Highlights From the Annual Meeting of the American Epilepsy Society 2022

With more than 6000 attendees between in-person and virtual offerings, the American Epilepsy Society Meeting 2022 in Nashville, felt as busy as in prepandemic times. An ever-growing number of physicians, scientists, and allied health professionals gathered to learn a variety of topics about epilepsy. The program was carefully tailored to meet the needs of professionals with different interests and career stages. This article summarizes the different symposia presented at the meeting. Basic science lectures addressed the primary elements of seizure generation and pathophysiology of epilepsy in different disease states. Scientists congregated to learn about anti-seizure medications, mechanisms of action, and new tools to treat epilepsy including surgery and neurostimulation. Some symposia were also dedicated to discuss epilepsy comorbidities and practical issues regarding epilepsy care. An increasing number of patient advocates discussing their stories were intertwined within scientific activities. Many smaller group sessions targeted more specific topics to encourage member participation, including Special Interest Groups, Investigator, and Skills Workshops. Special lectures included the renown Hoyer and Lombroso, an ILAE/IBE joint session, a spotlight on the impact of Dobbs v. Jackson on reproductive health in epilepsy, and a joint session with the NAEC on coding and reimbursement policies. The hot topics symposium was focused on traumatic brain injury and post-traumatic epilepsy. A balanced collaboration with the industry allowed presentations of the latest pharmaceutical and engineering advances in satellite symposia

Is Spontaneity Overrated? The Value of Cortical Stimulation-Induced Seizures

Association of Cortical Stimulation-Induced Seziure With Surgical Outcome in Patients With Focal Drug-Resistant Epilepsy Cuello Oderiz C, von Ellenrieder N, Dubeau F, et al. JAMA Neurol. 2019. Importance: Cortical stimulation is used during presurgical epilepsy evaluation for functional mapping and for defining the cortical area responsible for seizure generation. Despite wide use of cortical stimulation, the association between cortical stimulation-induced seizures and surgical outcome remains unknown. Objective: To assess whether the removal of the seizure-onset zone resulting from cortical stimulation is associated with a good surgical outcome. Design, Setting, and Participants: This cohort study used data from 2 tertiary epilepsy centers: Montreal Neurological Institute in Montreal, Quebec, Canada, and Grenoble Alpes University Hospital in Grenoble, France. Participants included consecutive patients (n = 103) with focal drug-resistant epilepsy who underwent stereoelectroencephalography between January 1, 2007, and January 1, 2017. Participant selection criteria were cortical stimulation during implantation, subsequent open surgical procedure with a follow-up of 1 or more years, and complete neuroimaging data sets for superimposition between intracranial electrodes and the resection. Main Outcomes and Measures: Cortical stimulation-induced typical electroclinical seizures, the volume of the surgical resection, and the percentage of resected electrode contacts inducing a seizure or encompassing the cortical stimulation-informed and spontaneous seizure-onset zones were identified. These measures were correlated with good (Engel class I) and poor (Engel classes II-IV) surgical outcomes. Electroclinical characteristics associated with cortical stimulation-induced seizures were analyzed. Results: In total, 103 patients were included, of whom 54 (52.4%) were female, and the mean (standard deviation) age was 31 (11) years. Fifty-nine (57.3%) patients had cortical stimulation-induced seizures. The percentage of patients with cortical stimulation-induced electroclinical seizures was higher in the good outcome group than in the poor outcome group (31 [70.5%] of 44 vs 28 [47.5%] of 59; P = .02). The percentage of the resected contacts encompassing the cortical stimulation-informed seizure-onset zone correlated with surgical outcome (median [range] percentage in good vs poor outcome: 63.2% [0%-100%] vs 33.3% [0%-84.6%]; Spearman rho = 0.38; P = .003). A similar result was observed for spontaneous seizures (median [range] percentage in good vs poor outcome: 57.1% [0%-100%] vs 32.7% [0%-100%]; Spearman rho = 0.32; P = .002). Longer elapsed time since the most recent seizure was associated with a higher likelihood of inducing seizures (>24 hours: 64.7% vs P = .04). Conclusions and Relevance: Seizure induction by cortical stimulation appears to identify the epileptic generator as reliably as spontaneous seizures do; this finding might lead to a more time-efficient intracranial presurgical investigation of focal epilepsy as the need to record spontaneous seizures is reduced

Cutting the Losses of Pregnant Women With Epilepsy

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Mind the Gap: SUDEP in the United States

Objective: To determine the impact of socioeconomic status (SES) on sudden unexpected death in epilepsy (SUDEP) rates. Methods: We queried all decedents presented for medicolegal investigation at 3 medical examiner offices across the country (New York City, Maryland, San Diego County) in 2009 to 2010 and 2014 to 2015. We identified all decedents for whom epilepsy/seizure was listed as cause/contributor to death or comorbid condition on the death certificate. We then reviewed all available reports. Decedents determined to have SUDEP were included for analysis. We used median income in the ZIP code of residence as a surrogate for SES. For each region, zip code regions were ranked by median household income and divided into quartiles based on total population for 2 time periods. Region-, age-, and income-adjusted epilepsy prevalence were estimated in each zip code. Sudden unexpected death in epilepsy rates in the highest and lowest SES quartiles were evaluated to determine disparity. Examined SUDEP rates in 2 time periods were also compared. Results: There were 159 and 43 SUDEP cases in the lowest and highest SES quartiles. Medical examiner-investigated SUDEP rate ratio between the lowest and highest SES quartiles was 2.6 (95% CI: 1.7-4.1, P < .0001) in 2009 to 2010 and 3.3 (95% CI: 1.9-6.0, P < .0001) in 2014 to 2015. There was a significant decline in overall SUDEP rate between the 2 study periods (36% decrease, 95% CI: 22%-48%, P < .0001). Conclusion: Medical examiner-investigated SUDEP incidence was significantly higher in people with the lowest SES compared to the highest SES. The difference persisted over a 5-year period despite decreased overall SUDEP rates

