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Objective: To determine the incidence rates of sudden unexpected death in epilepsy (SUDEP) in different epilepsy populations and address the question of whether risk factors for SUDEP have been identified. Methods: Systematic review of evidence; modified Grading Recommendations Assessment, Development and Evaluation process for developing conclusions; recommendations developed by consensus. Results: Findings for incidence rates based on 12 Class I studies include the following: SUDEP risk in children with epilepsy (aged 0-17 years) is 0.22/1,000 patient-years (95% CI 0.16-0.31) (high confidence in evidence). SUDEP risk increases in adults to 1.2/1,000 patient-years (95% CI 0.64-2.32) (low confidence in evidence). The major risk factor for SUDEP is the occurrence of generalized tonic-clonic seizures (GTCS); the SUDEP risk increases in association with increasing frequency of GTCS occurrence (high confidence in evidence). Recommendations: Level B: Clinicians caring for young children with epilepsy should inform parents/guardians that in 1 year, SUDEP typically affects 1 in 4,500 children; therefore, 4,499 of 4,500 children will not be affected. Clinicians should inform adult patients with epilepsy that SUDEP typically affects 1 in 1,000 adults with epilepsy per year; therefore, annually 999 of 1,000 adults will not be affected. For persons with epilepsy who continue to experience GTCS, clinicians should continue to actively manage epilepsy therapies to reduce seizures and SUDEP risk while incorporating patient preferences and weighing the risks and benefits of any new approach. Clinicians should inform persons with epilepsy that seizure freedom, particularly freedom from GTCS, is strongly associated with decreased SUDEP risk. [ABSTRACT FROM AUTHOR]

We developed a technique to produce images of dynamic changes in ictal high-frequency oscillations (HFOs) >40 Hz recorded on subdural electroencephalography (EEG) that are time-locked to the ictal EEG and ictal semiology video. We applied this technique to Jacksonian seizures to demonstrate ictal HFO propagation along the homunculus in the primary sensory-motor cortex to visualize the underlying epileptic network. We analyzed intracranial ictal EEGs from two patients with intractable Jacksonian seizures who underwent epilepsy surgery. We calculated the degrees of increase in amplitude within 40-80, 80-200, and 200-300 Hz frequency bands compared to the interictal period and converted them into topographic movies projected onto the brain surface picture. We combined these data with the ictal EEGs and video of the patient demonstrating ictal semiology. The ictal HFOs began in the sensory cortex and appeared concomitantly with the sensory aura. They then propagated to the motor cortex at the same time that focal motor symptoms evolved. As the seizure progressed, the ictal HFOs spread or reverberated in the rolandic region. However, even when the seizure became secondarily generalized, the ictal HFOs were confined to the rolandic region. In both cases, there was increased amplitude of higher frequency bands during seizure initiation compared to seizure progression. This combined movie showed the ictal HFO propagation corresponding to the ictal semiology in Jacksonian seizures and revealed the epileptic network involved in seizure initiation and progression. This method may advance understanding of neural network activities relating to clinical seizure generation and propagation. [ABSTRACT FROM AUTHOR]

The American Epilepsy Society and the Epilepsy Foundation jointly convened a task force to assess the state of knowledge about sudden unexplained death in epilepsy (SUDEP). The task force had five charges: (1) develop a position statement describing if, when, what, and how SUDEP should be discussed with patients and their families and caregivers; (2) design methods by which the medical and lay communities become aware of the risk of SUDEP; (3) recommend research directions in SUDEP; (4) explore steps that organizations can take to perform large-scale, prospective studies of SUDEP to identify risk factors; and (5) identify possible preventive strategies for SUDEP. Some of the major task force recommendations include convening a multidisciplinary workshop to refine current lines of investigation and to identify additional areas of research for mechanisms underlying SUDEP; performing a survey of patients and their families and caregivers to identify effective means of education that will enhance participation in SUDEP research; conducting a campaign aimed at patients, families, caregivers, coroners, and medical examiners that emphasizes the need for complete autopsy examinations for patients with suspected SUDEP; and securing infrastructure grants to fund a consortium of centers that will conduct prospective clinical and basic research studies to identify preventable risk factors and mechanisms underlying SUDEP. For now, the principal effort in preventing SUDEP should be prompt and optimal control of seizures, especially generalized convulsive seizures. [ABSTRACT FROM AUTHOR]

