Migraine with prolonged aphasic aura associated with a CACNA1A mutation: A case report and narrative review.

OBJECTIVE: To demonstrate that a known CACNA1A variant is associated with a phenotype of prolonged aphasic aura without hemiparesis. BACKGROUND: The usual differential diagnosis of prolonged aphasia without hemiparesis includes vascular disease, seizure, metabolic derangements, and migraine. Genetic mutations in the CACNA1A gene can lead to a myriad of phenotypes, including familial hemiplegic migraine (FHM) type 1, an autosomal dominant disorder characterized by an aura of unilateral, sometimes prolonged weakness. Though aphasia is a common feature of migraine aura, with or without hemiparesis, aphasia without hemiparesis has not been reported with CACNA1A mutations. METHODS: We report the case of a 51-year-old male who presented with a history of recurrent episodes of aphasia without hemiparesis lasting days to weeks. His headache was left sided and was heralded by what his family described as "confusion." On examination, he had global aphasia without other focal findings. Family history revealed several relatives with a history of severe headaches with neurologic deficits including aphasia and/or weakness. Imaging revealed T2 hyperintensities in the left parietal/temporal/occipital regions on MRI scan with corresponding hyperperfusion on SPECT. Genetic testing revealed a missense mutation in the CACNA1A gene. CONCLUSIONS: This case expands the phenotypic spectrum of the CACNA1A mutation and FHM to include prolonged aphasic aura without hemiparesis. Our patient's SPECT imaging demonstrated hyperperfusion in areas correlating with aura symptoms which can occur in prolonged aura.

Recognizing seizure clusters in the community: The path to uniformity and individualization in nomenclature and definition.

Seizure emergencies experienced by patients with epilepsy include status epilepticus and seizure clusters. Although an accepted definition of status epilepticus exists, no clear consensus definition of seizure clusters has emerged; this is further complicated by the appearance in the literature of various empirically based definitions that have been developed for clinical trial study designs. In general, patients with intractable epilepsy have been shown to have a significant risk for acute episodes of increased seizure activity called seizure clusters (also referred to as acute repetitive seizures, among other terms) that differ from their usual seizure pattern. Duration (e.g., number of hours or days) is often included in the definition of a seizure cluster; however, the duration may vary among patients, with some seizure clusters lasting ≥24 h and requiring long-acting treatment for this period. In addition to seizure cluster duration, the time between seizures and possible acceleration in seizure frequency during the cluster may be important variables. The recognition and treatment of seizure clusters require urgent action because episodes that are not quickly and appropriately treated may lead to injury or progress to status epilepticus or potentially death. Most seizure clusters occur outside a medical facility (in the community) and treatment is usually administered by nonmedical individuals; therefore, health care providers may benefit from a clear description of these potential seizure emergencies that they can then use to educate patients and caregivers on the prompt and appropriate identification of seizure clusters and administration of rescue therapy. Here we explore why greater uniformity is needed in the discussion of seizure clusters. This exploration examines epidemiologic studies of seizure clusters and status epilepticus, inconsistencies in nomenclature and definitions for seizure clusters, practical application of seizure cluster terminology, and the potential use of acute seizure action plans and patient-specific individualized definitions in the clinical setting.

Epilepsy-related stigma and attitudes: Systematic review of screening instruments and interventions - Report by the International League Against Epilepsy Task Force on Stigma in Epilepsy.

