Objective
The evaluation to determine candidacy and treatment for epilepsy surgery in persons with drug‐resistant epilepsy (DRE) is not uniform. Many non‐invasive and invasive tests are available to ...ascertain an appropriate treatment strategy. This study examines expert response to clinical vignettes of magnetic resonance imaging (MRI)–positive lesional focal cortical dysplasia in both temporal and extratemporal epilepsy to identify associations in evaluations and treatment choice.
Methods
We analyzed annual report data and a supplemental epilepsy practice survey reported in 2020 from 206 adult and 136 pediatric epilepsy center directors in the United States. Non‐invasive and invasive testing and surgical treatment strategies were compiled for the two scenarios. We used chi‐square tests to compare testing utilization between the two scenarios. Multivariable logistic regression modeling was performed to assess associations between variables.
Results
The supplemental survey response rate was 100% with 342 responses included in the analyses. Differing testing and treatment approaches were noted between the temporal and extratemporal scenarios such as chronic invasive monitoring selected in 60% of the temporal scenario versus 93% of the extratemporal scenario. Open resection was the most common treatment choice; however, overall treatment choices varied significantly (p < .001). Associations between non‐invasive testing, invasive testing, and treatment choices were present in both scenarios. For example, in the temporal scenario stereo‐electroencephalography (SEEG) was more commonly associated with fluorodeoxyglucose–positron emission tomography (FDG‐PET) (odds ratio OR 1.85; 95% confidence interval CI 1.06–3.29; p = .033), magnetoencephalography (MEG) (OR 2.90; 95% CI 1.60–5.28; p = <.001), high density (HD) EEG (OR 2.80; 95% CI 1.27–6.24; p = .011), functional MRI (fMRI) (OR 2.17; 95% CI 1.19–4.10; p = .014), and Wada (OR 2.16; 95% CI 1.28–3.66; p = .004). In the extratemporal scenario, choosing SEEG was associated with increased odds of neuromodulation over open resection (OR 3.13; 95% CI 1.24–7.89; p = .016).
Significance
In clinical vignettes of temporal and extratemporal lesional DRE, epilepsy center directors displayed varying patterns of non‐invasive testing, invasive testing, and treatment choices. Differences in practice underscore the need for comparative trials for the surgical management of DRE.
Childhood epilepsies are a heterogeneous group of conditions that differ in diagnostic criteria and management and have dramatically different outcomes. Despite increasing data on treatment of ...epilepsy, research findings on childhood epilepsy are more limited and many clinical questions remain unanswered, so that clinicians must often rely on clinical judgment. In such clinical situations, expert opinion can be especially helpful.
A survey on pediatric epilepsy and seizures (33 questions and approximately 650 treatment options) was sent to 57 European physicians specializing in pediatric epilepsy, 42 (74%) of whom completed it. In some questions, the experts were asked to recommend overall treatment approaches for specific syndromes (the order in which they would use certain strategies). Most of the questions asked the experts to rate options using a modified version of the RAND 9-point scale for medical appropriateness. Consensus was defined as a non-random distribution of scores by chi-square test, with ratings used to assign a categorical rank (first line/usually appropriate, second line/equivocal, and third line/usually not appropriate) to each option.
Valproate was treatment of choice for symptomatic myoclonic and generalized tonic-clonic seizures. For initial monotherapy for complex partial seizures, carbamazepine and oxcarbazepine were treatments of choice, with valproate also first line. As initial therapy for infantile spasms caused by tuberous sclerosis, viagabatrin was treatment of choice. As initial therapy for infantile spasms that are symptomatic in etiology, vigabatrin was also treatment of choice, with adrenocorticotropic hormone (ACTH) and prednisone other first-line options. As initial therapy for Lennox-Gastaut syndrome, valproate was treatment of choice. For acute treatment of a prolonged febrile seizure or cluster of seizures, rectal diazepam was treatment of choice. Valproate was treatment of choice as preventive therapy for febrile seizures. For benign childhood epilepsy with centro-temporal spikes, valproate was treatment of choice. For childhood and juvenile absence epilepsy, valproate was treatment of choice, with lamotrigine another first-line option (ethosuximide was another first-line option for childhood absence epilepsy). For juvenile myoclonic epilepsy in adolescent males, valproate was treatment of choice, with lamotrigine another first-line option; for juvenile myoclonic epilepsy in adolescent females, lamotrigine was treatment of choice, with valproate another firstline option. As initial therapy for neonatal status epilepticus, intravenous (IV) phenobarbital was treatment of choice. As initial therapy for all types of pediatric status epilepticus, IV diazepam was treatment of choice. For generalized tonic-clonic status epilepticus, rectal diazepam and IV lorazepam were also treatments of choice; for complex partial status epilepticus, IV lorazepam was another first-line option.
