Search

ortho -Nitrosocumene ( o -NC) exhibits dynamic N,N bonding, interchanging monomer and E / Z -azodioxide dimer structures, the extent of which depends on the environment. As a solid, o -NC is a Z-dimer; in organic solvent, the monomer is favored; and in water, dimers are favored. A supramolecular assembly of o -NC is observed as a separate species by NMR in water, shown to be a novel nanometer-sized aggregate containing ∼2000 molecules.

Background and Objectives: The objective of this study was to determine patient-specific factors known proximate to the presentation to emergency care associated with the development of refractory convulsive status epilepticus (RSE) in children., Methods: An observational case-control study was conducted comparing pediatric patients (1 month-21 years) with convulsive SE whose seizures stopped after benzodiazepine (BZD) and a single second-line antiseizure medication (ASM) (responsive established status epilepticus [rESE]) with patients requiring more than a BZD and a single second-line ASM to stop their seizures (RSE). These subpopulations were obtained from the pediatric Status Epilepticus Research Group study cohort. We explored clinical variables that could be acquired early after presentation to emergency medical services with univariate analysis of the raw data. Variables with p < 0.1 were retained for univariable and multivariable regression analyses. Multivariable logistic regression models were fit to age-matched and sex-matched data to obtain variables associated with RSE., Results: We compared data from a total of 595 episodes of pediatric SE. Univariate analysis demonstrated no differences in time to the first BZD (RSE 16 minutes [IQR 5-45]; rESE 18 minutes [IQR 6-44], p = 0.068). Time to second-line ASM was shorter in patients with RSE (RSE 65 minutes; rESE 70 minutes; p = 0.021). Both univariable and multivariable regression analyses revealed a family history of seizures (OR 0.37; 95% CI 0.20-0.70, p = 0.0022) or a prescription for rectal diazepam (OR 0.21; 95% CI 0.078-0.53, p = 0.0012) was associated with decreased odds of RSE., Discussion: Time to initial BZD or second-line ASM was not associated with progression to RSE in our cohort of patients with rESE. A family history of seizures and a prescription for rectal diazepam were associated with a decreased likelihood of progression to RSE. Early attainment of these variables may help care for pediatric rESE in a more patient-tailored manner., Classification of Evidence: This study provides Class II evidence that patient and clinical factors may predict RSE in children with convulsive seizures., (© 2023 American Academy of Neurology.)

The intermolecular (monomer-dimer equilibrium) and intramolecular (C-NO and C-NMe 2 rotations) dynamics of 4-nitrosocumene ( 1a ) and 4-( N , N -dimethylamino)nitrosobenzene ( 1b ), respectively, were found to be controlled by the medium (water) and the host environment (organic capsules and cavitands). The ability of water to shift the equilibrium toward the dimer appears to result from dipolar stabilization of the polar dimer structure and has a resemblance to water's known ability to favor organic cycloaddition reactions. In an aqueous medium, a range of organic hosts selectively include only the nitrosocumene monomer 1a . Encapsulation in the octa acid duplex (OA 2 ) selects two 1a monomers rather than a dimer structure. Octa acid encapsulation also results in more restricted intramolecular C-N rotations of the guest 1b ., Competing Interests: The authors declare no competing financial interest., (© 2021 The Authors. Published by American Chemical Society.)

Objectives: To determine how continuous spike and wave during slow wave sleep (CSWS) is currently managed and to compare the effectiveness of current treatment strategies using a database from 11 pediatric epilepsy centers in the US., Study Design: This retrospective study gathered information on baseline clinical characteristics, CSWS etiology, and treatment(s) in consecutive patients seen between 2014 and 2016 at 11 epilepsy referral centers. Treatments were categorized as benzodiazepines, steroids, other antiseizure medications (ASMs), or other therapies. Two measures of treatment response (clinical improvement as noted by the treating physician; and electroencephalography improvement) were compared across therapies, controlling for baseline variables., Results: Eighty-one children underwent 153 treatment trials during the study period (68 trials of benzodiazepines, 25 of steroids, 45 of ASMs, 14 of other therapies). Children most frequently received benzodiazepines (62%) or ASMs (27%) as first line therapy. Treatment choice did not differ based on baseline clinical variables, nor did these variables correlate with outcome. After adjusting for baseline variables, children had a greater odds of clinical improvement with benzodiazepines (OR 3.32, 95%CI 1.57-7.04, P = .002) or steroids (OR 4.04, 95%CI 1.41-11.59, P = .01) than with ASMs and a greater odds of electroencephalography improvement after steroids (OR 3.36, 95% CI 1.09-10.33, P = .03) than after ASMs., Conclusions: Benzodiazepines and ASMs are the most frequent initial therapy prescribed for CSWS in the US. Our data suggests that ASMs are inferior to benzodiazepines and steroids and support earlier use of these therapies. Multicenter prospective studies that rigorously assess treatment protocols and outcomes are needed., (Copyright © 2021 Elsevier Inc. All rights reserved.)

