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The clinical, radiologic, neurophysiologic, and pathologic findings in 10 children with histologically proven Alexander's disease are described, and the presence of two broad clinical subgroups is confirmed. Macrocephaly, regression, and seizures are found in the infantile form, whereas bulbar signs predominate the so-called juvenile form. None of the clinical or neurophysiologic findings is pathognomonic, and radiologic features suggesting the diagnosis are not present in all cases. However, the combination of abnormalities may suggest the diagnosis and justify histologic confirmation. ( J Child Neurol 1993;8:134-144).

The tantalizing problem of the “quenching” of the Coulomb sum rule observed in medium weight nuclei is investigated in light nuclei at high momentum transfer. Inclusive electron scattering cross sections for 3,4He have been measured in the quasielastic region at electron energies between 0.9 GeV and 4.3 GeV, and scattering angles of 15° and 85°. Longitudinal (RL) and transverse (RT) response functions have been extracted using a Rosenbluth separation at constant ‖ q ‖ of 1.050 (GeV/c). The ratio of the longitudinal to the transverse reduced response functions in the negative y region reaches unity. The experimental Coulomb sum rule although with large uncertainty and conversly to the case of medium weight nuclei, saturates at high momentum to the He nucleus total charge namely Z=2.

Understanding the molecular details of CO(2)-sorbent interactions is critical for the design of better carbon-capture systems. Here we report crystallographic resolution of CO(2) molecules and their binding domains in a metal-organic framework functionalized with amine groups. Accompanying computational studies that modeled the gas sorption isotherms, high heat of adsorption, and CO(2) lattice positions showed high agreement on all three fronts. The modeling apportioned specific binding interactions for each CO(2) molecule, including substantial cooperative binding effects among the guest molecules. The validation of the capacity of such simulations to accurately model molecular-scale binding bodes well for the theory-aided development of amine-based CO(2) sorbents. The analysis shows that the combination of appropriate pore size, strongly interacting amine functional groups, and the cooperative binding of CO(2) guest molecules is responsible for the low-pressure binding and large uptake of CO(2) in this sorbent material.

Endometrial and colorectal cancers are the most common cancers in Lynch syndrome. Consensus guidelines recommend annual endometrial biopsy (EMB) and regular colonoscopies. We assessed the feasibility of concurrently performing EMB and colonoscopy and evaluated women's perception of pain, satisfaction, and acceptability.From July 2002 to December 2009, women who had a gene mutation for Lynch syndrome, met the Amsterdam II criteria, or had a high-risk situation that required screening were prospectively enrolled. After conscious sedation, the procedures were sequentially performed. Patients completed pre- and postprocedure questionnaires assessing pain, level of satisfaction, and acceptability. The Wilcoxon rank test and Mann-Whitney test were used to compare pain scores.Forty-two women completed the study. Median age was 37 years (range, 25 to 73). Nineteen had previously had an EMB in the office setting. Women reported significantly lower median levels of pain in the combined procedure compared with previous office setting biopsies (P.001). Regardless of parity, women reported significantly less pain for an EMB as part of the combined screen compared with an office EMB (parous, P = .003; nulliparous, P = .026). Women also reported a high level of satisfaction and more convenience in the combined procedure. All participants preferred combined to separately scheduled procedures and would recommend the combined procedure to their relatives.Combined colon and endometrial cancer screening is a patient-centered approach that is feasible, acceptable, and may improve adherence to Lynch syndrome screening recommendations.

When a patient presents with wheezing, pulmonary embolism is not usually considered as a possible cause. However, undoubtedly bronchoconstriction can be caused by pulmonary emboli and occasionally wheezing may be so obvious as to suggest a diagnosis of bronchial asthma. Eleven cases are reported in which wheezing was attributable to recurrent pulmonary emboli and one in which it was a clamant feature after a single embolic incident.