OBJECTIVE: This is a systematic review aimed at summarizing the evidence related to instruments that have been developed to measure stigma or attitudes toward epilepsy and on stigma-reducing interventions. METHODS: This review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) standards. A broad literature search (1985-2019) was performed in 13 databases. Articles were included if they described the development and testing of psychometric properties of an epilepsy-related stigma or attitude scale or stigma-reducing interventions. Two reviewers independently screened abstracts, reviewed full-text articles, and extracted data. Basic descriptive statistics are reported. RESULTS: We identified 4234 abstracts, of which 893 were reviewed as full-text articles. Of these, 38 met inclusion criteria for an instrument development study and 30 as a stigma-reduction intervention study. Most instruments were initially developed using well-established methods and were tested in relatively large samples. Most intervention studies involved educational programs for adults with pre- and post-evaluations of attitudes toward people with epilepsy. Intervention studies often failed to use standardized instruments to quantify stigmatizing attitudes, were generally underpowered, and often found no evidence of benefit or the benefit was not sustained. Six intervention studies with stigma as the primary outcome had fewer design flaws and showed benefit. Very few or no instruments were validated for regional languages or culture, and there were very few interventions tested in some regions. SIGNIFICANCE: Investigators in regions without instruments should consider translating and further developing existing instruments rather than initiating the development of new instruments. Very few stigma-reduction intervention studies for epilepsy have been conducted, study methodology in general was poor, and standardized instruments were rarely used to measure outcomes. To accelerate the development of effective epilepsy stigma-reduction interventions, a paradigm shift from disease-specific, siloed trials to collaborative, cross-disciplinary platforms based upon unified theories of stigma transcending individual conditions will be needed.

Psychogenic nonepileptic seizures during the COVID-19 pandemic in New York City - A distinct response from the epilepsy experience.

Although psychogenic nonepileptic seizures (PNES) are a common neurologic condition, there remains a paucity of literature on the COVID-19 pandemic's effect on these patients. Using a cross-sectional questionnaire study, our group examined the experience of patients with PNES at a single Comprehensive Epilepsy Center in New York City, the epicenter of the initial COVID-19 outbreak in the United States. Among our cohort of 18 subjects with PNES, 22.2% reported an improvement in seizure control during the peak of the COVID-19 pandemic in New York City. Compared to the cohort of subjects with epilepsy without PNES, subjects with PNES were significantly more likely to report an improvement (p = 0.033). Our findings signal that sleep and stress may be relevant variables in both conditions that should be further investigated and potentially intervened upon. Larger dedicated studies of patients with PNES are needed to understand the impact of the pandemic's widespread societal effects on these patients.

Factors associated with patients not proceeding with proposed resective epilepsy surgery.

BACKGROUND: This study evaluated the association between eligible patients not proceeding with resective epilepsy surgery and various demographic, disease-specific, and epilepsy-evaluation variables. METHODS: This retrospective case-control study included patients identified as candidates for resective epilepsy surgery at the Montefiore Medical Center between January 1, 2009 and June 30, 2017. Chi-squared, two-tailed, independent sample t-test, Mann-Whitney U test and logistic regression were utilized to identify variables associated with patients not proceeding with surgery. RESULTS: Among the 159 potential surgical candidates reviewed over the 8.5-year study period, only 53 ultimately proceeded with surgery (33%). Eighty-seven (55%) out of these 159 patients were identified as appropriate for resective epilepsy surgery during the study period. Thirty-four (39%) of these 87 patients did not proceed with surgery. Variables independently correlated (either positively or negatively) with the patient not proceeding with surgery were: being employed [Odds Ratio (OR) 4.2, 95% confidence interval (CI) 1.12-15.73], temporal lobe lesion on MRI (OR 0.35, 95% CI 0.14-0.84), temporal lobe EEG ictal onsets (OR 0.21, 95% CI 0.07-0.62), and temporal lobe epileptogenic zone (OR 0.19, 95% CI 0.07-0.55). CONCLUSION: The novel finding in this study is the association between employment status and whether the patient had epilepsy surgery: employed patients were 4.2 times more likely to not proceed with surgery compared to unemployed patients. In addition, patients with a temporal lobe lesion on MRI, temporal lobe EEG ictal onsets, and/or a temporal epileptogenic zone were more likely to proceed with surgery. Future work will be needed to evaluate these findings prospectively, determine if they generalize to other patient populations, explore the decision whether or not to proceed with epilepsy surgery from a patient-centered perspective, and suggest strategies to reduce barriers to this underutilized treatment.

Folding a neuroscience center into streamlined COVID-19 response teams: Lessons in origami.