The expert panel reached consensus on many treatment options. Within the limits of expert opinion and with the understanding that new research data may take precedence, the experts' recommendations provide helpful guidance in situations where the medical literature is scant or lacking. The information in this report should be evaluated in conjunction with evidence-based findings.
The single equivalent current dipole (sECD) is the standard clinical procedure for presurgical language mapping in epilepsy using magnetoencephalography (MEG). However, the sECD approach has not been ...widely used in clinical assessments, mainly because it requires subjective judgements in selecting several critical parameters. To address this limitation, we developed an automatic sECD algorithm (AsECDa) for language mapping.
The localization accuracy of the AsECDa was evaluated using synthetic MEG data. Subsequently, the reliability and efficiency of AsECDa were compared to three other common source localization methods using MEG data recorded during two sessions of a receptive language task in 21 epilepsy patients. These methods include minimum norm estimation (MNE), dynamic statistical parametric mapping (dSPM), and dynamic imaging of coherent sources (DICS) beamformer.
For the synthetic single dipole MEG data with a typical signal-to-noise ratio, the average localization error of AsECDa was less than 2 mm for simulated superficial and deep dipoles. For the patient data, AsECDa showed better test-retest reliability (TRR) of the language laterality index (LI) than MNE, dSPM, and DICS beamformer. Specifically, the LI calculated with AsECDa revealed excellent TRR between the two MEG sessions across all patients (Cor = 0.80), while the LI for MNE, dSPM, DICS-event-related desynchronization (ERD) in the alpha band, and DICS-ERD in the low beta band ranged lower (Cor = 0.71, 0.64, 0.54, and 0.48, respectively). Furthermore, AsECDa identified 38% of patients with atypical language lateralization (i.e., right lateralization or bilateral), compared to 73%, 68%, 55%, and 50% identified by DICS-ERD in the low beta band, DICS-ERD in the alpha band, MNE, and dSPM, respectively. Compared to other methods, AsECDa's results were more consistent with previous studies that reported atypical language lateralization in 20-30% of epilepsy patients.
Our study suggests that AsECDa is a promising approach for presurgical language mapping, and its fully automated nature makes it easy to implement and reliable for clinical evaluations.
•CWE are vulnerable to supply chain disruptions due to natural disasters.•Many CWE ran out of antiseizure medications during a winter storm in Texas in 2021.•Better disaster preparation for ...vulnerable populations are needed for the future.
In February 2021 a series of winter storms caused power outages for nearly 10 million people in the United States, Northern Mexico and Canada. In Texas,the storms caused the worst energy infrastructure failure in state history, leading to shortages of water, food and heat for nearly a week. Impacts on health and well-being from natural disasters are greater in vulnerable populations such as individuals with chronic illnesses, for example due to supply chain disruptions. We aimed to determine the impact of the winter storm on our patient population of children with epilepsy (CWE).
We conducted a survey of families with CWE that are being followed at Dell Children’s Medical Center in Austin, Texas.
Of the 101 families who completed the survey, 62% were negatively affected by the storm. Twenty-five percent had to refill antiseizure medications during the week of disruptions, and of those needing refills, 68% had difficulties obtaining the medications, leading to nine patients—or 36% of those needing a refill—running out of medications and two emergency room visits because of seizures and lack of medications.
Our results demonstrate that close to 10% of all patients included in the survey completely ran out of antiseizure medications, and many more were affected by lack of water, heat, power and food. This infrastructure failure emphasizes the need for adequate disaster preparation for vulnerable populations such as children with epilepsy for the future.