An elastic beam suspended horizontally over a substrate in liquid electrolyte was subjected to electric, osmotic, and van der Waals forces. The problem, which is governed by four non-dimensional parameters, was solved using the finite element method. The sum of the electric and osmotic forces, the electrochemical force, is usually attractive. However, the electrochemical force can be repulsive for a narrow range of the ion concentration, the initial separation and surface potentials. Furthermore, the beam deflection is not a monotonic function of the applied surface potentials, the bulk ion concentration, or the initial separation between the beam and the substrate. As these parameters are increased monotonically, the beam bends up and then down. The pull-in voltage increases as the bulk ion concentration increases. The pull-in voltage of a double-wall carbon nanotube suspended over a graphite substrate in liquid can be less than or greater than the pull-in voltage in air, depending on the bulk ion concentration. The critical separation between the DWCNT and the substrate increases with the bulk ion concentration. However, for a given bulk ion concentration, the critical separation is independent of the electric potentials. Furthermore, the critical separation is approximately equal in liquid and air.

Inclusive electron scattering cross sections for {sup 3,4}He have been measured in the quasielastic region at electron energies between 0.9 and 4.3 GeV, and scattering angles of 15{degree} and 85{degree}. Longitudinal ({ital R}{sub {ital L}}) and transverse ({ital R}{sub {ital T}}) response functions have been extracted using a Rosenbluth separation at constant {vert bar}{bold q}{vert bar} of 1.050 GeV/{ital c}. The ratio of the longitudinal to the transverse reduced response functions in the negative {ital y} region reaches unity.

Summary A detailed comparative seroepidemiological study of antibody responses was performed in 271 pigeon fanciers and 100 farmers. Overall 73 % of pigeon fanciers had IgG antibodies at a titre ⩾ 16 to Chlamydia pneumoniae , 39% to Chlamydia psittaci , and 6·6% to Chlamydia trachomatis . The prevalence of chlamydial antibodies was significantly lower in the farmers at 47% for C. pneumoniae , 6% for C. psittaci , and 2% for C. trachomatis . Both populations were exposed to complex microbiological and antigenic environments: 50·5 % of the pigeon fanciers had antibodies to pigeon antigens, 34% to egg membrane, and 0·73% to yolk sac antigen, and 59% of the farmers had antibodies to Micropolyspora faeni , but the high prevalence of chlamydial antibodies could not be attributed to interactions with these antigens. There was considerable overlap of chlamydial antibody responses in the pigeon fanciers but not in the farmers: 36 % of the pigeon fanciers had antibodies to C. pneumoniae alone, 5% to C. psittaci alone, and 31% to both agents, whereas only 3% of farmers had antibodies to both C. pneumoniae and C. psittaci . The high prevalence of antibodies to C. psittaci in the pigeon fanciers is compatible with the known avian reservoir for this infection. The particularly high prevalence of antibodies to C. pneumoniae suggests that complex interactions may be occurring in a population exposed to two chlamydial organisms, whereby infection with one species may provoke an anamnestic response against other chlamydial organisms with which the subject has previously been infected.

We study the critical behaviour of the chiral phase transition of SU(3) lattice QCD with one species of staggered fermions in the strong coupling limit. We find a second-order phase transition which seems to be controlled by an effective action which is in the same universality class as three-dimensional O(2) spin models. In particular, we find for the exponent-delta, 0.18 < 1/delta < 0.25, in good agreement with the three-dimensional O(2) value, 1/delta = 0.21.

This article examines the concept of sustainable competitive advantage in the context of two theoreticalframeworks: environmental determinism (which encompasses macroeconomic and industrial organization traditions) and "strategic selection" (which incorporates Schumpeterian economic and strategic choice perspectives). It is argued that by ascribing competitive advantage to industry/market imperatives, the I/O-based model apparently overlooks the idiosyncratic competencies that potentially generate a sustainable competitive advantage for the firm. An alternative conceptualization of sustainable competitive advantage from a resource-based perspective is offered. Specifically, a systems model that integrally links four components of a firm's "distinctive competencies" (managerial competencies and strategic focus, resource-based, transformation-based, and outputbased competencies) is proposed.

