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The article presents a case study of patients with epilepsy, one being a 17-year-old and the other being a 35-year-old, to demonstrate the potential benefits of wearable digital health technology in managing seizures. Topics discussed include the challenges faced by individuals with epilepsy and their caregivers, the types of wearable devices available for seizure detection, and the role of these devices in improving patient safety and treatment monitoring.

Summary: The aim of this study was to develop a novel and entirely potato‐based staple bread. Bread‐making performance of individual potato components (starch, protein and fibre) were independently evaluated and optimised by their ternary mixture. Flours from yellow and purple potatoes were then incorporated up to 25% of full formula weight to maximise the utilisation of resources. Fresh tuber was further added up to 50% for the full benefits from potatoes. The excessive oven‐rising after the crust hardened was manipulated by delicately balancing the strong swelling power of potato starch, which effectively resolved the cracks on the crust. The resultant potato breads showed a typical bread appearance, loaf size, low porosity but high cell density crumb structure and unique favourable sensory qualities, especially those with 2% potato fibre or 30% fresh tuber. Potato breads staled quickly during storage, but reheating could reverse the bread staleness to a nearly freshly baked state. [ABSTRACT FROM AUTHOR]

The article describes the case of a 13-year-old female with chronic medical history of presumed Rett syndrome (RTT) based on developmental regression and behavioral observation, microcephaly, seizure disorder, and gastrostomy tube dependence. Topics discussed include findings on medical examination of the patient, clinical characteristics of Rett syndrome, and differential diagnosis, workup, and outcomes of therapeutic interventions trialed for patient's agitation.

Oven or microwave roasting and alginate encapsulation of pea flour and starch to produce novel pea ingredients for enrichment of slowly digestible starch (SDS) and resistant starch (RS) content in pea bread were investigated. Pea flour treated either by oven roasting (160 °C, 30 min) or by microwave roasting (1.1 kW, 6 min) effectively retained its low starch digestibility similar to its native form (∼25% SDS; ∼60% RS). When oven roasting was applied to pea starch, SDS content increased triply compared to the fully boiled counterpart. Alginate encapsulation effectively controlled carbohydrate release to simulated gastric, intestinal and colonic fluids, and thus largely enriched the SDS and RS fractions in starch. Pea bread containing up to 37.5% of encapsulated roasted MPS pea starch not only provided high SDS and RS fractions (23.9% SDS and 30.2% RS) compared to a white bread control (0.2% SDS and 2.5% RS), but also provided an acceptable palatability. [ABSTRACT FROM AUTHOR]

Summary: Physicochemical and nutritional properties of flour and isolated starch from eight Canadian lentil cultivars were assessed to identify unique samples and key factors affecting starch digestion. The results showed that nearly half of apparent amylose in lentil flours was underestimated because it was complexed and embedded within the flour matrix, which led to slower starch digestion of cooked flour. Cooked red lentil flours showed significantly higher resistant starch content (11.0%) than flours from green lentils (6.8%) (P < 0.05). Among green lentils, Asterix and Greenland were unique for their high slowly digestible starch content after cooking, possibly owing to their high phenolic content and α‐glucosidase inhibitory activity. Long‐ and short‐range ordering in starch was more indicative of low starch digestion for raw or cooked lentil flour rather than for isolated starch. The results suggest the flour matrix protects the starch ordered structure from enzyme hydrolysis. [ABSTRACT FROM AUTHOR]

The present work examines the ability to reactively modify a pea starch by phosphorylation in a twin-screw extruder without the loss of its granular form, in order to produce a product for functional food uses. This reaction was studied by varying formulation variables of moisture content as well as reactant and sodium sulfate concentration at 95°C and 150 rpm. The modified starch was characterized for its phosphorus content, in vitro digestibility by the Englyst method, thermal gelatinization properties, and pasting behavior. Phosphorus contents of intact starch granules as high as 0.3% were found (close to the 0.4% limit for foods), produced continuously under extrusion conditions corresponding to a residence time of approximately one minute. Higher moisture content and low sodium sulfate concentration favored higher phosphorus content but increased the chances of amylose leaching and agglomerating of the exiting granules. In all cases, the pea starch experienced some change in its gelatinization transition consistent with simultaneous heat-moisture treatment occurring along with the reaction inside the extruder. The current configuration of the process was not found suitable to major increases in resistant starch content though minor improvements in slowly digestible starch content were reported. [ABSTRACT FROM AUTHOR]

Two branched canola protein isolates (CPI) with different branching degree were prepared using bis-epoxide as a chain extender. It was found that the branching reaction may take place in any protein fraction in native CPI; furthermore, by increasing the amount of chain extender, more native protein chains in CPI were coupled to form branches. In the study of flow properties of native and branched CPI solutions, it was found that the apparent viscosities of two branched CPI remarkably increased compared to that of native CPI over all concentrations tested. At 22 °C and pH 7, native CPI solution showed slight shear thinning behavior with the increase of shear rate; however, shear thickening was observed at low shear rate for branched CPI. Furthermore, shear thickening was more pronounced with the increase of branching degree and concentration of branched CPI solution. Shear thinning behavior of native CPI became marked at pH 11; however, the shear thickening property of branched sample was weakened at high pH. The novel findings in flow properties of the branched CPI solution could be potentially applied in the food industry, such as increasing foaming property and gelling ability of food products. [ABSTRACT FROM AUTHOR]

