.g., optimism). Results revealed that 70.2% of the variance associated with DES score was explained by psychological distress and locus of control. The other factors examined did not make a significant contribution to the regression model. These results suggest that traditional CBT targets - mood symptoms, mood distress, and dysfunctional beliefs about locus of control - overlap substantially with self-reported dissociative symptoms.

Cognitive behavioral therapy for psychogenic neurological disorders.

BACKGROUND: Psychogenic neurological disorders (PNDs) represent a significant problem in neurology, due to the difficulty in diagnosis and lack of effective and widely available treatment options. Treatment options for this population are limited. Preliminary evidence reveals cognitive behavioral therapy (CBT) may be useful in these disorders. REVIEW SUMMARY: The types of PNDs and their presentations are summarized, and the utilization of CBT in treatment of these disorders is reviewed. Accurate and timely diagnosis of the disorders is paramount and provides direction for implementing appropriate treatment. CONCLUSIONS: Neurologists should be familiar with the types of PNDs, clinical findings, and treatment principles of CBT. Early and accurate diagnosis may lead to improved treatment outcomes. Controlled treatment trials for this population are needed to determine efficacy. Further study of CBT in these patients may also help to elucidate the underlying etiology of these disorders by contributing to the understanding of associated psychopathology.

Nonconvulsive status epilepticus: value of a benzodiazepine trial for predicting outcomes.

OBJECTIVES: Managing nonconvulsive status epilepticus (NCSE) poses many challenges that would benefit from additional early measures to predict patient outcomes. Here, we evaluate clinical and electroencephalographic responses to an acute antiepileptic drug trial as an added measure for predicting outcomes in patients presenting with suspected NCSE. METHODS: We analyzed all patients referred to our Neurology Service with suspected NCSE assessed by a standard acute intravenous (IV) benzodiazepine (BDZ) protocol. We correlated patients' clinical and electrographic (EEG) responses to the BDZ trial with their subsequent outcomes, including survival, recovery of consciousness, and functional status at hospital discharge. RESULTS: From 1990 to 2001, we identified 62 patients with NCSE who were initially evaluated with an acute IV BDZ protocol trial. A favorable clinical response with improvement in consciousness was observed in 22 patients (35%), whereas 40 (65%) were clinical nonresponders. All of the positive clinical responders (100%) survived, recovered consciousness, and exhibited good functional outcomes. In contrast, outcomes were significantly poorer (P<0.001) for the clinical nonresponders; only 14 (35%) recovered consciousness and 22 (55%) survived, with 59% of those survivors demonstrating poor functional outcomes. EEG improvement with BDZs also predicted better outcome, but it was less robust than the clinical response, with better subsequent recovery of consciousness (P<0.05), but not functional outcome or survival. CONCLUSIONS: This study demonstrates that a clinical and, to a lesser degree, EEG response to an acute trial of IV BDZs are predictive of subsequent outcome in patients with suspected NCSE, and warrant further consideration and investigation for assessing and managing patients.