Neuromodulation in Drug Resistant Epilepsy

Epilepsy affects approximately 70 million people worldwide, and it is a significant contributor to the global burden of neurological disorders. Despite the advent of new AEDs, drug resistant-epilepsy continues to affect 30-40% of PWE. Once identified as having drug-resistant epilepsy, these patients should be referred to a comprehensive epilepsy center for evaluation to establish if they are candidates for potential curative surgeries. Unfortunately, a large proportion of patients with drug-resistant epilepsy are poor surgical candidates due to a seizure focus located in eloquent cortex, multifocal epilepsy or inability to identify the zone of ictal onset. An alternative treatment modality for these patients is neuromodulation. Here we present the evidence, indications and safety considerations for the neuromodulation therapies in vagal nerve stimulation (VNS), responsive neurostimulation (RNS), or deep brain stimulation (DBS)

Neuroimaging in the evaluation of epilepsy

Neuroimaging has provided extraordinary insight into the pathologic substrate of epilepsy. The excellent spatial resolution and soft tissue contrast of magnetic resonance imaging (MRI) allows identification of a substantial number of pathologies including hippocampal sclerosis, malformations of cortical development, low grade tumors, and vascular abnormalities, among others. Complementary imaging modalities such as positron emission tomography, single photon emission computed tomography, and magnetoencephalography can be diagnostically helpful as well. Identification of a pathologic substrate is particularly important in patients with medically refractory epilepsy who are undergoing evaluation for surgery, and essential in determining the likelihood of seizure freedom after surgical intervention. This article reviews current and emerging neuroimaging techniques in the field of epilepsy

1230 An unusual presentation of dementia with Lewy bodies in a septuagenarian

Abstract Introduction Dementia with Lewy bodies (DLB) can be associated with degeneration of the hypocretin system and reduced hypocretin levels. However, reports of DLB initially presenting with narcolepsy with cataplexy symptoms are sparse. Report of Case A 77-year-old man presents with two years of new onset hypersomnia, tremors, memory difficulties, and gait instability. Before his sleep evaluation, he was started on levetiracetam for suspected seizures and levodopa for suspected Parkinson’s disease, without improvement. During his evaluation for hypersomnia, he reported loud snoring, witnessed apneas, sleep maintenance difficulty, hypnagogic hallucinations of well-formed images, and vivid dreams with dream enactment behavior occurring almost nightly. He had witnessed episodes of cataplexy described as transient lower extremity weakness with associated bifacial weakness, inability to speak, and no loss of consciousness, self-injury, loss of bowel/bladder control, or post-event symptoms. These events completely resolved after 1-minute, occurred while sitting or standing, and were spontaneous or triggered by anger or anxiety. His Epworth Sleepiness Scale was 18 and Ullanlinna Narcolepsy Scale was 35, suggestive of narcolepsy with cataplexy. Physical examination did not reveal Parkinsonism or resting tremor. He subsequently underwent a baseline polysomnogram that showed an apnea-hypopnea index of 32.7 events/hour. During an in-laboratory titration study, he had REM sleep without atonia. A 30-minute EEG was interpreted as normal and a typical cataplexy-like event captured during continuous EEG monitoring had no ictal correlate. Despite adherence to positive airway pressure therapy, he continued to have hypersomnia, cataplexy, and visual hallucinations during transitions from wake to sleep. He was tapered off levetiracetam and levodopa with a plan for further evaluation of probable DLB. Conclusion Narcolepsy with cataplexy symptoms is an uncommon presentation of probable DLB as seen in this case with two core clinical features of DLB (RBD and visual hallucinations), suggesting neurodegeneration of the hypocretin system

Foramen ovale electrodes in the evaluation of epilepsy surgery: Conventional and unconventional uses

Foramen ovale (FO) electrodes have been used in the evaluation of epilepsy surgery for more than 25years. Their traditional application was in patients with mesial temporal lobe epilepsy. Due in part to advances in neuroimaging, their use has declined. We describe our cumulative experience with FO electrodes and use examples to illustrate a range of indications for FO recordings that extend beyond their conventional utility for mesial temporal lobe cases. We also summarize the pros and cons of FO electrodes implantation and attempt to reestablish their utility in presurgical evaluation. ► Foramen ovale electrodes (FO) have traditionally been used in patients with mesial temporal lobe epilepsy. ► We describe our experience with their use beyond their conventional utility. ► In 5years we performed 22 implantations that guided treatment with a small complication rate. ► FO may assist diagnosis of lateral temporal & extra-temporal epilepsy, epileptic pseudodementia, & nonepileptic seizures