Death in children with epilepsy is profoundly disturbing, with lasting effects on the family, community, and health care providers. The overall risk of death for children with epilepsy is about ten times that of the general population. However, the risk of premature death for children without associated neurological comorbidities is similar to that of the general population, and most deaths are related to the cause of the epilepsy or associated neurological disability, not seizures. The most common cause of seizure-related death in children with epilepsy is sudden unexpected death in epilepsy (SUDEP). SUDEP is relatively uncommon in childhood, but the risk increases if epilepsy persists into adulthood. Although the direct cause of SUDEP remains unknown, most often death follows a generalized convulsive seizure and the risk of SUDEP is strongly related to drug-resistant epilepsy and frequent generalized tonic-clonic seizures. The most effective SUDEP prevention strategy is to reduce the frequency of seizures, although a number of seizure detection devices are under development and in the future may prove to be useful for seizure detection for those at particularly high risk. There are distinct benefits for health care professionals to discuss mortality with the family soon after the diagnosis of epilepsy. An individual approach is appropriate. When a child with epilepsy dies, particularly if the death was unexpected, family grief may be profound. Physicians and other health care professionals have a critical role in supporting families that lose a child to epilepsy. This review will provide health care providers with information needed to discuss the risk of death in children with epilepsy and support families following a loss. [ABSTRACT FROM AUTHOR]

Cross-linked canola protein isolate (CPI) films were prepared using a bisepoxide as a chain extender by wet cast followed by heat compression. By DSC measurements, it was verified that the cross-linking reaction took place in the CPI matrix and was completed in 10 min at 90 °C. FTIR analysis confirmed that the chain extenders were covalently bonded with CPI chains. The reaction led to the formation of a cross-linked architecture which was revealed by an increase in molecular weight of the modified CPI films. The newly formed chain architecture increased the thermostability of CPI as evaluated by TGA; furthermore, the thermostability increased with the increase in cross-linking degree of the modified CPI films. DMA results of the unmodified CPI film showed that microcosmic phase separation occurred between two major proteins, cruciferin and napin in CPI; however, the extent of phase separation decreased with the increase of chain extenders and finally disappeared because of the increased compatibility between cruciferin and napin. Elastomeric mechanical behavior was observed with the introduction of cross-linked architecture in the modified CPI films. At low humidity of 55%, the cross-linked CPI films showed an increase in tensile strength and a decrease in elongation at break. At high humidity of 98%, the increase in hydrophobicity of cross-linked CPI matrix resisted the decreasing tensile strength seen in unmodified films. [ABSTRACT FROM AUTHOR]

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]

This study aimed to investigate effects of starch-protein interactions on physicochemical properties and in vitro starch digestibility of composite potato starch/protein blends (0, 5, 10, or 15% protein) during processing (cooking, cooling and reheating). The effect on recrystallization and short-range ordering in starch was studied by light microscopy, differential scanning calorimetry and Fourier transform infrared spectroscopy. The results show that protein in the blend proportionally restricted starch granule swelling during cooking and facilitated amylopectin recrystallization during cold-storage. The facilitating effect of protein diminished with increasing blend ratio. Resistant starch content in the processed blends was positively correlated to intensity ratio of 1053/1035 cm −1 in FTIR spectra arising from slow retrogradation of amylopectin ( r 2 > 0.88, P ≤ 0.05), whose formation was favored by the presence of protein in the blends and further enhanced by cooling of cooked blends. As a conclusion, starch-protein interaction reduced starch digestibility of the processed blends. [ABSTRACT FROM AUTHOR]

A water resistant surface was first obtained by immobilizing hydrophobic copolymers, poly (styrene- co -glycidyl methacrylate) (PSG), with functional groups on soy protein isolate (SPI) films. XPS and AFM results showed that PSG copolymers were immobilized on the film by chemical bonding, and formed a rough surface with some bumps because of the segregation of two different phases on PSG copolymers. Water resistance of the modified films could be adjusted dramatically by further immobilizing different amounts of guanidine-based antimicrobial polymers, poly (hexamethylene guanidine hydrochloride) (PHMG) on the resulting hydrophobic surface. The introduction of hydrophilic PHMG on the resulting surface generated many micropores, which potentially increased the water uptake of the modified films. Furthermore, the modified SPI films showed higher thermostability compared to native SPI film and broad-spectrum antimicrobial activity by contact killing, attributed to the presence of PHMG on the surface. The modified SPI film with a multi-functional surface showed potential for applications in the packaging and medical fields. [ABSTRACT FROM AUTHOR]

The devastating effects of sudden unexpected death in epilepsy ( SUDEP) can be difficult to navigate, even for experienced clinicians. Mounting evidence supports full disclosure of the risks of epilepsy to those affected and their caregivers, and recommendations from regulatory and professional groups encourage the same. Following a death, families are faced with tragedy, guilt, and sometimes anger. Clinicians are often called upon to provide information and support. The development of a comprehensive approach to SUDEP education requires careful consideration of the people living with epilepsy, facts about SUDEP and known risk factors, as well as experiences of families and care providers. In this article, we share the experiences of those working in SUDEP education and epilepsy care, including the voluntary sector. We explore the experience of bereaved families and clinicians, derive lessons from published research, highlight areas where more research is needed, and report on preliminary data from a nationwide study from France. [ABSTRACT FROM AUTHOR]

