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Objective To assess the change in inpatient and emergency department utilization and health care costs in children on the ketogenic diet for treatment of epilepsy. Methods Data on children with epilepsy initiated on the ketogenic diet (KD) Jan 1, 2000 and Dec 31, 2010 at Ontario pediatric hospitals were linked to province wide inpatient, emergency department (ED) data at the Institute for Clinical Evaluative Sciences. ED and inpatient visits and costs for this cohort were compared for a maximum of 2 years (730 days) prior to diet initiation and for a maximum of 2 years (730 days) following diet initiation. KD patient were compared to matched group of children with epilepsy who did not receive the ketogenic diet (no KD). Results Children on the KD experienced a mean decrease in ED visits of 2.5 visits per person per year [95% CI (1.5–3.4)], and a mean decrease of 0.8 inpatient visits per person per year [95% CI (0.3–1.3)], following diet initiation. They had a mean decrease in ED costs of $630 [95% CI (249–1012)] per person per year and a median decrease in inpatient costs of $1059 [IQR: 7890; p < 0.001] per child per year. Compared with the no KD children, children on the diet experienced a mean reduction of 2.1 ED visits per child per year [95% CI (1.0–3.2)] and a mean decrease of 0.6 [95% CI (0.1–1.1)] inpatient visits per child per year. Patients on the KD experienced a reduction of $442 [95% CI (34.4–850)] per child per year more in ED costs than the matched group. The ketogenic diet group had greater median decrease in inpatient costs per child per year than the matched group [p < 0.001]. Significance Patients initiated on ketogenic diet, experienced decreased ED and inpatient visits as well as costs following diet initiation in Ontario, Canada. [ABSTRACT FROM AUTHOR]

Summary: We present a child with myoclonic seizures, epileptic spasms and generalized epileptiform discharges on scalp EEG. Magnetoencephalography and coherence-phase analysis for scalp EEG suggested focal onset in the left hemisphere. Intracranial video EEG confirmed seizure onset in the left frontal lobe with ictal high-frequency oscillations. Left frontal lobectomy suppressed his seizures. Subsets of myoclonic seizures can be focal in origin despite bilaterally synchronized epileptiform discharges on scalp EEG. [Copyright &y& Elsevier]

Hippocampal sclerosis (HS) is a common surgical substrate in adult epilepsy surgery cohorts but variably reported in various pediatric cohorts. We aimed to study the epilepsy phenotype, radiological and pathological variability, seizure and neurocognitive outcomes in children with drug-resistant epilepsy and hippocampal sclerosis (HS) with or without additional subtle signal changes in anterior temporal lobe who underwent surgery. This retrospective study enrolled children with drug-resistant focal epilepsy and hippocampal sclerosis with or without additional subtle T2-Fluid Attenuated Inversion Recovery (FLAR)/Proton Density (PD) signal changes in anterior temporal lobe who underwent anterior temporal lobectomy with amygdalohippocampectomy. Their clinical, EEG, neuropsychological, radiological and pathological data were reviewed and summarized. Thirty-six eligible patients were identified. The mean age at seizure onset was 3.7 years; 25% had daily seizures at time of surgery. Isolated HS was noted in 22 (61.1%) cases and additional subtle signal changes in ipsilateral temporal lobe in 14 (38.9%) cases. Compared to the normative population, the group mean performance in intellectual functioning and most auditory and visual memory tasks were significantly lower than the normative sample. The mean age at surgery was 12.3 years; 22 patients (61.1%) had left hemispheric surgeries. ILAE class 1 outcomes was seen in 28 (77.8%) patients after a mean follow up duration of 2.3 years. Hippocampal sclerosis was noted pathologically in 32 (88.9%) cases; type 2 (54.5%) was predominant subtype where further classification was possible. Additional pathological abnormalities were seen in 11 cases (30.6%); these had had similar rates of seizure freedom as compared to children with isolated hippocampal sclerosis/gliosis (63.6% vs 84%, p=0.21). Significant reliable changes were observed across auditory and visual memory tasks at an individual level post surgery. Favourable seizure outcomes were seen in most children with isolated radiological hippocampal sclerosis. Patients with additional pathological abnormalities had similar rates of seizure freedom as compared to children with isolated hippocampal sclerosis/gliosis. • Subtle anterior temporal signal changes were noted in 38.9% children with hippocampal sclerosis (HS). • 77.8% children became seizure free after anterior temporal lobectomy with amygdalohippocampectomy. • Overall, 30.6% cases had additional pathological abnormalities. • Seizure freedom rates of children with additional pathological abnormalities similar to those with isolated HS. [ABSTRACT FROM AUTHOR]