In response to the COVID-19 pandemic epicenter in Bronx, NY, the Montefiore Neuroscience Center required rapid and drastic changes when considering the delivery of neurologic care, health and safety of staff, and continued education and safety for house staff. Health care leaders rely on principles that can be in conflict during a disaster response such as this pandemic, with equal commitments to ensure the best care for those stricken with COVID-19, provide high-quality care and advocacy for patients and families coping with neurologic disease, and advocate for the health and safety of health care teams, particularly house staff and colleagues who are most vulnerable. In our attempt to balance these principles, over 3 weeks, we reformatted our inpatient neuroscience services by reducing from 4 wards to just 1, in the following weeks delivering care to over 600 hospitalized patients with neuro-COVID and over 1,742 total neuroscience hospital bed days. This description from members of our leadership team provides an on-the-ground account of our effort to respond nimbly to a complex and evolving surge of patients with COVID in a large urban hospital network. Our efforts were based on (1) strategies to mitigate exposure and transmission, (2) protection of the health and safety of staff, (3) alleviation of logistical delays and strains in the system, and (4) facilitating coordinated communication. Each center's experience will add to knowledge of best practices, and emerging research will help us gain insights into an evidence-based approach to neurologic care during and after the COVID-19 pandemic.

Are we responding effectively to bone mineral density loss and fracture risks in people with epilepsy?

OBJECTIVE: A 2007 study performed at Montefiore Medical Center (Bronx, NY) identified high prevalence of reduced bone density in an urban population of patients with epilepsy and suggested that bone mineralization screenings should be regularly performed for these patients. We conducted a long-term follow-up study to determine whether bone mineral density (BMD) loss, osteoporosis, and fractures have been successfully treated or prevented. METHODS: In the current study, patients from the 2007 study who had two dual-energy absorptiometry (DXA) scans performed at least 5 years apart were analyzed. The World Health Organization (WHO) criteria to diagnose patients with osteopenia or osteoporosis were used, and each patient's probability of developing fractures was calculated with the Fracture Risk Assessment Tool (FRAX). RESULTS: The median time between the first and second DXA scans for the 81 patients analyzed was 9.4 years (range 5-14.7). The median age at the first DXA scan was 41 years (range 22-77). Based on WHO criteria, 79.0% of patients did not have worsening of bone density, while 21.0% had new osteopenia or osteoporosis; many patients were prescribed treatment for bone loss. Older age, increased duration of anti-epileptic drug (AED) usage, and low body mass index (BMI) were risk factors for abnormal BMDs. Based on the first DXA scan, the FRAX calculator estimated that none of the patients in this study had a 10-year risk of more than 20% for developing major osteoporotic fracture (hip, spine, wrist, or humeral fracture). However, in this population, 11 patients (13.6%) sustained a major osteoporotic fracture after their first DXA scan. SIGNIFICANCE: Despite being routinely screened and frequently treated for bone mineral density loss and fracture prevention, many patients with epilepsy suffered new major osteoporotic fractures. This observation is especially important as persons with epilepsy are at high risk for falls and traumas.

EEG findings in acutely ill patients investigated for SARS-CoV-2/COVID-19: A small case series preliminary report.

OBJECTIVE: Acute encephalopathy may occur in COVID-19-infected patients. We investigated whether medically indicated EEGs performed in acutely ill patients under investigation (PUIs) for COVID-19 report epileptiform abnormalities and whether these are more prevalent in COVID-19 positive than negative patients. METHODS: In this retrospective case series, adult COVID-19 inpatient PUIs underwent EEGs for acute encephalopathy and/or seizure-like events. PUIs had 8-channel headband EEGs (Ceribell; 20 COVID-19 positive, 6 COVID-19 negative); 2 more COVID-19 patients had routine EEGs. Overall, 26 Ceribell EEGs, 4 routine and 7 continuous EEG studies were reviewed. EEGs were interpreted by board-certified clinical neurophysiologists (n = 16). EEG findings were correlated with demographic data, clinical presentation and history, and medication usage. Fisher's exact test was used. RESULTS: We included 28 COVID-19 PUIs (30-83 years old), of whom 22 tested positive (63.6% males) and 6 tested negative (33.3% male). The most common indications for EEG, among COVID-19-positive vs COVID-19-negative patients, respectively, were new onset encephalopathy (68.2% vs 33.3%) and seizure-like events (14/22, 63.6%; 2/6, 33.3%), even among patients without prior history of seizures (11/17, 64.7%; 2/6, 33.3%). Sporadic epileptiform discharges (EDs) were present in 40.9% of COVID-19-positive and 16.7% of COVID-19-negative patients; frontal sharp waves were reported in 8/9 (88.9%) of COVID-19-positive patients with EDs and in 1/1 of COVID-19-negative patient with EDs. No electrographic seizures were captured, but 19/22 COVID-19-positive and 6/6 COVID-19-negative patients were given antiseizure medications and/or sedatives before the EEG. SIGNIFICANCE: This is the first preliminary report of EDs in the EEG of acutely ill COVID-19-positive patients with encephalopathy or suspected clinical seizures. EDs are relatively common in this cohort and typically appear as frontal sharp waves. Further studies are needed to confirm these findings and evaluate the potential direct or indirect effects of COVID-19 on activating epileptic activity.

