Home video review in a pediatric clinical neurophysiology led multidisciplinary team meeting.

Objective: Assessment of the value of review of home videos by a pediatric multidisciplinary team (MDT) in a pediatric neurophysiology department.

Methods: We describe and evaluate the review of home videos alongside clinical history and previous investigations from patients referred to the Evelina pediatric EEG department at a twice-monthly MDT meeting between 01/2021 and 09/2022. We retrospectively analyzed measures of video quality, quantity and duration, time taken from referral to MDT meeting, pre-MDT and post-MDT meeting proposed diagnosis and clinical outcomes.

High throughput production of single-wall carbon nanotube fibres independent of sulfur-source.

Floating catalyst chemical vapor deposition (FC-CVD) methods offer a highly scalable strategy for single-step synthesis and assembly of carbon nanotubes (CNTs) into macroscopic textiles. However, the non-uniform axial temperature profile of a typical reactor, and differing precursor breakdown temperatures, result in a broad distribution of catalyst particle sizes. Spun CNT fibres therefore contain nanotubes with varying diameters and wall numbers.

Photonic Sorting of Aligned, Crystalline Carbon Nanotube Textiles.

Floating catalyst chemical vapor deposition uniquely generates aligned carbon nanotube (CNT) textiles with individual CNT lengths magnitudes longer than competing processes, though hindered by impurities and intrinsic/extrinsic defects. We present a photonic-based post-process, particularly suited for these textiles, that selectively removes defective CNTs and other carbons not forming a threshold thermal pathway. In this method, a large diameter laser beam rasters across the surface of a partly aligned CNT textile in air, suspended from its ends.

Tunable Broadband Radiation Generated Via Ultrafast Laser Illumination of an Inductively Charged Superconducting Ring.

An electromagnetic transmitter typically consists of individual components such as a waveguide, antenna, power supply, and an oscillator. In this communication we circumvent complications associated with connecting these individual components and instead combine them into a non-traditional, photonic enabled, compact transmitter device for tunable, ultrawide band (UWB) radiation. This device is a centimeter scale, continuous, thin film superconducting ring supporting a persistent super-current.

Controlling the optical fiber output beam profile by focused ion beam machining of a phase hologram on fiber tip.

A phase hologram was machined on an optical fiber tip using a focused ion beam (FIB) system so that a ring-shaped beam emerges from the fiber tip. The fiber used for this work was a commercial single-mode optical fiber patch cable for a design wavelength of 633 nm with a germanosilicate core. The ring-shaped beam was chosen to ensure a simple geometry in the required phase hologram, though the Gerchberg-Saxton algorithm can be used to calculate a hologram for an arbitrary beam shape.

Trihexyphenidyl for acute life-threatening episodes due to a dystonic movement disorder in Rett syndrome.

In Rett syndrome (RS), acute life-threatening episodes (ALTEs) are usually attributed to epilepsy or autonomic dysfunction but they can represent a movement disorder (MD). We describe three girls with RS who experienced ALTEs from an early age. These were long considered epileptic until video-EEG in Patients 1 and 3 revealed their non-epileptic nature.

Mechanisms involved in the conduction of anterior temporal epileptiform discharges to the scalp.

Objective: To evaluate the mechanisms involved in the conduction to the scalp of anterior temporal epileptiform discharges in temporal lobe epilepsy.

Methods: During electroencephalographic recordings extra electrodes were applied around the eyes and on the cheeks in 13 patients with anterior temporal discharges. The amplitude of at least 34 discharges per patient was compared.

Structural basis for the recognition of histone H4 by the histone-chaperone RbAp46.

RbAp46 and RbAp48 (pRB-associated proteins p46 and p48, also known as RBBP7 and RBBP4, respectively) are highly homologous histone chaperones that play key roles in establishing and maintaining chromatin structure. We report here the crystal structure of human RbAp46 bound to histone H4. RbAp46 folds into a seven-bladed beta propeller structure and binds histone H4 in a groove formed between an N-terminal alpha helix and an extended loop inserted into blade six.