Preclinical data report within subject modifiable ailments emerge weeks prior to SUDEP, including sleep disorders and cardiorespiratory changes; findings which support anecdotal clinical data. Here, we bridge preclinical findings with future clinical/preclinical studies, and survey whether caretakers or family members of victims noticed transient changes prior to SUDEP. The aim of this pilot study is to identify potential modifiable changes that may synergistically increase SUDEP risk for future research. A mobile electronic survey was posted on SUDEP community websites. The survey queried whether changes in seizures, sleep, physical well-being, emotional well-being, cognition, breathing, or heart rate were noticed before SUDEP. The most profound finding was that 85% of victims had multiple transient ailments prior to SUDEP. Changes in seizures (28/54), and sleep (30/58) occurred in more than 50% of the victims and represent the most influential changes identified. The second and third most influential changes were a reduction in physical well-being (25/57) and emotional well-being (26/56). Changes were observed within the last two months of life in approximately one third of the cases, and more than four months prior to SUDEP in approximately one third of cases, indicating a potential time frame for proactive preventative strategies. Respondents also noted changes in cognition (16/55), breathing (9/54) or heart rate (8/55). Data indicate these changes may be associated with increased SUDEP risk within subject. Study limitations include the responses were based on memory, there was a potential for data to be over reported, and caretakers were not prompted to observe changes a priori, thus some existing changes may have gone unnoticed. Data support the preclinical findings that transient, subclinical (i.e., not severe enough to require medical intervention), modifiable ailments may increase risk of SUDEP. This suggests that just as an epilepsy type can change over a lifetime and epilepsy type-specific treatments can reduce SUDEP risk, further personalization of SUDEP risk will improve our understanding as to whether variables contribute to risk differently across lifespan. Thus, with a dynamic capacity to change, differing factors may contribute to the distribution of risk probability within an individual at any given time. Understanding whether different combinations of transient changes are specific to epilepsy type, age, or sex needs to be determined to move the field forward in hopes of developing a personalized approach to preventative strategies. • Loved ones of victims were asked whether they noticed any changes before SUDEP. • 85% of SUDEP victims had multiple subclinical comorbidities prior to SUDEP. • Seizure worsening and sleep problems emerged in the months prior to SUDEP in more than half of the victims. • Changes in emotional and physical well-being were observed in more than 40% of SUDEP cases. • Whether changes are specific to epilepsy type needs to be determined to move towards personalized preventative strategies. [ABSTRACT FROM AUTHOR]

Objectives The risk of drowning is reported to be 15–19 times greater in people with epilepsy compared to the general population. Despite this disproportionate burden, there is limited data about the circumstances surrounding drowning deaths in people with epilepsy. This population-based case series characterizes drowning deaths in people with epilepsy. Methods Postmortem data from coroner-ordered autopsies conducted in Ontario between 2014 and 2016 were screened for cases of drowning in people with a history of seizures. Demographic information, epilepsy characteristics, and circumstances surrounding death were extracted from post mortem reports. The incidence of drowning in people with epilepsy was calculated using government estimates of the Ontario population and the number of people with epilepsy. Results Twenty-five people with epilepsy drowned during the three-year study period, giving an estimated incidence of 1.5 per 10,000 epilepsy person-years (95% CI: 0.98, 2.23). Decedents were mostly young (mean age 36 years) and without physical or developmental disability. Approximately one-third had psychiatric comorbidities. Epilepsy severity ranged from well-controlled to drug refractory. Only 3 people had alcohol or illicit drugs detected on toxicological analysis. Forty-four percent of deaths were the result of an unwitnessed drowning in a bathtub. Conclusions This population-based case series confirms people with epilepsy drown at a rate nearly ten times greater than the general population (1.5 per 10,000 epilepsy person-years compared to the estimated provincial average of 0.13 per 10,000). Drowning deaths in people with epilepsy most often occur in the bathtub. These deaths are only rarely associated with intoxication. People with epilepsy should receive counseling on the increased risk of drowning, including information regarding the significant risk associated with bathtub use, the potential protective roles of anti-epileptic drug (AED) adherence and supervision when in or around water, and the fact that all people with epilepsy remain at an increased risk of drowning regardless of their apparent seizure control. [ABSTRACT FROM AUTHOR]