Individualizing the definition of seizure clusters based on temporal clustering analysis.

OBJECTIVE: Seizure clusters are often encountered in people with poorly controlled epilepsy. Detection of seizure clusters is currently based on simple clinical rules, such as two seizures separated by four or fewer hours or multiple seizures in 24 h. Current definitions fail to distinguish between statistically significant clusters and those that may result from natural variation in the person's seizures. Ability to systematically define when a seizure cluster is significant for the individual carries major implications for treatment. However, there is no uniform consensus on how to define seizure clusters. This study proposes a principled statistical approach to defining seizure clusters that addresses these issues. METHODS: A total of 533,968 clinical seizures from 1,748 people with epilepsy in the Seizure Tracker™ seizure diary database were used for algorithm development. We propose an algorithm for automated individualized seizure cluster identification combining cumulative sum change-point analysis with bootstrapping and aberration detection, which provides a new approach to personalized seizure cluster identification at user-specified levels of clinical significance. We develop a standalone user interface to make the proposed algorithm accessible for real-time seizure cluster identification (ClusterCalc™). Clinical impact of systematizing cluster identification is demonstrated by comparing empirically-defined clusters to those identified by routine seizure cluster definitions. We also demonstrate use of the Hurst exponent as a standardized measure of seizure clustering for comparison of seizure clustering burden within or across patients. RESULTS: Seizure clustering was present in 26.7 % (95 % CI, 24.5-28.7 %) of people with epilepsy. Empirical tables were provided for standardizing inter- and intra-patient comparisons of seizure cluster tendency. Using the proposed algorithm, we found that 37.7-59.4 % of seizures identified as clusters based on routine definitions had high probability of occurring by chance. Several clusters identified by the algorithm were missed by conventional definitions. The utility of the ClusterCalc algorithm for individualized seizure cluster detection is demonstrated. SIGNIFICANCE: This study proposes a principled statistical approach to individualized seizure cluster identification and demonstrates potential for real-time clinical usage through ClusterCalc. Using this approach accounts for individual variations in baseline seizure frequency and evaluates statistical significance. This new definition has the potential to improve individualized epilepsy treatment by systematizing identification of unrecognized seizure clusters and preventing unnecessary intervention for random events previously considered clusters.

Arfgef1 haploinsufficiency in mice alters neuronal endosome composition and decreases membrane surface postsynaptic GABAA receptors.