Factors limiting the application of electrical impedance tomography for identification of regional conductivity changes using scalp electrodes during epileptic seizures in humans.

Electrical impedance tomography (EIT) has the potential to produce images during epileptic seizures. This might improve the accuracy of the localization of epileptic foci in patients undergoing presurgical assessment for curative neurosurgery. It has already been shown that impedance increases by up to 22% during induced epileptic seizures in animal models, using cortical or implanted electrodes in controlled experiments.

Electrical impedance tomography of human brain function using reconstruction algorithms based on the finite element method.

Electrical impedance tomography (EIT) is a recently developed technique which enables the internal conductivity of an object to be imaged using rings of external electrodes. In a recent study, EIT during cortical evoked responses showed encouraging changes in the raw impedance measurements, but reconstructed images were noisy. A simplified reconstruction algorithm was used which modelled the head as a homogeneous sphere.

Continuous bitemporal rhythmic subclinical epileptiform activity in an adult without epileptic seizures.

We report the occurrence of an unusually sustained, non-reactive, bilateral, rhythmic, subclinical discharge in a young adult without epileptic seizures

Improved conditions for the removal of 2-oxoacyl groups from the N-terminus of proteins.

Proteins with R-CO-CO-NH- at the N-terminus, rather than the usual R-CH(-NH3+)-CO-NH-, are produced by non-enzymic transamination and also occur in the pyruvoyl enzymes. The oxoacyl group may be specifically removed from a model peptide, in yields of 70-80%, by treating them in 0.1 M phosphate buffer at 37 degreesC for 24 h with 25 mM of the N-phosphonomethyl derivative of phenylene-1,2-diamine.

[Reactions of decarboxylated and side transamination during interaction of glutamate decarboxylase from Escherichia coli with substrate analogs, modified through C3 and C4 atoms].

The interaction of glutamate decarboxylase with the aspartate and glutamate analogues modified at C3 and C4 was studied. 3-Arsonoalanine, 3-phosphonoalanine, 2-amino-4-arsonobutyric acid, 2-amino-4-phosphonobutyric acid, a mixture of diastereoisomers of 4-(methylthio) glutamic acid and erythro-4-(methylthio) glutamic acid were shown to be poor substrates for the enzyme. Their decarboxylation was accompanied by transamination of the coenzyme (PLP) to pyridoxamine phosphate (PMP) which reversibly inactivated the enzyme.

Arsenite release on enzymic transformation of arsonomethyl substrate analogues: a potentially lethal synthesis by glycerol-3-phosphate dehydrogenase.

The isosteric arsenical analogue of glycerol 3-phosphate, 3,4-dihydroxybutylarsonic acid, is a good substrate for rabbit muscle glycerol-3-phosphate dehydrogenase. Its oxidation is accompanied by release of arsenite. This release seems to be due to a spontaneous elimination of arsenite by 3-oxoalkylarsonic acids, as it is also observed in (1) the oxidation of 3-hydroxypropylarsonic acid by yeast alcohol dehydrogenase, (2) treatment of 3,4-dihydroxybutylarsonic acid with periodate and (3) nonenzymic transamination of the glutamate analogue 2-amino-4-arsonobutyric acid.

The interaction of glutamate decarboxylase from Escherichia coli with substrate analogues modified at C-3 and C-4.

The interaction of glutamate decarboxylase with the aspartate analogues 3-arsonoalanine and 3-phosphonoalanine, with the glutamate analogues 2-amino-4-arsonobutyric acid and 2-amino-4-phosphonobutyric acid, and with 4-(methylthio)-L-glutamic acid, both as a mixture of diastereoisomers and as the (2S,4R)-form, was studied. All these analogues were poor substrates for the enzyme and only weak inhibitors. Their decarboxylation was accompanied by transamination of the enzyme-bound pyridoxal phosphate (PLP) to pyridoxamine phosphate (PMP), thus inactivating the decarboxylase.

A rapid spectrophotometric estimation of individual phosphates and phosphonates: its application to chromatography of sugar phosphates and their phosphonate analogues.