Introduction The diagnosis and surgical management of pediatric patients with insular epilepsy is challenging due to variable and indistinct seizure semiology arising within the insular cortex. In addition, surgical approaches are associated with potential morbidity given the regional neurosurgical and vascular anatomy. Our institutional experience in evaluation and surgical management of insular epilepsy patients is presented. Diagnostic evaluation, surgical treatment and seizures outcomes are highlighted. Methods The institutional database for all pediatric surgical epilepsy patients was reviewed over a period of 15 years (2000–2015). Study subjects were defined as patients who had undergone surgical resection of the epileptogenic zone involving the insular cortex. Diagnostic imaging, electrophysiological studies and surgical records were assessed. Results Six (n = 6) patients with insular epilepsy were identified with a mean follow up of 2.8 years (range: 0.8–6.8 years). Mean age at surgical resection was 9.5 ± 2.5 years (range: 2.5–16 years). Majority of patients (n = 4/6, 67%) underwent more than 1 surgical procedure. Magnetoencephalography (MEG) identified insular cortex involvement presurgically in all cases. MEG cluster localization was useful in guiding invasive EEG monitoring in 5 patients and was concordant with identification of epileptogenic zone through intracranial monitoring in all 5 patients. Surgical resection of the epileptogenic zone, as delineated through MEG spike sources and invasive EEG monitoring, was associated with favorable seizure outcome in 4 of 6 patients (67%; Engel Class I). Cortical dysgenesis was identified on histopathology in 4 cases. Conclusion Diagnostic evaluation through invasive and noninvasive electrophysiological studies is critical to identify pediatric patients with insular epilepsy. Our findings suggest that MEG may help with identifying the epileptogenic zone within the insular cortex, which could be confirmed with invasive intracranial monitoring. [ABSTRACT FROM AUTHOR]

Our aim was to describe the risk factors known to be related to sudden unexpected death in epilepsy (SUDEP) that can be extracted from patients that utilizes an online seizure diary tool (SeizureTracker™). We conducted a descriptive analysis of SeizureTracker™ users across factors relevant to SUDEP risk. We also compared our app-using cohort to published SUDEP case-control studies. We report across seven risk factors from 30,813 users of SeizureTracker™ who had a median length of time using the app of 5.69 years (range from 1 month to 15 years). We found that they are at greater risk for SUDEP than groups from published studies (p <.00001) based on the risk factor of generalized tonic-clonic seizures. We demonstrated that the population using the SeizureTracker™ tool can be a valuable population for expanding investigation of SUDEP risk factors and is a first step towards establishing a large sample with a method to ascertain data prospectively that might be critical to developing a SUDEP risk algorithm. • Risk factors related to SUDEP that can be obtained from an epilepsy cohort that utilizes an online seizure diary tool. • We found that this app-using cohort is at risk for SUDEP based on number of GTCs, even more than published studies. • This is a viable approache to expanding our investigation of SUDEP risk factors. [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]

To investigate the utility of Low Density (LD) Electrical Source Imaging (ESI) to model the ictal onset zone (IOZ) for the surgical work up of children with medically refractory epilepsy. This was a retrospective review of 12 patients from a district and regional pediatric epilepsy center, who underwent focal resections between 2014 and 2019. ESI was generated using the Curry 8 software , incorporating T1 Magnetic Resonance Imaging (MRI) scans and scalp electroencephalogram (EEG) recordings. Concordance of the ictal LD-ESI localizations to the epileptogenic zone was assessed by comparing the location of the ictal LD-ESI to the focal resection margins on neuroimaging and noting the post-operative outcomes at one year. Localizations determined by ictal LD-ESI were also compared to interictal LD-ESI, positron emission tomography (FDG-PET) and interictal magnetoencephalography (MEG). Ictal ESI correctly localized the ictal onset zone in 4/6 patients, with all four being seizure free at one year. Similarly, interictal ESI localized the irritative zone in 7/9 patients with focal resections, with 6/7 being seizure free at one year. Additionally, we observed ictal ESI to be concordant to interictal ESI in 5/6 patients. Ictal ESI and interictal ESI were concordant to interictal MEG in 3/6 patients. Ictal ESI was concordant with FDG-PET in 6/7 cases. IOZ source localization through LD-ESI is a promising complementary method of assessing the epileptogenic focus in children. These findings may support the inclusion of ictal LD-ESI within the pre-surgical evaluation of children to supplement current diagnostic tools. • Ictal LD-ESI is a complementary tool to assess the epileptogenic focus in children. • Post-operative seizure freedom is likely when the presurgical data is concordant. • Ictal LD-ESI can be included in the pediatric epilepsy surgical evaluation. [ABSTRACT FROM AUTHOR]