Although the norm of formal, impersonal leader-subordinate relationships often prevails in contemporary organizations, it is proposed that the leadership role may be enhanced by the development of closer, less formal relationships. Using the Leader-Subordinate Friendship Questionnaire (LSFQ), the managerial dilemma of balancing such relationships is addressed. The questionnaire provides a tool for integrating the concept of leader-subordinate friendship into organizational behavior theory and practice by developing a forum for classroom discussion that can be applied to all levels of instruction, including both academic and consulting environments.

We systematically surveyed period variations of superhumps in SU UMa-type dwarf novae based on newly obtained data and past publications. In many systems, the evolution of the superhump period is found to be composed of three distinct stages: an early evolutionary stage with a longer superhump period, a middle stage with systematically varying periods, and a final stage with a shorter, stable superhump period. During the middle stage, many systems with superhump periods of less than 0.08 d show positive period derivatives. We present observational characteristics of these stages and give greatly improved statistics. Contrary to an earlier claim, we found no clear evidence for a variation of period derivatives among different superoutbursts of the same object. We present an interpretation that the lengthening of the superhump period is a result of the outward propagation of an eccentricity wave, which is limited by the radius near the tidal truncation. We interpret that late-stage superhumps are rejuvenated excitation of a 3:1 resonance when superhumps in the outer disk are effectively quenched. The general behavior of the period variation, particularly in systems with short orbital periods, appears to follow a scenario proposed in Kato, Maehara, and Monard (2008, PASJ, 60, L23). We also present an observational summary of WZ Sge-type dwarf novae. Many of them have shown long-enduring superhumps during a post-superoutburst stage having longer periods than those during the main superoutburst. The period derivatives in WZ Sge-type dwarf novae are found to be strongly correlated with the fractional superhump excess, or consequently with the mass ratio. WZ Sge-type dwarf novae with a long-lasting rebrightening or with multiple rebrightenings tend to have smaller period derivatives, and are excellent candidates for those systems around or after the period minimum of evolution of cataclysmic variables. © 2009. Astronomical Society of Japan.

We conducted a multicentre, randomized, double-blind, placebo-controlled, parallel group study to determine the efficacy of controlled release salbutamol (SCR) tablets in clinically stable patients with predominantly non-reversible chronic airflow obstruction (CAO). Patients received either 8 mg SCR or matching placebo tablets twice daily for 6 weeks. Fifty-eight patients (30 on SCR and 28 on placebo) completed the study. Outcome measurements included FEV 1 , FVC, 6-min walking distance, and patient assessment of efficacy recorded at the clinic visits and twice daily PEFR measurements plus daytime symptom score recorded in diary cards. The absolute change between baseline and 6-week measurements with SCR was compared to that with placebo. Favourable trends were observed with SCR for all variables tested except FVC. The only significant difference between SCR and placebo was in FEV 1 where there was a small net change of 95 ml in favour of SCR. We conclude, therefore, that the effects of lung function of long-term bronchodilator therapy with SCR in patients with stable non-reversible CAO are slight, and are likely to be of limited clinical benefit.

Formation of alpha helices from disordered polypeptides depends on the degree to which amino acids favour the helical state. The folding of helical oligopeptides can be modelled by two parameters: sigma which reflects helix initiation and s which reflects propagation of a pre-existing helix and measures helical bias. Scheraga has reported s values for oligopeptides of about 1.1, implying a weak helical bias for amino-acid residues. By contrast, certain helical peptides studied by Baldwin seem to require much larger s values for alanine. Resolution of this inconsistency requires experiments that disentangle the ease of propagation from that of initiation. In this study varying lengths of polyalanine are linked to a 'template' that initiates helical structure and permits study solely of propagation. We report here that the s value for alanine in water is close to 1, supporting the earlier results of Scheraga but not the more recent results of Baldwin.