To study the role of magnetoencephalography (MEG) in the surgical evaluation of children with recurrent seizures after epilepsy surgery. We studied 17 children with recurrent seizures after epilepsy surgery using interictal and ictal scalp EEG, intracranial video EEG (IVEEG), MRI, and MEG. We analyzed the location and distribution of MEG spike sources (MEGSSs) and the relationship of MEGSSs to the margins of previous resections and surgical outcome. Clustered MEGSSs occurred at the margins of previous resections within two contiguous gyri in 10 patients (group A), extended spatially from a margin by ≤3 cm in three patients (group B), and were remote from a resection margin by >3 cm in six patients (group C). Two patients had concomitant group A and C clusters. Thirteen patients underwent second surgeries. IVEEG was used in four patients. Six of seven patients with group A MEGSS clusters did not require IVEEG for second surgeries. Follow-up periods ranged from 0.6 to 4.3 years (mean: 2.6 years). Eleven children, including eight who became seizure-free, achieved Engel class I or II. Our data demonstrate the utility of MEG for evaluating patients with recurrent seizures after epilepsy surgery. Specific MEGSS cluster patterns delineate epileptogenic zones. Removing cluster regions adjacent to the margins of previous resections, in addition to removing recurrent lesions, achieves favorable surgical outcome. Cluster location and extent identify which patients require IVEEG, potentially eliminating IVEEG for some. Patients with remotely located clusters require IVEEG for accurate assessment and localization of the entire epileptogenic zone. [ABSTRACT FROM AUTHOR]

Purpose: To characterize the spatial and temporal course of ictal high-frequency oscillations (HFOs) recorded by subdural EEG in children with intractable neocortical epilepsy. Methods: We retrospectively studied nine children (four girls, five boys; 4–17 yr) who presented with intractable extrahippocampal localization-related epilepsy and who underwent extraoperative video subdural EEG (1000 Hz sampling rate) and cortical resection. We performed multiple band frequency analysis (MBFA) to evaluate the frequency, time course, and distribution of ictal HFOs. We compared ictal HFO changes before and after clinical onset and postsurgical seizure outcomes. Results: Seventy-eight of 79 seizures showed HFOs. We observed wide-band HFOs (∼250Hz, ∼120 electrodes) in six patients either with partial seizures alone (three patients) or with epileptic spasms (three patients). Three patients with partial seizures that secondarily generalized had wide-band HFOs (∼170 Hz) before clinical onset and sustained narrow-band HFOs (60–164 Hz) with electrodecremental events after clinical onset (∼28 electrodes). In four postoperatively seizure-free patients, more electrodes recorded higher-frequency HFOs inside the resection area than outside before and after clinical seizure onset. In five patients with residual seizures, electrodes recorded more HFOs that were of higher or equal frequency outside the surgical area than inside after clinical onset. Conclusion: For partial seizures alone and epileptic spasms, more electrodes recorded only wide-band HFOs; for partial seizures that secondarily generalized, fewer electrodes recorded wide-band HFOs, but in these seizures electrodes also recorded subsequent sustained narrow-band ictal HFOs. Resection of those brain regions having electrodes with ictal, higher HFOs resulted in postsurgical seizure-free outcomes. [ABSTRACT FROM AUTHOR]

Purpose: To understand the rapid dynamic changes of ictal intracranial high-frequency oscillations (HFOs) in neocortical epilepsy. Methods: We integrated multiple band frequency analysis and brain-surface topographic maps of HFOs from ictal subdural EEG (SDEEG) recordings. We used SDEEG to record partial seizures consisting of right-arm jerks with secondary generalization in a 17-year-old right-handed girl. We selected 20-s EEG sections that included preclinical seizure recordings. We averaged the HFO power between 60 and 120 Hz for 25 selected electrodes, made topographic maps from these averaged powers, and superimposed the maps on the brain-surface image. We filmed consecutive HFO maps at a 10-ms frame rate. Results: Before clinical seizure onset, high-power HFOs emerged at the superior portion of the left precentral gyrus, then appeared in the middle of the left postcentral gyrus, and subsequently reverberated between both regions as well as the posterior portion of the left postcentral gyrus. Right-arm extension and facial grimacing started as the HFO power decreased. As generalized tonic–clonic seizures evolved, HFO power increased but remained within the central region. Conclusions: Topographic movies of intracranial HFOs on the brain surface allow visualization of the dynamic ictal changes in neocortical epilepsy. [ABSTRACT FROM AUTHOR]

Childhood is a vulnerable time-developmentally, socially, and cognitively. Any disruption may have long-standing effects on the attainment of critical developmental milestones. The impact of ongoing seizures and epilepsy in children is significant and may result in cognitive delay, poorer educational outcomes, and lower employment rates in adulthood. The early recognition, intervention, and treatment of evolving pharmacoresistant epilepsy in children presents a unique opportunities for health care practitioners. Seizure freedom is the ultimate epilepsy treatment goal, given the broad and long-standing effects of seizures in childhood. However, even when we believe the chance of seizure freedom is low, we should not let the perceived unlikelihood of that goal limit the treatments we offer. All children with pharmacoresistant epilepsy should be evaluated at a comprehensive pediatric epilepsy center. Failing to offer state-of-the-art treatments to these children only increases the burden of epilepsy on children and their families. [ABSTRACT FROM AUTHOR]