The diagnosis of childhood absence epilepsy (CAE) is typically based on history and description of spells, supported by an office-based positive hyperventilation test and confirmed by routine electroencephalography (EEG). In the current coronavirus disease 2019 (COVID-19) pandemic, many pediatric neurologists have switched to telemedicine visits for nonemergent outpatient evaluations. We present a series of children diagnosed as having CAE on the basis of a positive hyperventilation test performed during remote televisits. Several of these children were begun on treatment for CAE prior to obtaining an EEG, with significant seizure reduction. Our series documents the feasibility of CAE diagnosis and management by telemedicine., Competing Interests: Declaration of competing interest None., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: To evaluate whether the onset of pediatric refractory status epilepticus (rSE) is related to time of day., Method: We analyzed the time of day for the onset of rSE in this prospective observational study performed from June 2011 to May 2019 in pediatric patients (1 month to 21 years of age). We evaluated the temporal distribution of pediatric rSE utilizing a cosinor analysis. We calculated the midline estimating statistic of rhythm (MESOR) and amplitude. MESOR is the estimated mean number of rSE episodes per hour if they were evenly distributed. Amplitude is the difference between MESOR and maximum rSE episodes/hour, or between MESOR and minimum rSE episodes/hour. We also evaluated the temporal distribution of time to treatment., Results: We analyzed 368 patients (58% males) with a median (p 25 - p 75 ) age of 4.2 (1.3-9.7) years. The MESOR was 15.3 (95% CI: 13.9-16.8) and the amplitude was 3.2 (95% CI: 1.1-5.3), p = 0.0024, demonstrating that the distribution is not uniform, but better described as varying throughout the day with a peak in the morning (11am-12 pm) and trough at night (11 pm-12 am). The duration from rSE onset to application of the first non-benzodiazepine antiseizure medication peaked during the early morning (2am-3 am) with a minimum during the afternoon (2 pm-3 pm) (p = 0.0179)., Conclusions: The distribution of rSE onset is not uniform during the day. rSE onset shows a 24-h distribution with a peak in the mid-morning (11am-12 pm) and a trough at night (11 pm-12am)., (Copyright © 2019 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Background and Purpose: Tuberous sclerosis complex (TSC) is a rare, genetic disease that is associated with multiple manifestations including epilepsy and autism. Self-injurious behaviors (SIBs) also occur in a subset of patients. This study used diffusion tensor imaging (DTI) in children with TSC for quantitative and volumetric analysis of brain regions that have been associated with SIB in other genetic conditions., Methods: We used DTI to compare 6 children with TSC-associated SIB and 10 children with TSC without SIB. Atlas-based analysis of DTI data and calculation of number of voxels; fractional anisotropy (FA); and mean, axial, and radial diffusivity were performed for multiple regions; DTI measures were summarized using medians and interquartile ranges and were compared using Wilcoxon rank sum tests and false discovery rates (FDRs)., Results: Analysis showed that children with TSC and SIB had reduced numbers of voxels (median) in the bilateral globus pallidus (right: 218 vs. 260, P = .008, FDR = .18; left: 222 vs. 274, P = .002, FDR = .12) and caudate nucleus (right: 712 vs. 896, P = .01, FDR = .26; left: 702 vs. 921, P = .03, FDR = .44) and reduced FA in the bilateral globus pallidus (right: .233 vs. .272, P = .003, FDR = .12; left: .223 vs. .247, P = .004, FDR = .12) and left caudate nucleus (.162 vs. .186, P = .03, FDR = .39) versus children without SIB. No other statistically significant differences were found., Conclusions: These data support a correlation between lower volumes of the globus pallidus and caudate with SIB in children with TSC., (© 2019 The Authors Journal of Neuroimaging published by Wiley Periodicals, Inc. on behalf of American Society of Neuroimaging.)