Inclusive electron-scattering cross sections have been measured for ^(56)Fe in the quasielastic region at electron energies between 0.9 and 4.3 GeV, at scattering angles of 15° and 85°. Longitudinal and transverse response functions at a q of 1.14 GeV/c have been extracted using a Rosenbluth separation. The experimental Coulomb sum has been obtained with aid of an extrapolation. The longitudinal response function, after correction for Coulomb distortion, is lower than quasifree-scattering-model predictions at the quasielastic peak and on the high-ω side.

We report results on the static quark potential in two-flavor full QCD. The calculation is performed for three values of lattice spacing $a^{-1}\approx 0.9, 1.3$ and 2.5 GeV on $12^3{\times}24, 16^3{\times}32$ and $24^3{\times}48$ lattices respectively, at sea quark masses corresponding to $m_\pi/m_\rho \approx 0.8-0.6$. An RG-improved gauge action and a tadpole-improved SW clover quark action are employed. We discuss scaling of $m_{\rho}/\sqrt{\sigma}$ and effects of dynamical quarks on the potential., Comment: LATTICE98(spectrum), 3 pages, 4 figures

Rotylenchulus reniformis is a major problem confronting cotton production in the central part of the cotton belt of the United States of America. In this study, the hypothesis that natural antagonists in some cases are responsible for unusually low densities of the nematode in certain fields was tested by assaying soils from 22 selected fields for the presence of transferable agents in pots containing cotton plants. In one field, soil from four different depth ranges was tested. In the first of two types of assays, 1 part nematode infested soil was added to 9 parts test soil that was left untreated or autoclaved before mixing; this mixture was used to fill pots. In the second type of assay, 1 part test soil was added to 9 or 19 parts pasteurized fine sand, and nematodes were introduced in aqueous suspension. In three experiments representing both types of assay, transferable or autoclavable agent(s) from four fields in South Texas suppressed nematode populations by 48, 78, 90 and 95%. In one experiment, transferable agents in five fields in Louisiana suppressed populations from 37 to 66%. Identification and evaluation of these agents for biological control of R. reniformis merits further study.

I discuss the issue of the “transition” between hadronic and quark degrees of freedom. I review the pertinent existing world data concerning this issue, particularly in low energy deuteron photodisintegration, including new unpublished results.

Leptin secretion exhibits a pulsatile, circadian pattern and may play a role in reproduction. No previous studies have compared leptin secretory burst characteristics in normal eumenorrheic women and women with polycystic ovary syndrome (PCOS) who are appropriately matched for body mass index (BMI). To determine if leptin secretory burst characteristics and/or the relationships of BMI, insulin, or testosterone to these characteristics differ between PCOS and normal women, we studied 9 normal eumenorrheic women and 9 women with PCOS. Each woman underwent blood sampling every 10 minutes for 24 hours to measure leptin and insulin under controlled conditions. Leptin secretory bursts were identified and characterized using multiparameter deconvolution procedures (Deconv), and the 24-hour periodicity of leptin was characterized with cosinor analysis. Relationships between BMI, area under the curve (AUC) insulin, and testosterone and leptin secretory burst characteristics in PCOS and normal women were sought using linear regression. There were no significant differences in mean serum leptin concentrations or in secretory burst characteristics between PCOS and normal women. Although the 24-hour serum leptin concentration correlated with BMI in both normal and PCOS women, leptin secretory burst mass correlated with BMI only in normal women. Similarly, the 24-hour serum leptin concentration correlated with serum insulin AUC in both normal and PCOS women; but insulin AUC correlated with leptin burst mass only in normal women. Although there was a strong trend toward a correlation between both mean 24-hour serum leptin concentration and leptin secretory burst mass with the serum testosterone concentration in normal women, such trends were not seen in PCOS women. Both normal and PCOS women exhibited a diurnal rhythm of leptin secretion with the peak occurring at night. However, neither the peak amplitude nor the timing of the peak amplitude differed between normal and PCOS women. The presence of strong relationships between BMI and insulin with both mean serum leptin and leptin secretory burst mass in normal women as opposed to PCOS women suggests that the mechanisms subserving leptin secretion differ in these 2 groups.