ARFGEF1 encodes a guanine exchange factor involved in intracellular vesicle trafficking, and is a candidate gene for childhood genetic epilepsies. To model ARFGEF1 haploinsufficiency observed in a recent Lennox Gastaut Syndrome patient, we studied a frameshift mutation (Arfgef1fs) in mice. Arfgef1fs/+ pups exhibit signs of developmental delay, and Arfgef1fs/+ adults have a significantly decreased threshold to induced seizures but do not experience spontaneous seizures. Histologically, the Arfgef1fs/+ brain exhibits a disruption in the apical lining of the dentate gyrus and altered spine morphology of deep layer neurons. In primary hippocampal neuron culture, dendritic surface and synaptic but not total GABAA receptors (GABAAR) are reduced in Arfgef1fs/+ neurons with an accompanying decrease in the number of GABAAR-containing recycling endosomes in cell body. Arfgef1fs/+ neurons also display differences in the relative ratio of Arf6+:Rab11+:TrfR+ recycling endosomes. Although the GABAAR-containing early endosomes in Arfgef1fs/+ neurons are comparable to wildtype, Arfgef1fs/+ neurons show an increase in the number of GABAAR-containing lysosomes in dendrite and cell body. Together, the altered endosome composition and decreased neuronal surface GABAAR results suggests a mechanism whereby impaired neuronal inhibition leads to seizure susceptibility.

Seizure self-prediction in a randomized controlled trial of stress management.

OBJECTIVE: Using electronic diaries as part of a randomized controlled trial of stress reduction for epilepsy, we evaluated factors associated with successful seizure self-prediction. METHODS: Adults with medication-resistant focal epilepsy were recruited from 3 centers and randomized to treatment with progressive muscle relaxation or control focused attention. An 8-week baseline was followed by 12 weeks of double-blind treatment. Twice daily, participants rated the likelihood of a seizure in the next 24 hours on a 5-point scale from very unlikely to almost certain, along with mood, premonitory symptoms, stress ratings, and seizure counts. We analyzed the association of mood, premonitory symptoms, stress, and circadian influences on seizure self-prediction. RESULTS: Sixty-four participants completed the trial (3,126 seizures). Diary entry adherence was >82%. Participant self-prediction was associated with seizure occurrence at 6, 12, and 24 hours (p < 0.0001). Odds ratio (OR) of seizure prediction increased systematically with participants' prediction of seizure likelihood (p < 0.0001, all levels of prediction and all time intervals). For the 12-hour prediction window, median specificity for seizure prediction was 0.94 and negative predictive value 0.94; median sensitivity was 0.10 and positive predictive value 0.13. A subset of 13 participants (20% of sample) met criteria for good predictors (median OR for seizure prediction 5.25). Mood, stress, premonitory symptoms, seizure time, and randomized group were not associated with seizure occurrence. CONCLUSION: In this prospective study, participants' prediction of a high probability of seizure was significantly associated with subsequent seizure occurrence within 24 hours. Future studies should focus on understanding factors that drive self-prediction. CLINICALTRIALSGOV IDENTIFIER: NCT01444183.

Behavioral interventions in epilepsy.

PURPOSE OF REVIEW: Behavioral intervention describes multiple modalities of treatments which are of increasing interest in epilepsy. This review addresses recent behavioral clinical trials in epilepsy including cognitive behavioral therapy (CBT), mindfulness, progressive muscle relaxation (PMR), and self-management. Results and conclusions from updated Cochrane reviews and the recent International League Against Epilepsy Psychology task force are presented. RECENT FINDINGS: Two recent large randomized controlled trials (mindfulness and progressive muscle relaxation) reported improved seizure frequency with behavioral treatments. In both studies, participants in both the active and the attentional control arms showed significant seizure reduction, whereas quality of life and stress reduction were better noted in the active arms. Additional behavioral modalities have reported improved seizure control including yoga, bio/neurofeedback, and music therapy.Significant improvements in multiple quality of life, cognitive domains, and medication adherence have been reported from randomized and open label trials of cognitive behavioral therapy, and self-management programs. Multiple promising self-management programs have been recently reported, often utilizing the power of web-based apps, and digitally delivered group therapy. In 2018, the International League Against Epilepsy Psychology task force recommended that 'psychological interventions should be incorporated into comprehensive epilepsy care.' SUMMARY: Behavioral treatments are successful and likely underutilized in the treatment of epilepsy. Given the challenge of conducting randomized clinical trials of behavioral therapy, much remains to be studied. However, for motivated and interested patients, appropriately chosen behavioral therapies appear to be important adjuncts to standard therapy. The timing is currently optimal to take best advantage of smartphone apps and web-based delivery systems, both for research and therapeutic purposes.

Characteristics of large patient-reported outcomes: Where can one million seizures get us?