Abstract: Importance: Sudden unexpected death in epilepsy (SUDEP) is a common cause of mortality in patients with the disease, but it is unknown how neurologists disclose this risk when counseling patients. Objective: This study aimed at examining SUDEP discussion practices of neurologists in the U.S. and Canada. Design: An electronic, web-based survey was sent to 17,558 neurologists in the U.S. and Canada. Survey questions included frequency of SUDEP discussion, reasons for discussing/not discussing SUDEP, timing of SUDEP discussions, and perceived patient reactions. We examined factors that influence the frequency of SUDEP discussion and perceived patient response using multivariate logistic regression. Participants: The participants of this study were neurologists who completed postgraduate training and devoted >5% of their time to patient care. Results: There was a response rate of 9.3%; 1200 respondents met eligibility criteria and completed surveys. Only 6.8% of the respondents discussed SUDEP with nearly all (>90% of the time) of their patients with epilepsy/caregivers, while 11.6% never discussed it. Factors that independently predicted whether SUDEP was discussed nearly all of the time were the following: number of patients with epilepsy seen annually (OR=2.01, 95% CI=1.20–3.37, p <0.01) and if the respondent had a SUDEP case in the past 24months (OR=2.27, 95% CI=1.37–3.66, p <0.01). A majority of respondents (59.5%) reported that negative reactions were the most common response to a discussion of SUDEP. Having additional epilepsy/neurophysiology training was associated with an increased risk of a perceived negative response (OR=1.36, 95% CI=1.02–1.82, p =0.038), while years in practice (OR=0.85, 95% CI=0.77–0.95, p <0.005) and seeing both adults and children were associated with a decreased likelihood of negative response (OR=0.15, 95% CI=0.032–0.74, p =0.02). Conclusions: U.S. and Canadian neurologists rarely discuss SUDEP with all patients with epilepsy/caregivers though discussions are more likely among neurologists who frequently see patients with epilepsy or had a recent SUDEP in their practice. Perceived negative reactions to SUDEP discussions are common but not universal; more experienced neurologists may be less likely to encounter negative reactions, suggesting that there may be ways to frame the discussion that minimizes patient/caregiver distress. [Copyright &y& Elsevier]

Abstract: This study investigated synergistic impact of amylose and phosphorus on the rheological, thermal, structural and nutritional properties of native potato starch, dry starch gel and freshly cooked gel. The results indicated that starch with a high amylose level and higher phosphorus content showed enhanced retrogradation extent, associated with a well-formed and rigid gel structure and more ordered structure, compared to starch with a lower phosphorus content. Phosphorus content in starch was positively correlated to resistant starch content in native starch (p <0.001) and to the slowly digestible starch content in the starch gel (p <0.05). Amylose content alone appeared not to be a good indicator of the starch digestibility. Phosphorus content played a dominant role in some physicochemical properties of starch when the amylose content was above a threshold level. The temperature ramp test appeared to be a sensitive method to detect the re-crystallisation of starch molecules during the early stages of retrogradation. [Copyright &y& Elsevier]

Abstract: In the present study, three potato varieties were treated with chlorpropham (CIPC, 35ppm), γ-irradiated (0.1kGy) and stored for up to 5months at 8°C, and the physicochemical properties and in vitro starch digestibility of native and cooked starches were investigated. Sprouting was found to be satisfactorily suppressed by γ-irradiation and CIPC treatment. However, irradiation increased total free glucose content in two potato varieties, and decreased the thermal transition and pasting temperature of starch. The crystallinity of starch in irradiated potatoes decreased significantly (p ⩽0.05) which may explain its decreased resistant starch content. Sprout inhibiting treatments and storage had no effect on in vitro starch digestibility in cooked starches, but cooling cooked starch significantly (p ⩽0.05) increased its resistant starch content. [Copyright &y& Elsevier]

Aim The aim of this study was to determine the feasibility of undertaking visual functional magnetic resonance imaging (fMRI) in very preterm children. Method Forty-seven infants born at less than 32 weeks gestational age (25 males, 22 females; mean (SD) age at birth 28.8wks [1.9]) were scanned using 1.5T MRI as part of a longitudinal neuroimaging study. These infants were scanned at preterm age (within 2wks of birth) and at term-equivalent age. Quantitative T2* data and fMRI in response to visual stimuli (flashing strobe) were acquired in this population. T2* values were compared at preterm age and at term-equivalent age using a two-tailed t-test. A general linear model was used to evaluate occipital lobe response to visual stimuli. Results T2* values were significantly higher at preterm age than at term-equivalent age in both the medial and lateral occipital lobes (preterm infants: 187.2ms and 198.4ms respectively; term infants: 110.9ms and 133.2ms respectively; p<0.002). Significant positive occipital lobe activation ( q<0.01) was found in 3 out of 65 (5%) fMRIs carried out at preterm age and in 19 out of 26 (73%) scans carried out at term-equivalent age. Interpretation Visual stimuli do not elicit a reliable blood oxygen level-dependent (BOLD) response in very preterm infants during the preterm period. This suggests that BOLD fMRI may not be the appropriate modality for investigating occipital lobe function in very preterm infants. [ABSTRACT FROM AUTHOR]

Abstract: Amylose, the amount of which varies significantly depending on the source, is one of the key components of starch. The proportion of the essentially linear amylose and the highly branched amylopectin greatly influences the physico-chemical properties of starch. In this study, potentiometric titration using an auto-titrator and colorimetric methods were compared for determining amylose contents of a variety of starch samples. Potentiometric titration results for starches from a variety of botanical sources were within the reported literature ranges while the colorimetric method seemed to overestimate amylose content. [Copyright &y& Elsevier]

Abstract: Starch was extracted from irradiated and stored potato tubers and the properties were compared to CIPC (chlorpropham) treated tubers. The granule properties and dynamic viscoelasticity in temperature ramp and frequency sweep modes were studied while heating the samples. Starch structural characteristics were investigated by high performance anion exchange chromatography (HPAEC) and Fourier transform infrared spectroscopy (FTIR). Gamma-irradiation of potato tubers at a dosage of 0.1kGy induced some degradation of starch molecules, resulting in earlier swelling of starch granules, and greater extents of amylose and total carbohydrate leaching. The early swelling phenomenon was also enhanced with tuber storage time. The retrogradation rate and extent for a concentrated starch gel also increased with tuber storage time whereas γ-irradiation delayed the gel retrogradation. Sprout inhibiting methods could be selected based on the specific processing and texture requirements of the end products. [Copyright &y& Elsevier]