The goal of this study was to determine the value of a certified child life specialist (CCLS) on the patient and staff experiences in an epilepsy monitoring unit (EMU). We integrated a CCLS into the EMU for all children as well as adults with intellectual disability. We surveyed families to determine the impact of child life services on their stay. EMU staff completed questionnaires to determine perceived impact to their job performance from the integration of the CCLS. All of the families (pediatric and adult patients) who responded to the survey reported the presence of the CCLS improved their hospital experience. Staff reported that the CCLS improved their daily work by allowing them to focus on their assigned medical duties. This preliminary pilot study suggests that CCLS can have a strong impact on the experience of patients and staff in an EMU.

Introduction: Bottom-of-sulcus focal cortical dysplasias are an under recognized, surgically treatable cause of focal epilepsy. Resection can dramatically reduce the seizure burden for children with refractory epilepsy, or eliminate seizures altogether., Material and Methods: We report the case and present the results of multimodality evaluation of a 15-year-old young man who presented with long-standing partial epilepsy affecting his right leg, which over the years became refractory to therapy., Results: High-resolution 3T MRI images acquired as a dedicated epilepsyprotocol were initially interpreted as unremarkable. On further review by an experienced specialist aware of clinical and electroencephalographic findings, a subtle focal cortical dysplasia was identified at the bottom of a sulcus near the medial aspect of the left precentral gyrus. After confirmation of the extent of the lesion with PET and ultra-high field 7T MRI, the patient underwent cortical mapping and focal resection and remains free of seizures., Coclusions: This case emphasizes the need for a multidisciplinary approach to the evaluation of refractory focal epilepsy in children and highlights the potential role of ultra-high field 7T MRI in identifying the often subtle causative anatomic abnormalities.

Purpose of the Review: The care of children with infantile spasms is full of areas of ambiguity, despite practice parameters and randomized trials. This review is to update the current care of children with infantile spasms., Recent Findings: Several recent studies have generated new data to guide management including a randomized trial supporting combination therapy of hormone treatment and vigabatrin as an initial treatment for infantile spasms. Studies have suggested that we are not consistent in our recognition of hypsarrhythmia, while additional studies suggest that the presence or absence of this pattern may not have any predictive value. Additionally, the use of appropriate medications as well as an early diagnosis has the most impact on short-term outcomes. Children with infantile spasms benefit most from early diagnosis and early treatment with appropriate standard medications such as hormonal therapy (ACTH or prednisolone) or vigabatrin.

Electrical status epilepticus of sleep (ESES), with the activation of profuse amounts of epileptiform discharges in sleep, may lead to intractable epilepsy and neurocognitive decline in children. Numerous varied treatments including antiseizure medications, steroids, and surgery have been investigated as possible treatment options. The ketogenic diet (KD) is an additional treatment option which may add to our treatment armamentarium for ESES. The KD may theoretically improve ESES by affecting GABA systems and reducing inflammation. Clinical reports of the KD for ESES have been heterogeneous, but to date 38 children have been described in six publications. Overall, 53% had EEG improvement, 41% had>50% seizure reduction, 45% had cognitive improvement, but only 9% had EEG normalization. This review will assess the efficacy of the KD in the treatment of ESES based on known data as well as possible mechanisms of action and the need for future study., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Objectives: Migraine and epilepsy are 2 of the most common neurologic disorders in children. In this cross-sectional study we investigated a population of children with epilepsy to determine if children with a greater seizure burden or certain epilepsy syndromes had a higher risk of migraines. We also examined how often migraine is addressed and treated in a pediatric epilepsy cohort., Methods: Between January 2010 and March 2011 we distributed questionnaires regarding headache symptoms and treatment to consecutive children with epilepsy seen in clinic at Johns Hopkins Hospital (400 children were studied). Records were subsequently reviewed for seizure type, age at onset, and treatment., Results: The prevalence of migraine in our pediatric epilepsy population was 25%, which is greater than reported for children without epilepsy (3%-23%). Migraine was more prevalent in children ≥10 years (p = 0.0009), children with benign epilepsy with centrotemporal spikes (BECTS) (p = 0.003), and children with juvenile myoclonic epilepsy (JME) (p = 0.008). Migraine onset was more likely to have occurred after epilepsy was diagnosed (p = 0.0002), but was not more prevalent in those with intractable epilepsy. Only 50% of patients with weekly or greater migraines had documented discussions regarding headaches with their neurologist., Conclusion: Migraine was comorbid in one-quarter of children with epilepsy in a tertiary care center. Children who were older or who had BECTS or JME were more likely to have migraines. Migraines were infrequently addressed within the neurology clinic. It is imperative to address comorbid migraine in treating children with epilepsy.