Neurophysiological studies of sleep have increasingly focused on underlying dynamic processes. This would appear particularly relevant to the developmental aspects of sleep. Involvement of sleep-dependent mechanisms in emotional processing, as well as perceptual-sensory, perceptual-motor, and cognitive learning, mostly studied in adults, may play major roles in development. Rapid eye movement (REM) sleep, which is generated by complex neuronal interactions within the pontine reticular formation, and non-REM sleep, which arises from activities in the thalamocortical network, are specifically implicated in different aspects of long-term memory systems. They evolve from previous physiological and behavioural states which can be traced back to the fetal period. Further studies are needed to clearly identify functions reflected by hallmarks of sleep stages, such as spindles and K complexes. A better understanding of the maturational aspects of sleep should provide important insights into physiological development. Assessment approaches taking dynamic characteristics of sleep into account may contribute to the design of better targeted management of sleep-related problems in neurodevelopmental conditions., Journal Article, Review, FLWIN, info:eu-repo/semantics/published

Introduction A large number of drugs from a variety of pharmacological classes have been demonstrated to cause adverse effects on cardiac rhythm, including the life-threatening arrhythmia Torsades de Pointes . These side effects are often associated with prolongation of the QT interval and are mediated via blockade of the human ether-a-go-go related gene (hERG) encoded potassium channel. In order to manage this risk in the pharmaceutical industry it is desirable to evaluate QT prolongation as early as possible in the drug discovery process. Methods Here we describe the development of a 384-well fluorescence polarization (FP) binding assay compatible with high-throughput assessment of compound blockade of the hERG channel during the lead optimisation process. To characterise the fluorescent ligand that was developed, competition binding studies, kinetic studies and electrophysiology studies were performed. Furthermore, to validate the assay as a key screening method a series of competition binding studies were performed and correlated with functional data obtained via patch-clamp. Results Evaluation of the assay indicates that high quality data is obtained ( Z ′ > 0.6), that the K i values determined are equivalent to more traditional radiometric methods and that it is predictive for functional hERG blockade as assessed by patch clamp. Discussion Whilst FP assays, utilizing a variety of fluors, have become well established for the evaluation of G-protein-coupled receptor (GPCRs) and kinase ligand interactions, this technique has not been applied widely to the study of ion channels. Therefore, this represents a novel assay format that is amenable to the evaluation of thousands of compounds per day. Whilst other assay formats have proven predictive or high throughput, this assay represents one of few that combines both attributes, moreover it represents the most cost effective assay, making it truly amenable to early assessment of hERG blockade.

The authors examined the relationship between team type (consultative or substantive); job characteristics; and the outcome variables of job satisfaction, satisfaction with team processes and activities, and team commitment. The present results supported the hypothesis that job characteristics mediate the relationship between team type and team commitment and partially mediate the relationship between team type and job satisfaction and satisfaction with team processes and activities. However, contrary to the hypothesis, consultative team members perceived the content of their jobs to be more enriched and reported higher levels of satisfaction and commitment than did substantive team members.