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]

Sudden death is over 20 times more frequent in people with epilepsy than the general population. The literature on clinical risk factors is now able to define individuals at the highest risk. Despite these advances in our understanding of risk, the mechanism of sudden unexpected death in epilepsy remains elusive. While it is unlikely that a single mechanism will be found to explain all deaths, there have been recent advances that identify factors that play a critical role. This review provides an update on new advances in the understanding of sudden unexpected death in epilepsy. [ABSTRACT FROM AUTHOR]

As a result of the increasingly popularity of vagal nerve stimulation (VNS) for intractable seizures, neurosurgeons not uncommonly encounter cases which require electrode revision. We examine our experience of VNS revision and reports the use of the ultra-sharp monopolar tip for safe dissection and removal of the electrode from the vagus nerve. A retrospective review was performed from January 2000 to Dec 2009 reviewed eight cases of VNS revision. The indications for VNS revision were device malfunction manifesting with increased seizures or increased impedance of the device and infection. The time from initial VNS implantation to revision ranged from 6 to 108 months (mean: 38 months). The entire VNS electrode system, was removed in seven cases and the helical coils were left in-situ in one case who did not derive any benefit from VNS and it was deemed unnecessary to subject the patient to the additional risk of vagal nerve injury. One case had dislodgement of the lower two coils and three cases had dense scarring to the vagus nerve causing high impedance and malfunction. The other three cases demonstrated no fibrotic scar tissue between the helical coils and the vagus nerve. Four cases had replacement of new VNS system but the case of infected VNS stimulator was not replaced as there was no benefit from the device. VNS revision is normally performed in cases of device malfunction or infection and can be safely performed using a combination of ultra-sharp monopolar coagulation and sharp dissection. [ABSTRACT FROM AUTHOR]

Linear nevus sebaceous syndrome is reported to occur in as many as 1 in 1000 live births, and is hypothesized to result from genetic mosaicism involving a lethal autosomal-dominant gene. The terms “epidermal nevus syndrome” and “linear nevus sebaceous syndrome” are often used interchangeably, although linear nevus sebaceous syndrome more strictly applies to patients with a typical midline nevus, and epidermal nevus syndrome is a more inclusive term that includes all varieties of epidermal nevi. Linear nevus sebaceous syndrome encompasses a broad spectrum of abnormalities that may affect every organ system, including the central nervous system. In these cases, seizures and mental retardation are the main manifestations. Many other organ systems were also reported to be involved in this syndrome, including the cardiovascular, skeletal, ophthalmologic, and urogenital systems, among others. Although linear nevus sebaceous syndrome occurs at a relatively high frequency and may affect different organ systems, many physicians are unaware of the syndrome, which may delay diagnosis and treatment. We present two cases of linear nevus sebaceous syndrome, and we describe their initial presentation and subsequent evolution, incorporating a review of the current literature in this field. [Copyright &y& Elsevier]

The article discusses focal epilepsy syndromes. Classification of focal epilepsy syndromes; Elaboration on the idiopathic focal epilepsy symptoms; Presentation of symptomatic focal epilepsy syndrome.

Neonatal seizures associated with white-matter changes on neuroimaging suggest an etiology of hypoxic-ischemic encephalopathy. Metabolic and idiopathic etiologies are also considerations but are less likely. Despite the fact that two disorders associated with neonatal seizures are diagnosed by cerebrospinal fluid neurotransmitter analysis, such an analysis is not standard in the workup for idiopathic neonatal seizures. We describe an infant who had a prolonged delivery, seizures on the first day of life, and white-matter changes on neuroimaging. A progressive seizure disorder that was refractory to standard antiepilepsy medications developed at 2 months of age. Analysis of cerebrospinal fluid neurotransmitters at that time demonstrated a pattern consistent with folinic acid-responsive seizures. Seizures ceased 24 hours after starting folinic acid. Serial neuroimaging, electroencephalograms, and metabolic changes from this patient are presented. This case illustrates the importance of cerebrospinal fluid neurotransmitter analysis as part of the work-up for idiopathic neonatal seizures. (J Child Neurol 2003;18:562-569). [ABSTRACT FROM AUTHOR]

The article features Dr. Elizabeth Donner and Tamzin Jeffs, founders of the Canadian nonprofit organization SUDEP Aware. It explains that SUDEP Aware promotes the discussion and understanding of sudden unexpected death in epilepsy (SUDEP). Donner and Tamzin has been working for 6 years already to bring support to families that are affected by SUDEP.

Sudden unexpected death in epilepsy (SUDEP) is the most tragic potential outcome of epilepsy. Despite recommendations from epilepsy organizations in the United Kingdom and the United States, many neurologists choose not to discuss the risk of SUDEP with their patients with epilepsy. Yet, the literature clearly demonstrates that people with epilepsy and their caregivers want to know more about SUDEP. When health care providers do not provide information, people with epilepsy turn to other sources, risking misinformation and potentially increasing anxiety and distress. Sharing accurate information about SUDEP can optimize epilepsy self-management and engage the person with epilepsy as a partner in their own care. Information about SUDEP must be part of the comprehensive education given to all people with epilepsy. [ABSTRACT FROM AUTHOR]

Outcomes following vagus nerve stimulation (VNS) improve over years after implantation in children with drug‐resistant epilepsy. The added value of deep brain stimulation (DBS) instead of continued VNS optimization is unknown. In a prospective, non‐blinded, randomized patient preference trial of 18 children (aged 8–17 years) who did not respond to VNS after at least 1 year, add‐on DBS resulted in greater seizure reduction compared with an additional year of VNS optimization (51.9% vs. 12.3%, p = 0.047). Add‐on DBS also resulted in less bothersome seizures (p = 0.03), but no change in quality of life. DBS may be considered earlier for childhood epilepsy after non‐response to VNS. ANN NEUROL 2024 [ABSTRACT FROM AUTHOR]