Summary: Introduction: To identify the perceived practice among Canadian epileptologists regarding discontinuation of antiepileptic drugs (AEDs) following successful resective surgery for temporal and extratemporal surgery. Methods: We performed a survey of pediatric and adult epileptologists in Canada, using a 77-item questionnaire to explore attitudes, timing, rate of withdrawal, and factors contributing to the decision to withdraw AEDs after successful epilepsy surgery. Surveys were mailed with a postage-paid return envelope. Two subsequent surveys were mailed to non-respondents at 15 days intervals. All procedures received institutional review board approval. Results: Surveys were sent to 82 epileptologists in all the Canadian provinces. Sixty-six physicians answered the survey (80.5%), representing all epilepsy centers across Canada. The minimum seizure free period required after epilepsy surgery before withdrawing AEDs, varied substantially among responders: <6 months in 10%, 6–11 months in 21%, >1 year in 50%, >2 years in 12%, >2 years in 3% after. The most important factors influencing the decision to withdraw AEDs a negative EEG before discontinuation (71%), patients’ preferences (78%) and the presence of unilateral mesial temporal sclerosis (70%). The most important factors against reduction were the following: patients’ wishes to resume driving (67%), focal (65%) or generalized (78%) epileptiform activity on EEG after surgery, persistent isolated auras (78%), any seizures after hospital discharge (81%), and presurgical multifocal/bilateral/diffuse findings (78%). Discussion: Canadian epileptologists indicated that AED levels, EEG and MRI are typically done before discontinuing AEDs. Generally, a good candidate for stopping AEDs has focal pathology, is completely seizure free, had an anterior temporal lobe resection, complete resection of seizure focus, and has no epileptiform discharges on postoperative EEG. The data pertaining to self-reported practice styles, and actual practice may differ. [Copyright &y& Elsevier]

Summary: Introduction: Occipital lobe epilepsy (OLE) poses a diagnostic challenge to clinicians. Here, we present our experience in the surgical management of OLE in children using magnetoencephalography (MEG) in the pre-operative evaluation. Methods: Retrospective chart review was performed from 2000 to 2010 to identify patients with OLE. Patients were analyzed in two categories: isolated OLE (11 patients) and extended OLE (parietooccipital, temporooccipital, and temporoparietooccipital; 30 patients). Survival analysis and multivariate Cox proportional hazards regression were used to identify independent predictors of seizure outcome. Results: Forty-one patients with a mean follow-up of 3.1 years were identified with an overall 68% rate of satisfactory seizure outcome. Patients with extended OLE had younger ages at seizure onset and different seizure semiologies compared with those with isolated OLE. None of the latter underwent insertion of subdural grid electrodes for localization of the epileptogenic zone compared with 77% of the former (p <0.001). On multivariate analysis, the strongest independent predictor of unsatisfactory outcome was MEG dipoles in the occipital lobe contralateral to resection. Conclusion: Here, we find similar seizure outcomes for isolated and extended OLE foci despite the use of less invasive strategies for the former. Furthermore, we describe the role of MEG in evaluation, surgical planning and prognostication of children with OLE. [Copyright &y& Elsevier]

Summary: Purpose: Studies of adults who underwent temporal lobectomy for intractable temporal lobe epilepsy (TLE) demonstrated declining seizure free rates over time. Using seizure and social parameters, we followed patients who had temporal lobe surgery (TLS) in childhood to determine long-term outcomes. Methods: We identified 42 patients who underwent TLS for medically intractable epilepsy during childhood. Follow-up data were collected from 10 to 20 years after surgery (median, 12 years). We studied histopathology, seizure outcome, employment, school enrolment, and driver''s licensing. Patients or parents graded their satisfaction with TLS. Results: Number of Engel class I patients was 34 (81%) after 6months; 32 (76%) after 1 year; 30 (71%) after 5 years; and 28 (67%) at last ≥10 years follow-up. Nineteen (79%) of 24 children with tumors or cavernous angioma achieved class I outcomes in contrast to 9 (50%) of 18 children with other pathologies (p <0.05). Ten (56%) of 18 recurrent seizure patients experienced seizures within the first year; 4 required reoperation for seizure freedom. More seizure free patients (24, 86%) than residual seizure patients (8, 57%) were employed or in school (p =0.05). Twelve (63%) of 19 eligible patients obtained driver''s licenses. Twenty-three (82%) of 28 seizure free patients discontinued anticonvulsants. Surgery grading averaged “satisfied.” Class I patients reported greater satisfaction than class III/IV patients (p <0.001). Conclusion: Two-thirds of children who underwent TLS achieved seizure freedom at ≥10 year follow-up. Children with tumors or cavernous angiomas achieved better long-term outcomes than those with other histopathologies. Long-term seizure free patients were most often satisfied with surgery and employed or in school. [Copyright &y& Elsevier]