Organizations that implement strategic energy management programs have the potential to achieve sustained energy savings if the programs are carried out properly. A key opportunity for achieving energy savings that plant managers can take is to determine an appropriate level of energy performance by comparing the plant performance with that of similar plants in the same industry. Manufacturing plants can set energy efficiency targets by using performance-based indicators. The U.S. Environmental Protection Agency (EPA), through its ENERGY STAR{reg_sign} program, has been developing plant energy performance indicators (EPIs) to encourage a variety of U.S. industries to use energy more efficiently. This report describes work with the cement manufacturing industry to provide a plant-level indicator of energy efficiency for assembly plants that produce a variety of products, including Portland cement and other specialty cement products, in the United States. Consideration is given to the role that performance-based indicators play in motivating change; the steps needed to develop indicators, including interacting with an industry to secure adequate data for an indicator; and the actual application and use of an indicator when complete. How indicators are employed in the EPA's efforts to encourage industries to voluntarily improve their use of energy is discussed as well. The report describes the data and statistical methods used to construct the EPI for cement manufacturing plants. Individual equations are presented, as are the instructions for using them in an associated Excel spreadsheet.

Video telemetry in a 15-year-old boy with moderate learning difficulties revealed episodes of staring and cessation of activity, followed by sudden stiffening of the body for several seconds, abduction of the arms and a brief vocal utterance ("ugh"). Each episode lasts around 30 seconds, 3-4 times/day despite treatment. The EEG showed generalized 3-4Hz spike-wave discharges during the "absence" period followed immediately by a run of fast polyspikes typical of a tonic seizure, terminating in a run of 1-2 Hz sharp-slow wave complexes. Although tonic-absence seizures have been reported rarely, the clinical sequence appears to be reversed in this patient, with the occurrence of "absence-tonic" attacks. We present video documentation of such attacks and discuss their nosology.[Published with video sequences].

Equilibrium and energy analyses were performed for an electrostatic actuator consisting of two plane parallel electrodes, operated using DC voltages, separated by a liquid electrolyte. One electrode is fixed, and the other electrode is connected to a spring and is free to move. The mechanical equilibrium includes the spring force, the van der Waals force, and the electrochemical force as given by the solution of the linearized Poisson-Boltzmann equation. The electrode separation is determined as a function of the applied potential, the natural (i.e., zeta) potential, the Debeye length, the initial electrode separation, the spring constant, and the Hamaker constant. The actuator exhibits the classical "pull-in" instability. The natural potential increases the critical applied potential but does not significantly affect the critical separation. For zero natural potential, the spring constant does not affect the critical separation. Ratios of the maximum spring energy, the maximum van der Waals energy, and the maximum electrochemical energy were plotted as functions of the Hamaker constant and the initial electrode separation.

Extrinsic allergic alveolitis (also known as hypersensitivity pneumonitis) is caused by repeated inhalation of mainly organic antigens by sensitized subjects. This induces a hypersensitivity response in the distal bronchioles and alveoli and subjects may present clinically with a variety of symptoms. The aims of this review are to describe the current concepts of the immunological response, the diverse clinical presentation of this disease, the relevant investigations and management, and areas for future studies.

An in vitro semiquantitative reactive metabolite detection assay is described that incorporates NADPH-supplemented human liver microsomes, a novel quaternary ammonium glutathione analogue conjugating agent (QA-GSH), and liquid chromatography-tandem mass spectrometry (LC-MS/MS) for detection. The assay was developed to have high sample capacity and the potential for high sample throughput. MS/MS detection is selective and sensitive for the QA-GSH conjugating agent and semiquantitation of QA-GSH-reactive metabolite conjugates is performed using QA-GSH standards added to samples prior to analysis [i.e., internal standards (ISs)]. The reactive metabolite trapping capability of the free thiol group in QA-GSH was assessed using model drugs acetaminophen, clozapine, and flutamide, which are bioactivated to afford reactive metabolites. MS signal responses of equimolar amounts of QA-GSH standards were compared to assess the feasibility of using a QA-GSH IS approach to semiquantify reactive metabolite levels in vitro. The full scan Q1 MS response for each standard was within 3.3-fold of one another even though the "parent" moiety structure of each QA-GSH conjugate standard differed significantly. Standard curve analysis using selected reaction monitoring for each QA-GSH standard gave slope values that differed by only 1.5-fold. The QA-GSH IS semiquantitation method was tested by determining the level of QA-GS-acetaminophen conjugate formation at three different concentrations of acetaminophen and comparing the results to those from linear regression of authentic standards. The calculated levels of conjugate formed compared closely with those calculated from linear regression data of authentic standard curves. These results show that the QA-GSH semiquantitation assay described herein is a viable method for semiquantitatively assessing the bioactivation potential in vitro and is well-suited for use in early drug discovery high throughput screening paradigms.