Objectives Methods Results Significance We aimed to develop consensus on comorbidities (frequency, severity, and prognosis) and overall outcomes in epilepsy, development, and cognition for the five phenotypes of SCN8A‐related disorders.A core panel consisting of 13 clinicians, 1 researcher, and 6 caregivers was formed and split into three workgroups. One group focused on comorbidities and prognosis. All groups performed a literature review and developed questions for use in a modified‐Delphi process. Twenty‐eight clinicians, one researcher, and 13 caregivers from 16 countries participated in three rounds of the modified‐Delphi process. Consensus was defined as follows: strong consensus ≥80% fully agree; moderate consensus ≥80% fully or partially agree, <10% disagree; and modest consensus 67%–79% fully or partially agree, <10% disagree.Consensus was reached on the presence of 14 comorbidities in patients with Severe Developmental and Epileptic Encephalopathy (Severe DEE) spanning non‐seizure neurological disorders and other organ systems; impacts were mostly severe and unlikely to improve or resolve. Across Mild/Moderate Developmental and Epileptic Encephalopathy (Mild/Moderate DEE), Neurodevelopmental Delay with Generalized Epilepsy (NDDwGE), and NDD without Epilepsy (NDDwoE) phenotypes, cognitive and sleep‐related comorbidities as well as fine and gross motor delays may be present but are less severe and more likely to improve compared to Severe DEE. There was no consensus on comorbidities in the SeL(F)IE phenotype but strong conesensus that seizures would largely resolve. Seizure freedom is rare in patients with Severe DEE but may occur in some with Mild/Moderate DEE and NDDwGE.Significant comorbidities are present in most phenotypes of SCN8A‐related disorders but are most severe and pervasive in the Severe DEE phenotype. We hope that this work will improve recognition, early intervention, and long‐term management for patients with these comorbidities and provide the basis for future evidence‐based studies on optimal treatments of SCN8A‐related disorders. Identifying the prognosis of patients with SCN8A‐related disorders will also improve care and quality‐of‐life for patients and their caregivers. [ABSTRACT FROM AUTHOR]

Objective Methods Results Significance We aimed to develop consensus for diagnosis/management of SCN8A‐related disorders. Utilizing a modified Delphi process, a global cohort of experienced clinicians and caregivers provided input on diagnosis, phenotypes, treatment, and management of SCN8A‐related disorders.A Core Panel (13 clinicians, one researcher, six caregivers), divided into three subgroups (diagnosis/phenotypes, treatment, comorbidities/prognosis), performed a literature review and developed questions for the modified Delphi process. Twenty‐eight expert clinicians, one researcher, and 13 caregivers from 16 countries participated in the subsequent three survey rounds. We defined consensus as follows: strong consensus, ≥80% fully agree; moderate consensus, ≥80% fully/partially agree, <10% disagree; and modest consensus, 67%–79% fully/partially agree, <10% disagree.Early diagnosis is important for long‐term clinical outcomes in SCN8A‐related disorders. There are five phenotypes: three with early seizure onset (severe developmental and epileptic encephalopathy [DEE], mild/moderate DEE, self‐limited (familial) infantile epilepsy [SeL(F)IE]) and two with later/no seizure onset (neurodevelopmental delay with generalized epilepsy [NDDwGE], NDD without epilepsy [NDDwoE]). Caregivers represented six patients with severe DEE, five mild/moderate DEE, one NDDwGE, and one NDDwoE. Phenotypes vary by age at seizures/developmental delay onset, seizure type, electroencephalographic/magnetic resonance imaging findings, and first‐line treatment. Gain of function (GOF) versus loss of function (LOF) is valuable for informing treatment. Sodium channel blockers are optimal first‐line treatment for GOF, severe DEE, mild/moderate DEE, and SeL(F)IE; levetiracetam is relatively contraindicated in GOF patients. First‐line treatment for NDDwGE is valproate, ethosuximide, or lamotrigine; sodium channel blockers are relatively contraindicated in LOF patients.This is the first‐ever global consensus for the diagnosis and treatment of SCN8A‐related disorders. This consensus will reduce knowledge gaps in disease recognition and inform preferred treatment across this heterogeneous disorder. Consensus of this type allows more clinicians to provide evidence‐based care and empowers SCN8A families to advocate for their children. [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]

Organic acids (5% lactic acid and 5% acetic acid, w/v) and biosolvents (5% ethyl lactate, 5% and 10% d‐limonene, w/v) are used as extraction agents during starch isolation from pea flour. NaOH at 0.4% w/v served as a control. The results show few differences in starch granular morphology, apparent amylose content, and most thermal parameters, and negligible differences in starch crystalline structure. However, noticeable differences are observed in protein and starch damage content, and pasting viscosities among starch isolated by different extraction agents. The starch extracted by 5% ethyl lactate has the highest protein residue (1.1%) compared to others (≤0.4%), while the starch extracted by 5% acetic acid shows the lowest pasting viscosities. d‐Limonene at 5% seems to be a promising biosolvent to isolate pea starch for its low residual protein, and no solvent residue, yet the highest pasting viscosities in starch. Fourier transform infrared spectroscopy (FTIR) detects bands of residual protein in all samples, and also unique bands at 1564, 1575, and 1720 cm−1 related to acid residue in starches extracted by the two organic acids. Chemometric analysis of FTIR spectra differentiates starches extracted by biosolvents from those extracted by alkali and acidic agents. [ABSTRACT FROM AUTHOR]