When a child on anticonvulsant medications continues to have seizures, what other options should be considered? Over the past 100 years, dietary therapies for the treatment of intractable epilepsy have become more widely recognized, and their use has continued to expand throughout the world. An increasing number of studies has shown efficacy of these metabolic treatments in improving seizure control. Currently, 4 types of dietary therapy are available in the clinic: the classic long chain fatty acid "ketogenic" diet, the medium chain triglyceride diet, the modified Atkins diet, and the low glycemic index treatment. These therapies should be considered earlier in the treatment of intractable epilepsy because they offer a different approach to treatment that has proven efficacious, tolerable, and cost-effective., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Doose syndrome, otherwise traditionally known as myoclonic-astatic epilepsy, was first described as a unique epilepsy syndrome by Dr Hermann Doose in 1970. In 1989, the International League Against Epilepsy classified it formally as a symptomatic generalized epilepsy, and 20 years later it was renamed 'epilepsy with myoclonic-atonic seizures'. In this review, we discuss the components of this unique disorder including its incidence, clinical features, and electroencephalographic findings. Recent evidence has suggested possible genetic links to the GEFS+ (generalized epilepsy with febrile seizures plus) family, and, additionally, some children with structural brain lesions can mimic the Doose syndrome phenotype. Treatment strategies such as corticosteroids, ethosuximide, and valproate have been described as only partially effective, but newer anticonvulsants, such as levetiracetam and zonisamide, may provide additional seizure control. The most effective treatment reported to date appears to be the ketogenic diet. Prognosis is quite varied in this disorder; however, many children can have a remarkably normal neurodevelopmental outcome., (© The Authors. Journal compilation © Mac Keith Press 2010.)

This article examined the effects of maternal depression during the postpartum period (Time 1) on the later behavior problems of toddlers (Time 3) and tested if this relationship was moderated by paternal psychopathology during toddlers' lives and/or mediated by maternal parenting behavior observed during mother-child interaction (Time 2). Of the 101 mothers who participated in this longitudinal study with their toddlers, 51 had never experienced an episode of Major Depressive Disorder (MDD) and 50 had experienced an episode of MDD during the first 18 months of their toddlers' lives. Maternal depression at Time 1 was significantly associated with toddlers' externalizing and internalizing behavior problems only when paternal psychopathology was present. As predicted, maternal negativity at Time 2 was found to mediate the relationship between maternal depression at Time 1 and toddlers' externalizing behavior problems at Time 3.

A novel, potent series of indole analogs were recently developed as MR antagonists, culminating in 14. This compound represents the first MR antagonist in this class of molecules, exhibiting picomolar binding affinity and in vivo blood pressure lowering at pharmaceutically relevant doses.

Syntheses and SAR studies of 3,3-bisaryloxindole analogues provided potent mineralocorticoid receptor (MR) antagonists that were selective over other steroid nuclear hormone receptors.

This study examined relations between maternal control and evaluative feedback during the second year of life and children's mastery motivation and expressions of self-evaluative affect a year later. Participants were 75 toddlers (35 girls, 40 boys) and their mothers. Maternal controlling behavior and evaluative feedback were examined while mothers taught their 24-month-olds a challenging task. Children's mastery motivation and expressions of self-evaluative affect were assessed during easy and difficult achievement-like tasks when they were 36 months old. Maternal evaluative feedback and control style at 24 months predicted children's shame, persistence, and avoidance of mastery activities at 36 months. Specifically, negative maternal evaluations at age two related to children's later shame, especially when feedback was linked to children's actions or products; positive maternal feedback overall, as well as corrective feedback, related to children's later persistence; mothers who engaged in more autonomy-supporting control with their 2-year-olds had children who were less likely to avoid challenging activities at age 3. Children's pride at 36 months was not predicted by mothers' behavior at 24 months.

Lid loading with gold weights inserted into a submuscular pocket in the upper eyelid is a useful, simple, and effective method for the treatment of lagophthalmos in patients with temporary or permanent facial nerve palsy. The incidence of complications in our series was high. The reason for this are discussed, and methods of reducing the rate of complications are suggested.