Objective: To analyze data from Seizure Tracker, a large electronic seizure diary, including comparison of seizure characteristics among different etiologies, temporal patterns in seizure fluctuations, and specific triggers. Methods: Zero-inflated negative binomial mixed-effects models were used to evaluate temporal patterns of seizure events (during the day or week), as well as group differences in monthly seizure frequency between children and adults and between etiologies. The association of long seizures with seizure triggers was evaluated using a mixed-effects logistic model with subject as the random effect. Incidence rate ratios (IRRs) and odds ratios were reported for analyses involving zero-inflated negative binomial and logistic mixed-effects models, respectively. Results: A total of 1,037,909 seizures were logged by 10,186 subjects (56.7% children) from December 2007 to January 2016. Children had more frequent seizures than adults did (median monthly seizure frequency 3.5 vs. 2.7, IRR 1.26; p < 0.001). Seizures demonstrated a circadian pattern (higher frequency between 07:00 a.m. and 10:00 a.m. and lower overnight), and seizures were reported differentially across the week (seizure rates higher Monday through Friday than Saturday or Sunday). Longer seizures (>5 or >30 min) had a higher proportion of the following triggers when compared with shorter seizures: "Overtired or irregular sleep," "Bright or flashing lights," and "Emotional stress" (p < 0.004). Significance: This study explored a large cohort of patients with self-reported seizures; strengths and limitations of large seizure diary databases are discussed. The findings in this study are consistent with those of prior work in smaller validated cohorts, suggesting that patient-recorded databases are a valuable resource for epilepsy research, capable of both replication of results and generation of novel hypotheses.

Behavioral interventions as a treatment for epilepsy: A multicenter randomized controlled trial.

OBJECTIVE: To evaluate the effect of a stress-reduction intervention in participants with medication-resistant epilepsy. METHODS: Adults with medication-resistant focal epilepsy (n = 66) were recruited from 3 centers and randomized to 1 of 2 interventions: (1) progressive muscle relaxation (PMR) with diaphragmatic breathing, or (2) control focused-attention activity with extremity movements. Following an 8-week baseline period, participants began 12 weeks of double-blind treatment. Daily self-reported mood and stress ratings plus seizure counts were completed by participants using an electronic diary, and no medication adjustments were permitted. The primary outcome was percent reduction in seizure frequency per 28 days comparing baseline and treatment; secondary outcomes included stress reduction and stress-seizure interaction. RESULTS: In the 66 participants in the intention-to-treat analysis, seizure frequency was reduced from baseline in both treatment groups (PMR: 29%, p < 0.05; focused attention: 25%, p < 0.05). PMR and focused attention did not differ in seizure reduction (p = 0.38), although PMR was associated with stress reduction relative to focused attention (p < 0.05). Daily stress was not a predictor of seizures. CONCLUSIONS: Both PMR and the focused-attention groups showed reduced seizure frequency compared to baseline in participants with medication-resistant focal seizures, although the 2 treatments did not differ. PMR was more effective than focused attention in reducing self-reported stress. CLINICALTRIALSGOV IDENTIFIER: NCT01444183.

Clinical characterization of the pre-ictal state in the pediatric population: A caretaker's perspective.

The unpredictability of seizures causes distress to patients with epilepsy and their caretakers. To date, no studies have explored seizure prediction specifically in the pediatric population. If the period of time preceding a seizure can be reliably identified, either by child or caretaker, there may be a role for pre-emptive interventions. The aim of this study was to investigate caretaker seizure prediction. A questionnaire was distributed to caretakers of patients with epilepsy. The patients were 0-21years old and experienced ≥1 seizure within the past year. We excluded patients with non-epileptic seizures or daily seizures. One hundred and fifty of 240 questionnaires met criteria. Of these, 32 (21.6%) caretakers indicated a positive report of seizure prediction. Age of seizure onset was earlier in the positive predictive group (3.3±3.3years) than in the non-predictor group (5.3±4.8years) (p=0.01). The most common pre-ictal symptoms reported were being tired, hazy look, and sleepiness. A total of 76.6% of caretakers reported at least one seizure precipitant. The prevalence of positive caretaker seizure prediction in this study is similar to that of seizure self-prediction in adult studies. These findings will be used to design prospective online or electronic diary studies to further investigate the caretaker's, as well as children's, perspectives on seizure prediction. We anticipate that this investigation may lead to novel treatments during times of high seizure risk.