Abnormal patterns identified on electroencephalogram (EEG) are one of the primary diagnostic tests for epilepsy. However, epidemiological studies have established that both benign and epileptiform abnormalities (EAs) occur on the EEG of nonepileptic, seizure‐free people as well. The reported rates of EAs in nonepileptic, seizure‐free populations vary, and the true prevalence is unknown. The primary objective of this systematic review and meta‐analysis was to estimate the overall prevalence of EAs in the EEG of people without a history of seizures. Secondary aims were to characterize (1) the cortical localization of focal abnormalities, (2) the proportion of findings that occurred during standard EEG stimulation protocols, and (3) the persistence and implications of abnormalities at follow‐up. A comprehensive electronic search of six bibliographic databases was completed: Embase, MEDLINE, PsycInfo, Cumulative Index of Nursing and Allied Health Literature, Cochrane Central Register for Controlled Trials, and Web of Science. No search date restrictions were applied. Overall effect size was calculated using a generalized linear mixed‐effects model. Fifty‐three studies, totaling 73 990 individuals, met our inclusion criteria. The overall point prevalence of EAs was 1.74% (95% confidence interval [CI] = 1.13–2.67). Due to the risk of bias in the literature, especially from participant selection, we believe this to be an overestimate of the true prevalence. Prevalence of EAs was greater in children (2.45%, 95% CI = 1.41–4.21) and the elderly (5.96%, 95% CI = 1.39–22.13) compared with adults (.93%, 95% CI =.48–1.80). Reports of developing epilepsy after an EA‐positive EEG were rare. The likelihood of subsequent positive findings on follow‐up EEG may be as high as 50%. Our study has limitations in that males were overrepresented in the study samples, there is substantial heterogeneity among studies, and many studies provided insufficient detail about their exclusion criteria. Nonetheless, our estimates provide benchmark data for future studies examining EAs in clinical populations, particularly behavioral and psychiatric populations. [ABSTRACT FROM AUTHOR]

The present work examines the ability to reactively modify pea starch by lipid complexing in a twin‐screw extruder in order to produce a functional food product. The study considers the influence of moisture content, lipid type (myristic acid, palmitic acid) and content, in tests with differing screw designs to reduce enzymatic digestion. The modified starch was characterized for its physicochemical properties (bound lipid content, pasting properties, and Englyst digestion profiles). With near complete conversion at all tested lipid concentrations, differences found in enzyme resistance and pasting properties for the extruded samples were attributed to differences in the mixing environment. The lipid complexed pea starches under optimized conditions achieved a significant but moderate increase in either resistant starch (from 7.8% to 20%) or slowly digestible starch (from 12% to 23%) content compared to their native counterparts; however, the sought nutritional fractions (slowly digestible and resistant) could only be improved simultaneously with palmitic acid. The highest resistant fraction produced in the study corresponded to the higher shear environment tested and for complexes prepared at the highest lipid content. [ABSTRACT FROM AUTHOR]

We compared sudden unexpected death in epilepsy (SUDEP) diagnosis rates between North American SUDEP Registry (NASR) epileptologists and original death investigators, to determine degree and causes of discordance. In 220 SUDEP cases with post‐mortem examination, we recorded the epileptologist adjudications and medical examiner‐ and coroner‐ (ME/C) listed causes of death (CODs). COD diagnosis concordance decreased with NASR's uncertainty in the SUDEP diagnosis: highest for Definite SUDEP (84%, n = 158), lower in Definite Plus (50%, n = 36), and lowest in Possible (0%, n = 18). Rates of psychiatric comorbidity, substance abuse, and toxicology findings for drugs of abuse were all higher in discordant cases than concordant cases. Possible SUDEP cases, an understudied group, were significantly older, and had higher rates of cardiac, drug, or toxicology findings than more certain SUDEP cases. With a potentially contributing or competing COD, ME/Cs favored non–epilepsy‐related diagnoses, suggesting a bias toward listing CODs with structural or toxicological findings; SUDEP has no pathognomonic features. A history of epilepsy should always be listed on death certificates and autopsy reports. Even without an alternate COD, ME/Cs infrequently classified COD as "SUDEP." Improved collaboration and communication between epilepsy and ME/C communities improve diagnostic accuracy, as well as bereavement and research opportunities. [ABSTRACT FROM AUTHOR]

Cognitive flexibility encompasses the ability to efficiently shift focus and forms a critical component of goal-directed attention. The neural substrates of this process are incompletely understood in part due to difficulties in sampling the involved circuitry. We leverage stereotactic intracranial recordings to directly resolve local-field potentials from otherwise inaccessible structures to study moment-to-moment attentional activity in children with epilepsy performing a flexible attentional task. On an individual subject level, we employed deep learning to decode neural features predictive of task performance indexed by single-trial reaction time. These models were subsequently aggregated across participants to identify predictive brain regions based on AAL atlas and FIND functional network parcellations. Through this approach, we show that fluctuations in beta (12–30 Hz) and gamma (30–80 Hz) power reflective of increased top-down attentional control and local neuronal processing within relevant large-scale networks can accurately predict single-trial task performance. We next performed connectomic profiling of these highly predictive nodes to examine task-related engagement of distributed functional networks, revealing exclusive recruitment of the dorsal default mode network during shifts in attention. The identification of distinct substreams within the default mode system supports a key role for this network in cognitive flexibility and attention in children. Furthermore, convergence of our results onto consistent functional networks despite significant inter-subject variability in electrode implantations supports a broader role for deep learning applied to intracranial electrodes in the study of human attention. [ABSTRACT FROM AUTHOR]