Background. Childhood epilepsies are a heterogeneous group of conditions that differ in diagnostic criteria and management and have dramatically different outcomes. Despite increasing data on ...treatment of epilepsy, research findings on childhood epilepsy are more limited and many clinical questions remain unanswered, so that clinicians must often rely on clinical judgment. In such clinical situations, expert opinion can be especially helpful.Methods. A survey on pediatric epilepsy and seizures (33 questions and 645 treatment options) was sent to 41 U.S. physicians specializing in pediatric epilepsy, 39 (95%) of whom completed it. In some questions, the experts were asked to recommend overall treatment approaches for specific syndromes (the order in which they would use certain strategies). Most of the questions asked the experts to rate options using a modified version of the RAND 9-point scale for medical appropriateness. Consensus was defined as a non-random distribution of scores by chisquare test, with ratings used to assign a categorical rank (first line/usually appropriate, second line/equivocal, and third line/usually not appropriate) to each option.Results. Valproate was treatment of choice for symptomatic myoclonic and generalized tonic-clonic seizures except in the very young, with lamotrigine and topiramate also first line (usually appropriate). Zonisamide was first line only if the child also has myoclonic seizures. For initial monotherapy for complex partial seizures, oxcarbazepine and carbamazepine were treatments of choice, with lamotrigine and levetiracetam also first line. As initial therapy for infantile spasms caused by tuberous sclerosis, viagabatrin was treatment of choice, with adrenocorticotropic hormone (ACTH) also first line. As initial therapy for infantile spasms that are symptomatic in etiology, ACTH was treatment of choice, with topiramate also first line As initial therapy for Lennox-Gastaut syndrome, valproate was treatment of choice, with topiramate and lamotrigine also first line. For acute treatment of a prolonged febrile seizure or cluster of seizures, rectal diazepam was treatment of choice. For benign childhood epilepsy with centro-temporal spikes, oxcarbazepine and carbamazepine were treatments of choice, with gabapentin, lamotrigine, and levetiracetam also first line. For childhood absence epilepsy, ethosuximide was treatment of choice, with valproate and lamotrigine also first line. For juvenile absence epilepsy, valproate and lamotrigine were treatments of choice. For juvenile myoclonic epilepsy in adolescent males, valproate and lamotrigine were treatments of choice, with topiramate also first line; for juvenile myoclonic epilepsy in adolescent females, lamotrigine was treatment of choice, with topiramate and valproate other first-line options. As initial therapy for neonatal status epilepticus, intravenous phenobarbital was treatment of choice, with intravenous lorazepam or fosphenytoin also first line. As initial therapy for all types of pediatric status epilepticus, lorazepam was treatment of choice, with intravenous diazepam also first line. For generalized tonic-clonic status epilepticus, rectal diazepam and fosphenytoin were also first line; for complex partial status epilepticus, fosphenytoin was also first line; and for absence status epilepticus, intravenous valproate was also first line.Conclusion. The expert panel reached consensus on many treatment options. Within the limits of expert opinion and with the understanding that new research data may take precedence, the experts' recommendations provide helpful guidance in situations where the medical literature is scant or lacking. The information in this report should be evaluated in conjunction with evidence-based findings. (J Child Neurol 2005;20:Sl-S56)
It is well documented that children with autistic spectrum disorder (ASD) have an increased prevalence of seizures; however, studies have not been done to evaluate the prevalence of ASD in children ...with epilepsy. This comorbidity is important to define as early diagnosis and intervention in some children with ASD has been shown to improve outcome.
Method: Children with epilepsy seen in a tertiary care epilepsy clinic were evaluated using validated autism screening questionnaires (ASQ). In addition, questions about sleep‐related disorders, behavior, seizure characteristics, antiepileptic agents, and body mass index (BMI) were requested. An attempt was then made to determine if there was a correlation between the factors identified and ASD.
Results: Of the 107 questionnaires returned, 97 ASQ's were properly completed and used in this study. Approximately 32% of children fit the ASQ criteria for having ASD. Most children had not been previously diagnosed. Worst behavior and daytime sleepiness was seen in those at greater risk (p < 0.01). Seizures also occurred earlier (approximately 2 years) in children at risk of having ASD.
Conclusion: Though confirmatory diagnostic evaluations are needed, this questionnaire‐based study suggests that children with epilepsy are at greater risk of having ASD, and illustrates the need for more clinical vigilance. Behavioral difficulties and daytime sleepiness identified in these children could potentially affect their ability to learn. It is of interest that the age of seizure onset identified in those at greater risk corresponds with the approximate age of regression identified in some children with ASD.
Abstract
BACKGROUND
Pediatric refractory epilepsy affects quality of life, clinical disability, and healthcare costs for patients and families.
OBJECTIVE
To show the impact of surgical treatment for ...pediatric epilepsy on healthcare utilization compared to medically treated pediatric epilepsy over 5 yr.