A compound containing the -PO3H2 group (phosphoric acid, one of its monoesters, or an alkylphosphonic acid) may be rapidly assayed by the decrease it produces in the absorbance at 450 nm of a buffered acidic solution of Fe3+ and N(-3) [corrected]. The method has been used to follow chromatograms of sugar phosphates and their phosphonomethyl analogues.

Organizational and morphological features of cat sacrocaudal motoneurons.

We have investigated the organizational and morphological features of motoneurons from cat sacrocaudal spinal cord, the portion of the neuraxis that innervates the tail. This information is pertinent for development of a new model of spinal cord injury. An understanding of sacrocaudal circuitry is essential for physiological and behavioral assessment of the effects of sacrocaudal lesions.

Lesions of cat sacrocaudal spinal cord: a minimally disruptive model of injury.

As part of our studies of the organization of the cat sacrocaudal spinal cord (S3-Ca7), the portion of the neuraxis that innervates the tail, we have begun to evaluate the behavioral effects of hemisection or complete transection at the level of Ca1. Clinical observations that the tail strongly deviated to the side of a hemisection indicated the presence of an ipsilateral hypertonia. After complete transection of the spinal cord, the tail became ventroflexed in a midline position and exhibited spasticity, i.

Descending projections to the rat sacrocaudal spinal cord.

Descending supraspinal and propriospinal neurons projecting to the female rat sacrocaudal spinal cord, the portion of the spinal cord that innervates the tail, were identified following injection of Fluoro-Gold into the S1-Ca2 spinal cord segments. This study attempted to determine anatomical substrates for propriospinal and supraspinal control of the tail. Propriospinal neurons were identified throughout laminae V-VIII and X at all levels of the spinal cord.

The preparation and properties of bromoacetylphosphonic acid.

Bromoacetylphosphonic acid, Br-CH2-CO-PO3H2, was made by brominating dimethyl acetylphosphonate and de-esterifying with HBr. It proves to be a powerful alkylating agent, reacting rapidly with GSH, with a rate constant of about 6M(-1).s(-1) at pH6.

Crossed and uncrossed projections to cat sacrocaudal spinal cord: II. Axons from muscle spindle primary endings.

The terminal fields of primary afferent fibers from tail muscle spindle primary endings were mapped within cat sacrocaudal spinal cord (S3-Ca7), using intra-axonal recording and horseradish peroxidase staining techniques. We sought to determine the ipsilateral and contralateral projection patterns and to relate these to the fibers' muscles of origin. Fifty-three group Ia fibers were successfully stained.

A synthesis of acylphosphonic acids and of 1-aminoalkylphosphonic acids: the action of pyruvate dehydrogenase and lactate dehydrogenase on acetylphosphonic acid.

Acylphosphonic acids, R-CO-PO(OH)2, have been synthesized by the steps [formula: see text] of which the last is new and provides a mild method for de-esterifying acylphosphonic acids. Their reductive amination gives a simple way of making 1-aminoalkylphosphonic acids. Acetylphosphonic acid inhibited NAD+ reduction by pyruvate with the pyruvate dehydrogenases from Escherichia coli and Bacillus stearothermophilus.

The synthesis of 3-phosphonoalanine, phosphonopyruvic acid and phosphonolactic acid. Scission of the C-P bond during diazotization of phosphonoalanine.

3-Phosphonoalanine has been made by the Strecker synthesis from phosphonoacetaldehyde, which is easily prepared from vinyl acetate. It gives phosphonopyruvate by transamination when treated with glyoxylate. Phosphonolactate, an analogue of phosphoglycerate, is prepared by reducing phosphonopyruvate.

Mapping studies of the serum cholinesterase-2 locus (CHE2).

Serum cholinesterase (butyrylcholinesterase, EC 3.1.1.

Application of the synthetic method to other amines.

Taurine and 2-aminoethylphosphonic acid were synthesized by the method of the main paper [Geoghegan & Dixon (1989) Biochem. J. 260, 295-296], i.