Samples of canola meal (CM), a by-product of canola oil extraction, with different particle sizes and canola protein (CP) content were obtained by ball milling followed by air classification. In an effort to produce highly deformable "green" biodegradable materials, these CM samples were used to prepare CM bioplastics and their mechanical properties were characterized and compared. It was found that greater CP content led to an increase in elongation at break and a decrease in tensile strength; however, particle size of CM had no apparent impact on the tensile properties. Considering its high mechanical properties and miscibility with the CM matrix, cellulose fiber (CF) was added to the composite formulation to fabricate samples with enhanced tensile strength. The effects of fiber loading on mechanical and thermal properties were characterized using tensile testing and dynamic mechanical analysis. Marked improvements in tensile strength and modulus were found with increasing fiber content above 5 wt%, while mechanical properties were constant between fiber contents of 1-5 wt% because of the inefficient packing of the CF, indicating that the fiber content of 5 wt% is a percolation threshold to increase the mechanical property of the CM composite. The DMA results illustrate that the addition of fiber produced an increase in storage modulus and decreased damping factors for the composites with > 5 wt% CF, reflecting strong interfacial adhesion between CM and CF which resulted in a decrease in chain mobility. [ABSTRACT FROM AUTHOR]

Abstract Background Sudden Unexpected Death in Epilepsy (SUDEP) is a significant cause of death in childhood epilepsy, and causes considerable concern to patients and their families. Despite this, the condition remains poorly understood. This systematic review investigates the risk factors, pathophysiology, and circumstances associated with childhood SUDEP. It aimed to explore the etiology of SUDEP and inform clinicians approaching SUDEP risk disclosure. Methods A structured electronic database search of MEDLINE, CENTRAL, EMBASE, and ISI web of science was conducted. Studies were included if they described clinical details of one or more patients, aged 18 years of age and below, who had SUDEP. Two reviewers independently reviewed each article for data extraction and quality assessment. Results Information on 108 cases of pediatric SUDEP was extracted from 22 included studies. These comprised five cohort studies, four retrospective case control studies, seven case series, and five case reports. Factors that appeared to be linked to pediatric SUDEP included those associated with severe epilepsy (early age of onset, high seizure frequency, intellectual impairment and developmental delay, multiple antiepileptic drug therapy, and structural abnormalities). The majority of included studies was noncomparative and had significant risk of bias. Conclusions There is currently insufficient evidence to determine the etiology of pediatric SUDEP. Current best practice to prevent pediatric SUDEP is to optimize the management of epilepsy. A national SUDEP registry would provide invaluable high-quality data and insights into modifiable risk factors, genetic predispositions, and novel prevention strategies. Highlights • Very little is known about the pathophysiology, risk factors and circumstances associated with childhood SUDEP. • In this systematic review, 22 studies were included with information on 108 cases of pediatric SUDEP. • Factors which appeared to be linked to pediatric SUDEP included those associated with severe epilepsy. • This is the first systematic review of childhood SUDEP in the literature, drawn from analysis of the largest compilation of pediatric SUDEP cases. [ABSTRACT FROM AUTHOR]

Pullulanase (P) and ultrasonication (U) were simultaneously applied to debranch pea starch to enhance slowly digestible starch (SDS) and resistant starch (RS) fractions in the debranched pea starch (DPS). A synergistic debranching effect was found under conditions of pullulanase (40 npun/g) and ultrasonication (100% amplitude in pulse mode, 1 min on followed by 9 min off), which produced 73.5% linear glucans, 18% SDS and 26% RS in the resulting DPS-PU after 6 h of debranching. Even when autoclaving the DPS-PU at 118 °C for 30 min, following cooldown, 11% SDS and 25% RS were retained in the DPS-PU, compared with 0% SDS and 12% RS in autoclaved native pea starch. The SDS fraction in autoclaved DPS-PU further increased to 16% while the RS content remained constant during 14 days of cold storage. In summary, DPS-PU is high in linear glucans, low in starch digestibility and has a thermally stable RS fraction. [ABSTRACT FROM AUTHOR]

Children with epilepsy commonly have comorbid neurocognitive impairments that severely affect their psychosocial well‐being, education, and future career prospects. Although the provenance of these deficits is multifactorial, the effects of interictal epileptiform discharges (IEDs) and anti‐seizure medications (ASMs) are thought to be particularly severe. Although certain ASMs can be leveraged to inhibit IED occurrence, it remains unclear whether epileptiform discharges or the medications themselves are most deleterious to cognition. To examine this question, 25 children undergoing invasive monitoring for refractory focal epilepsy performed one or more sessions of a cognitive flexibility task. Electrophysiological data were recorded to detect IEDs. Between repeated sessions, prescribed ASMs were either continued or titrated to <50% of the baseline dose. Hierarchical mixed‐effects modeling assessed the relationship between task reaction time (RT), IED occurrence, ASM type, and dose while controlling for seizure frequency. Both presence (β ± SE = 49.91 ± 16.55 ms, p =.003) and number of IEDs (β ± SE = 49.84 ± 12.51 ms, p <.001) were associated with slowed task RT. Higher dose oxcarbazepine significantly reduced IED frequency (p =.009) and improved task performance (β ± SE = −107.43 ± 39.54 ms, p =.007). These results emphasize the neurocognitive consequences of IEDs independent of seizure effects. Furthermore, we demonstrate that inhibition of IEDs following treatment with select ASMs is associated with improved neurocognitive function. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 14-year-old girl and 12-year-old boy suffering from epilepsy. The girl had history of myoclonic jerks and diagnosed with juvenile myoclonic epilepsy for which she was treated with valproic acid. The boy had history of drug-resistant epilepsy, moderately severe cognitive impairment and features of autism. It further mentions their death due to seizure.