METHODS
The Pediatric Health Information System database was used to conduct a cohort study using 5 published algorithms. Refractory epilepsy patients treated with antiepileptic medications (AEDs) only or AEDs plus epilepsy surgery between 1/1/2008 and 12/31/2014 were included. Healthcare utilization following the index date at 2 and 5 yr including inpatient, emergency department (ED), and all epilepsy-related visits were evaluated. The propensity scores (PS) method was used to match surgically and medically treated patients. PS. SAS® 9.4 and Stata 14.0 were used for data management and statistical analysis.
RESULTS
A total of 2106 (17.1%) and 10186 (82.9%) were surgically and medically treated. A total of 4050 matched cases, 2025 per each treated group, were included. Compared to medically treated patients, utilization was reduced in the surgical group: at 2 and 5 yr postindex date, there was a reduction of 36% to 37% of inpatient visits and 47% to 50% of ED visits. The total number (inpatient, ED, ambulatory visits) of epilepsy-associated visits were reduced by 39% to 43% in the surgical group compared to the medically treated group. In those who had surgery, the average reduction in AEDs was 16% at 2 and 5 yr after treatment.
CONCLUSION
Patients with refractory epilepsy treated with surgery had significant reductions in healthcare utilization compared with patients treated only with medications.
Graphical Abstract
Graphical Abstract
MRI-guided thermal ablation is a relatively new technique utilising heat to ablate both tumours and epileptogenic lesions. Its use against epilepsy offers some patients a new and relatively safe way ...of reducing or aborting seizures. Most cases of MRI-guided thermal ablation have been performed in patients with isolated lesions. Placement of depth electrodes prior to laser ablation has been rarely performed. We present a case with bilateral independent lesions traversing eloquent cortex, which, after sampling for seizures and successful ablation, retained normal function. The patient is, to date, seizure-free.
Symptomatic intracranial hemorrhage is a rare complication of laser interstitial thermal therapy (LITT).
To present a unique case of delayed symptomatic intracranial hemorrhage occurring after a LITT ...procedure for epilepsy in a high-volume center (68 LITT procedures for pediatric epilepsy have been performed).
An 18-year-old male with epilepsy underwent LITT to an area of heterotopia near the right lateral ventricle. He did well initially and was discharged home on postoperative day 1 but returned on postoperative day 9 with headache and left hemiparesis. He was found to have intraparenchymal and intraventricular hemorrhage in the region of the LITT catheter tract.
CT angiography on admission revealed a small vascular abnormality near the focus of hemorrhage suspicious for pseudoaneurysm, although conventional angiography was negative. The patient declined neurologically and underwent craniotomy and hemorrhage evacuation. He eventually convalesced and was discharged to inpatient rehabilitation with persistent left hemiparesis. He has been seizure free since the intervention but remains on antiepileptic drugs.
Evidence from the literature suggests that the pathophysiology of symptomatic hemorrhage after LITT may be related to vascular injury and pseudoaneurysm formation from LITT catheter placement and/or thermal injury from the ablation itself.
Abstract
BACKGROUND
Various studies suggest that the insular cortex may play an underappreciated role in pediatric frontotemporal/parietal epilepsy. Here, we report on the postsurgical outcomes in 26 ...pediatric patients with confirmed insular involvement by depth electrode monitoring.
OBJECTIVE
To describe one of the largest series of pediatric patients with medically refractory epilepsy undergoing laser interstitial thermal therapy (LITT) or surgical resection of at least some portion of the insular cortex.
METHODS
Pediatric patients in whom invasive insular sampling confirmed insular involvement and who subsequently underwent a second stage surgery (LITT or open resection) were included. Complications and Engel Class outcomes at least 1 yr postsurgery were compiled as well as pathology results in the open surgical cases.
RESULTS
The average age in our cohort was 10.3 yr, 58% were male, and the average length of follow-up was 2.43 ± 0.20 (SEM) yr. A total of 14 patients underwent LITT, whereas 12 patients underwent open resection. Complications in patients undergoing either LITT or open resection were mostly minimal and generally transient. Forty-three percent of patients who underwent LITT were Engel Class I, compared to 50% of patients who underwent open insular resection.
CONCLUSION
Both surgical resection and LITT are valid management options in the treatment of medically refractory insular/opercular epilepsy in children. Although LITT may be a less invasive alternative to craniotomy, further studies are needed to determine its noninferiority in terms of complication rates and seizure freedom, especially in cases of cortical dysplasia that may involve extensive regions of the brain.
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