This paper addresses a relatively simple method of measuring Young's modulus of electroplated nickel using Atomic Force Microscope. Thin layer of nickel to be measured is electroplated onto the tip side of AFM silicon cantilever, whose Young's modulus and the geometric dimensions are defined from manufacturer. The resonant frequency and the quality factor of the electroplated AFM cantilever are measured by the tapping mode of AFM and its spring constant is calculated using Sader's method. The spring constant of the electroplated cantilever is also calculated by using the laminar composite beam theory. Comparing two spring constants, Young's modulus of the electroplated nickel is determined. The measured elastic modulus of nickel in each time step is in the range of between and the average elastic modulus is with relative uncertainty of less than 5%Copyright © 2006 by ASME

Contact resistance can be influenced by a number of different parameters including autodoping of the underlying substrate from dielectric films, incomplete removal of damaged Silicon, etch depth into the underlying substrate and subsequent contact area with the diffused region. Evaluation of the effect of different etch chemistries, etch times and resist strip chemistries on the contact resistances of a number of processes took place. The effect of each of the variables is quantified from the experimental results and an explanation for the behaviour proposed.

Status epilepticus refractory to sequential trials of multiple medication is a rare but significant problem in children. We describe stimulus sensitivity arising during the treatment of convulsive status epilepticus in children (stimulus-sensitive burst-spiking in burst-suppression). We reviewed retrospectively clinical and EEG features in six children (three months to ten years), with status epilepticus requiring intensive care, in whom tactile, auditory and visual stimulation induced myoclonic jerks and bursts of EEG spikes. Sensitivity was not present at onset, but appeared after 24 hours as myoclonic jerks of the eyes, face and limbs, irrespective of the modality and site of stimulation. These were associated with burst-suppression in the EEG, the induced spiking forming the burst component. Various antiepileptic drugs, including GABAergic and NMDA blockers had no effect, but halogenated agents (used in two patients) abolished the sensitivity. Two children died, but the remainder returned to their previous clinical state. We conclude that stimulus sensitivity may appear in the context of refractory status epilepticus treated with high-dose barbiturates. Outcome may be more favorable than previously reported in adults, mostly in the context of post-anoxic or toxic coma. Evaluation of ventilated children in status epilepticus should include electroclinical assessment using sensory stimulation. If present, the drug regime should be reviewed and halogenated agents considered.

Aseries of practitioner-focused educational meetings held across the UK and facilitated by Johnson and Johnson, raised a number of issues pertinent to delegates

Angelman syndrome is one of the most studied human diseases related to a gene that is expressed on the maternal chromosome only in at least some brain cells. It is caused by inactivation of the UBE3A gene in the brain due to various abnormalities of the 15q11-q13 chromosome inherited from the mother. It is characterized by severe developmental delay, seizures, virtual absence of speech, motor impairment, and a particular behavioral phenotype. Studies of cortical, electromyographic and cerebellar electrophysiology in patients with Angelman syndrome and a mouse model revealed unique rhythmic neurophysiological activities in the cerebral cortex, cerebellar cortex, and muscles. The oscillatory patterns may be linked to molecular pathophysiology of the syndrome involving dysregulation of synaptic neurotransmission through UBE3A-related modulation of functional GABAA receptor complexes., Journal Article, FLWIN, info:eu-repo/semantics/published