Abstract Objective Limited evidence on the relationship between antiepileptic drug (AED) tapering and the likelihood of a seizure during an Epilepsy Monitoring Unit (EMU) admission is available, and no evidence specific to the pediatric population has been published. Our study sought to determine whether AED tapering leads to increased seizure likelihood in a pediatric EMU setting. Methods We performed a retrospective chart review of children admitted to the pediatric EMU at the Hospital for Sick Children in Toronto between June 1, 2014 and June 1, 2016. Data collected included demographics, reason for EMU referral, and epilepsy and medical characteristics. Among those with nondaily seizures, Kaplan–Meier curves were fit to compare probability of EMU seizure in those who were tapered fully from at least one AED to those not tapered. A Cox proportional hazards model was fit to evaluate this relationship after adjustment for subject sex, distance traveled to hospital, epilepsy duration, seizure frequency, time since last seizure, whether EMU referral was part of presurgical planning, magnetic resonance imaging (MRI) findings, and number of prescribed AEDs. An interaction between medication taper and number of prescribed AEDs was also included. Terms not significant at p < 0.3 were removed from the model, and the reduced model was recomputed. Results Of the 281 children included in the study, 159 had nondaily seizures. Kaplan–Meier curves indicated fully tapering at least one AED was associated with increased likelihood of seizure during EMU; however, after adjustment for confounding variables, this association was not preserved. Abnormal MRI findings, referral for presurgical evaluation, and shorter (≤ 3 months) time since last seizure were associated with increased likelihood of seizure during EMU. Significance Short-term AED tapering in pediatric patients may not be effective for increasing seizure likelihood in the EMU. Highlights • Evaluates the efficacy of AED tapering in a pediatric EMU setting • Fully tapering AED(s) in EMU patients was not associated with seizure likelihood. • Associated factors: MRI findings, presurgical referral, and time since last seizure [ABSTRACT FROM AUTHOR]

Highlights: [•] Annual percent of potentially eligible population who actually have surgery is only 1%. [•] Time from first seizure to EMU referral was approximately 4.6 years. [•] Time from EMU referral to surgery 1.6 years with iEEG and 1.1 years without iEEG. [•] Surgical patients: 95% reduced seizure frequency and 74% seizure free one year post-surgery. [ABSTRACT FROM AUTHOR]

Abstract: Objective: During seizures, intracranial EEG electrodes can record ictal muscle movements. Our purpose was to differentiate the high-frequency oscillations (HFOs) of extracranial muscle contractions from those of intracranial epileptogenic discharges. Methods: Using intracranial video-EEG (IVEEG), we recorded seizures in a 17-year-old boy with left mesial–temporal lobe epilepsy. We used multiple band frequency analysis (MBFA) to differentiate extracranial HFOs of craniofacial muscle activities from intracranial HFOs recorded ictally and interictally. Results: During 11 seizures, IVEEG showed low-amplitude fast waves (∼60Hz) starting at the left mesial–temporal electrodes. Ictal facial grimacing projected low-amplitude (∼20μV) fast waves (∼160Hz) on inferior lateral–temporal electrodes. Interictal chewing projected medium-amplitude (∼100μV) fast waves (∼140Hz) correlating to mouth movements. MBFA topographic power spectrograms revealed a sustained, consistent ictal fast-frequency band from electrodes in the seizure-onset zone and randomly scattered HFOs without a specific frequency band from ictal and interictal extracranial muscle contractions. Conclusions: MBFA power spectrograms differentiated randomly scattered muscle HFOs without a specific frequency band at electrodes close to temporal muscles from ictal epileptic HFOs with a sustained, fast-frequency band in the seizure-onset zone. Significance: The pattern and distribution of frequency power spectrograms of extracranial HFOs differ from those of intracranial HFOs. [Copyright &y& Elsevier]

Abstract: Gelastic seizures are relatively uncommon and rarely observed secondary to frontal lobe lesions. This report presents magnetoencephalography (MEG) and diffusion tensor imaging (DTI) findings in an adolescent with gelastic seizures secondary to a left anterior cingulate gyrus lesion. Ictal scalp video EEG showed bilateral frontal 4Hz theta discharges. Interictal EEG showed left fronto-temporal spikes or sharp waves. Interictal MEG showed spike sources over bilateral temporal regions. DTI and tractography delineated slightly shifted corpus callosum posterior to the lesion, unaffected uncinate and inferior longitudinal fasciculi. The patient became seizure free for 12 months after surgical excision of a pleomorphic xanthoastrocytoma in the left anterior cingulate region. In our patient, MEG and EEG did not localize the deep-seated epileptogenic zone. The combination of DTI and neurophysiologic studies, however, possibly disclosed neuronal connections within the epileptic network and indicated that epileptic discharges propagated via the uncinate fibers from the primary epileptogenic zone in the anterior cingulate region to the mesial temporal region in this case with gelastic seizures secondary to a cingulate lesion. [Copyright &y& Elsevier]