Instruction manual for the ILAE 2017 operational classification of seizure types.

This companion paper to the introduction of the International League Against Epilepsy (ILAE) 2017 classification of seizure types provides guidance on how to employ the classification. Illustration of the classification is enacted by tables, a glossary of relevant terms, mapping of old to new terms, suggested abbreviations, and examples. Basic and extended versions of the classification are available, depending on the desired degree of detail. Key signs and symptoms of seizures (semiology) are used as a basis for categories of seizures that are focal or generalized from onset or with unknown onset. Any focal seizure can further be optionally characterized by whether awareness is retained or impaired. Impaired awareness during any segment of the seizure renders it a focal impaired awareness seizure. Focal seizures are further optionally characterized by motor onset signs and symptoms: atonic, automatisms, clonic, epileptic spasms, or hyperkinetic, myoclonic, or tonic activity. Nonmotor-onset seizures can manifest as autonomic, behavior arrest, cognitive, emotional, or sensory dysfunction. The earliest prominent manifestation defines the seizure type, which might then progress to other signs and symptoms. Focal seizures can become bilateral tonic-clonic. Generalized seizures engage bilateral networks from onset. Generalized motor seizure characteristics comprise atonic, clonic, epileptic spasms, myoclonic, myoclonic-atonic, myoclonic-tonic-clonic, tonic, or tonic-clonic. Nonmotor (absence) seizures are typical or atypical, or seizures that present prominent myoclonic activity or eyelid myoclonia. Seizures of unknown onset may have features that can still be classified as motor, nonmotor, tonic-clonic, epileptic spasms, or behavior arrest. This "users' manual" for the ILAE 2017 seizure classification will assist the adoption of the new system.

A dedicated scholarly research program in an adult and pediatric neurology residency program.

OBJECTIVE: To describe and assess the effectiveness of a formal scholarly activity program for a highly integrated adult and pediatric neurology residency program. METHODS: Starting in 2011, all graduating residents were required to complete at least one form of scholarly activity broadly defined to include peer-reviewed publications or presentations at scientific meetings of formally mentored projects. The scholarly activity program was administered by the associate residency training director and included an expanded journal club, guided mentorship, a required grand rounds platform presentation, and annual awards for the most scholarly and seminal research findings. We compared scholarly output and mentorship for residents graduating within a 5-year period following program initiation (2011-2015) and during the preceding 5-year preprogram baseline period (2005-2009). RESULTS: Participation in scholarship increased from the preprogram baseline (24 of 53 graduating residents, 45.3%) to the postprogram period (47 of 57 graduating residents, 82.1%, p < 0.0001). Total scholarly output more than doubled from 49 activities preprogram (0.92/resident) to 139 postprogram (2.44/resident, p = 0.0002). The proportions of resident participation increased for case reports (20.8% vs 66.7%, p < 0.0001) and clinical research (17.0% vs 38.6%, p = 0.012), but were similar for laboratory research and topical reviews. The mean activities per resident increased for published abstracts (0.15 ± 0.41 to 1.26 ± 1.41, p < 0.0001), manuscripts (0.75 ± 1.37 to 1.00 ± 1.40, p = 0.36), and book chapters (0.02 ± 0.14 to 0.18 ± 0.60, p = 0.07). Rates of resident participation as first authors increased from 30.2% to 71.9% (p < 0.0001). The number of individual faculty mentors increased from 36 (preprogram) to 44 (postprogram). CONCLUSIONS: Our multifaceted program, designed to enhance resident and faculty engagement in scholarship, was associated with increased academic output and an expanded mentorship pool. The program was particularly effective at encouraging presentations at scientific meetings. Longitudinal analysis will determine whether such a program portfolio inspires an increase in academic careers involving neuroscience-oriented research.