The proximity of the alpha-amine and beta-thiol of alpha-amino terminal-cysteine (NT-Cys) residues in peptides imparts unique chemical properties that have been exploited for inter- and intra-molecular ligation of unprotected peptides obtained through both synthetic and biological means. A reversible protecting group orthogonal to other protection strategies and reversible under mild conditions would be useful in simplifying the synthesis, cleavage, purification and handling of such NT-Cys peptides. It could also be useful for the sequential ligation of peptides. To this end, we explored tri-one chemistry and found that ninhydrin (indane-1,2,3 trione) reacted readily with cysteine or an NT-Cys-containing peptide on- or off-resin at pH 2-5 to form Ninhydrin-protected Cys (Nin-Cys) as a thiazolidine (Thz). The Thz ring, protecting both the amino and thiol groups in Nin-Cys, completely avoids the formylation and Thz side reactions found during hydrofluoric acid (HF) cleavage when N-pi-benzyloxymethyl histidine groups are present. Nin-Cys is stable during coupling reactions and various cleavage conditions with trifluoroacetic acid or HF, but is deprotected under thiolytic or reducing conditions. These properties enable a facile one-step deprotection and end-to-end-cyclization reaction of Nin-Cys peptides containing C-terminal thioesters.

Two children with an unusual gait disorder, one combined with acquired aphasia, in association with focal epilepsy are reported. Both children also showed paroxysmal "dystonic" phenomena, and a clear therapeutic response to corticosteroids. This newly described condition widens the range of discrete, recoverable defects of cerebral function that are associated with epilepsy in the developing nervous system and suggests that the site of action is at a functionally combined bilateral motor/sensory level of the cerebral cortex.

Coupling between the electric field, magnetic field, and strain of composite materials is achieved when electro-elastic (piezoelectric) and magneto-elastic (piezomagnetic) particles are joined by an elastic matrix. Although the matrix is neither piezoelectric nor piezomagnetic, the strain field in the matrix couples the E field of the piezoelectric phase to the B field of the piezomagnetic phase. This three-phase electro-magneto-elastic composite should have greater ductility and formability than a two-phase composite in which E and B are coupled by directly bonding two ceramic materials with no compliant matrix. A finite element analysis and homogenization of a representative volume element is used to determine the effective electric, magnetic, mechanical, and coupled-field properties of an elastic (epoxy) matrix reinforced with piezoelectric and piezomagnetic fibers. The effective magnetoelectric moduli of this three-phase composite are, however, less than the effective magnetoelectric coefficients of a two-phase piezoelectric/piezomagnetic composite, because the epoxy matrix is not stiff enough to transfer significant strains between the piezomagnetic and piezoelectric fibers.

Modeling ionic diffusion in electrolytes requires the simultaneous solution of the Nernst-Planck (electro-diffusion) equation and Gauss' law. Unfortunately, the Nernst-Planck equation is not applicable in the ionic double layer that forms at an electrode/electrolyte interface. Furthermore, the large gradients of the electric potential in the double layer can cause numerical instabilities. The double layer is usually modeled using the Gouy-Chapman theory, a steady state solution, which predicts an exponential decay of the electric potential and ion concentration in the direction normal to the electrode. Therefore it is necessary to match, at some arbitrary location, the electric potential and ion concentration from the two theories, and solve two sets of field equations. In the present paper we present a novel theory in which the Gouy-Chapman equation, a three-dimensional theory, is replaced by an interface (2-D) theory of the double layer. The effects of the double layer are then modeled as boundary conditions applied to the Nemst-Planck equation and Gauss' law. Interfacial equations are derived for the species mass balances, the conservation of charge, Gauss's law, and the quasi-static form of Faraday's law. Each of these physical principles is derived for both a regular (or single) interface and a double interface representing an electric double layer. The standard interfacial variables are augmented with an electric charge, electric potential, electric field, electric polarization, and electric displacement, whereas conventional electrostatics includes only interfacial charge.