Canola meal (CM), a by-product of canola oil extraction, was modified by poly (styrene-co-glycidyl methacrylate) (PSG) and could potentially be used as a sustainable natural composite. Infrared analysis indicated that PSG could efficiently react with the proteins in CM and become covalently bonded. DSC results revealed that the modified CM composites showed new glass transition temperatures ( T g ) decreasing with the introduction of PSG, which were attributed to the disruption of intermolecular association. Torque curves and melt flow index tests showed the flow property of the modified CM composites increased due to interference by PSG of the hydrogen and disulfide bonding among the protein species. DMA results showed greater amorphous character for the modified CM composites and new glassy plateaus with increased storage moduli, indicating a change in molecular structure and enhancement in mechanical property and usability below T g . Furthermore, the thermostability, the tensile strength, and the elastic moduli of the modified CM composites increased because of the formation of cross-linked proteins in the CM composites, which will potentially increase the application value in some fields, such as single-use agricultural planting containers replacing high-impact polystyrene (HIPS) containers, and food or coffee trays. [ABSTRACT FROM AUTHOR]

To increase the usage value of canola meal (CM), a by-product of canola oil extraction, poly (styrene- co -maleic anhydride-co-glycidyl methacrylate) (PSMG) was prepared and used as a novel chain extender to modify CM. FTIR tests showed that cross-linking took place in the CM matrix and PSMG was covalent bonded with canola proteins; furthermore, the surface of fibers present in the CM composite was covered by proteins, attributed to the reactivity of anhydride groups on PSMG. The modified CM composites showed more apparent amorphous characteristic with the addition of PSMG, according to DSC results. DMA results suggested that cross-linked proteins could be further coupled by glycerol reacting with the anhydride groups remaining in the cross-linked proteins, and thus the storage moduli and the glass transition temperature ( T g ) of the modified CM composites increased with the addition of more PSMG. The reactions of proteins with proteins, proteins with fibers, and proteins with glycerol were further confirmed by TGA. SEM images showed that the CM matrix became much denser and the interfacial adhesion between fibers and the CM matrix was increased by the chemical modification, which also contributed to an increase in the tensile strength of the modified CM composites. [ABSTRACT FROM AUTHOR]

Objective: The theory of transient cognitive impairment in epilepsy posits that lapses in attention result from ephemeral disruption of attentional circuitry by interictal events. Eye movements are intimately associated with human attention and can be monitored in real time using eye‐tracking technologies. Here, we sought to characterize the associations between interictal epileptiform discharges (IEDs), gaze, and attentional behavior in children with epilepsy. Methods: Eleven consecutive children undergoing invasive monitoring with stereotactic electrodes for localization‐related epilepsy performed an attentional set‐shifting task while tandem intracranial electroencephalographic signals and eye‐tracking data were recorded. Using an established algorithm, IEDs were detected across all intracranial electrodes on a trial‐by‐trial basis. Hierarchical mixed‐effects modeling was performed to delineate associations between trial reaction time (RT), eye movements, and IEDs. Results: Hierarchical mixed‐effects modeling revealed that both the presence of an IED (β ± SE = 72.74 ± 24.21 ms, p =.003) and the frequency of epileptiform events (β ± SE = 67.54 ± 17.30 ms, p <.001) were associated with prolonged RT on the attentional set‐shifting task. IED occurrence at the time of stimulus presentation was associated with delays in gaze initiation toward the visual targets (p =.017). Significance: The occurrence of epileptiform activity in close temporal association with stimulus presentation is associated with delays in target‐directed gaze and prolonged response time, hallmarks of momentary lapses in attention. These findings provide novel insights into the mechanisms of transient impairments in children and support the use of visual tracking as a correlate of higher order attentional behavior. [ABSTRACT FROM AUTHOR]

Objective: Working memory deficits are prevalent in childhood epilepsy. Working memory processing is thought to be supported by the phase of hippocampal neural oscillations. Disruptions in working memory have previously been linked to the occurrence of transient epileptic activity. This study aimed to resolve the associations between oscillatory neural activity, transient epileptiform events, and working memory in children with epilepsy. Methods: Intracranial recordings were acquired from stereotactically implanted electrodes in the hippocampi, epileptogenic zones, and working memory‐related networks of children with drug‐resistant epilepsy during a 1‐back working memory task. Interictal epileptic activity was captured using automated detectors. Hippocampal phase and interregional connectivity within working memory networks were indexed by Rayleigh Z and the phase difference derivative, respectively. Trials with and without transient epileptiform events were compared. Results: Twelve children (mean age = 14.3 ± 2.8 years) with drug‐resistant epilepsy were included in the study. In the absence of transient epileptic activity, significant delta and theta hippocampal phase resetting occurred in response to working memory stimulus presentation (Rayleigh z‐score = 9, Rayleigh z‐score = 8). Retrieval trials that were in phase with the preferred phase angle were associated with faster reaction times (p =.01, p =.03). Concurrently, delta and theta coordinated interactions between the hippocampi and working memory‐related networks were enhanced (phase difference derivative [PDD] z‐scores = 6–11). During retrieval trials with pre‐encoding or pre‐retrieval transient epileptic activity, phase resetting was attenuated (Rayleigh z‐score = 5, Rayleigh z‐score = 1), interregional connectivity was altered (PDD z‐scores = 1–3), and reaction times were prolonged (p =.01, p =.03). Significance: This work highlights the role of hippocampal phase in working memory. We observe poststimulus hippocampal phase resetting coincident with enhanced interregional connectivity. The precision of hippocampal phase predicts optimal working memory processing, and transient epileptic activity prolongs working memory processing. These findings can help guide future treatments aimed at restoring memory function in this patient population. [ABSTRACT FROM AUTHOR]

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]