Prognostic factors in epilepsy with eyelid myoclonia (Jeavons syndrome).

PURPOSE: To describe the prognostic factors of drug resistance in 40 patients with epilepsy with eyelid myoclonia or Jeavons syndrome. METHOD: Retrospective analysis from two French tertiary centers. RESULTS: Forty patients were enrolled (31 females and 9 males; mean age at epilepsy onset: 6.2±3.4 years [range: 1-15 years]). Half of the patients (20/40) achieved at least a one-year remission from all seizure types. In the responders, seizure freedom was achieved after a mean 13.85±13.43 years from the onset of epilepsy (range: 1-44). The presence of intellectual disability and an earlier onset of the disease (≤5 years) were the most powerful predictors of poor seizure control (P=0.003 and P=0.005, respectively). When considering the age of onset, patients with early-onset seizures (≤5 years) presented more frequently with intellectual disabilities, psychiatric comorbidities, absences, and a major risk of refractoriness (70% versus 30%, P=0.01) than patients with onset after 5 years. At the last follow-up, 15 patients (37.5%) were taking a single drug, 16 (40%) were taking two, and seven (17.5%) were taking more than two. The most frequent drugs were valproate (23/40, 57.7%), followed by levetiracetam (16/40, 40%), and lamotrigine (14/40, 35%). CONCLUSION: Patients with Jeavons syndrome present a high rate of pharmaco-resistance with the need for long-term treatment. Early onset of epilepsy and the presence of intellectual disability appeared to be the most relevant predictors of poor seizure control, suggesting the use of genetic tests to individualize specific etiologies and perhaps adapt the therapeutic strategy.

Role of a corrugated Dion-Jacobson 2D perovskite as an additive in 3D MAPbBr3 perovskite-based light emitting diodes.

Metal halide perovskites represent an intriguing class of materials, and a very promising approach to tune the properties of optoelectronic devices and improve their performance involves the implementation of architectures based on mixed 3D and 2D perovskites. In this work, we investigated the use of a corrugated 2D Dion-Jacobson perovskite as an additive to a classical 3D MAPbBr3 perovskite for applications in light-emitting diodes. Taking advantage of the properties of this emerging class of materials, we studied the effect of a 2D 2-(dimethylamino)ethylamine (DMEN)-based perovskite on the morphological, photophysical, and optoelectronic properties of 3D perovskite thin films. We used α-DMEN perovskite both in a mixture with MAPbBr3 creating mixed 2D/3D phases and as a passivating thin layer deposited on the top of a 3D perovskite polycrystalline film. We observed a beneficial modulation of the thin film surface, a blue shift in the emission spectrum, and enhanced device performance.

Polariton Bose-Einstein condensate from a bound state in the continuum.

Bound states in the continuum (BICs)1-3 are peculiar topological states that, when realized in a planar photonic crystal lattice, are symmetry-protected from radiating in the far field despite lying within the light cone4. These BICs possess an invariant topological charge given by the winding number of the polarization vectors5, similar to vortices in quantum fluids such as superfluid helium and atomic Bose-Einstein condensates. In spite of several reports of optical BICs in patterned dielectric slabs with evidence of lasing, their potential as topologically protected states with theoretically infinite lifetime has not yet been fully exploited. Here we show non-equilibrium Bose-Einstein condensation of polaritons-hybrid light-matter excitations-occurring in a BIC thanks to its peculiar non-radiative nature, which favours polariton accumulation. The combination of the ultralong BIC lifetime and the tight confinement of the waveguide geometry enables the achievement of an extremely low threshold density for condensation, which is reached not in the dispersion minimum but at a saddle point in reciprocal space. By bridging bosonic condensation and symmetry-protected radiation eigenmodes, we reveal ways of imparting topological properties onto macroscopic quantum states with unexplored dispersion features. Such an observation may open a route towards energy-efficient polariton condensation in cost-effective integrated devices, ultimately suited for the development of hybrid light-matter optical circuits.

Lacosamide in monotherapy in BTRE (brain tumor-related epilepsy): results from an Italian multicenter retrospective study.

PURPOSE: Lacosamide (LCM) is a third-generation anti-seizure medication (ASM) approved for focal onset epilepsy in patients aged ≥ 4.378 Previous studies have reported an efficacy of LCM as add-on treatment in brain tumor-related epilepsy (BTRE). To date, there are no studies in the literature focusing on lacosamide used in monotherapy to treat BTRE. In our retrospective study we investigated efficacy and tolerability of LCM in monotherapy in a multicenter national cohort of primary brain tumor patients. METHODS: We collected from 12 Italian Centers 132 patients with primary brain tumors who were treated with LCM in monotherapy. For each patient we evaluated seizure freedom at 3 and 6 months (primary endpoints), side effects and drop-out rate (secondary endpoints). RESULTS: Overall, LCM led to seizure freedom in 64.4% of patients at 3 months and 55% at 6 months. Patients who used two or more ASMs before LCM had a worse seizure control than patients in monotherapy with LCM as first choice. In 14 patients, we observed seizure control despite tumor progression on magnetic resonance (MRI). Multivariate analysis showed that gross-total resection at diagnosis was significantly associated with higher seizure freedom rate at 6 months. Side effects were mainly mild (grade 1-2 according to CTCAE classification) and drop-out rate was low (1.5%). Main side effects were dizziness and somnolence. CONCLUSIONS: This is the first study showing a good efficacy and tolerability of LCM when used in monotherapy in BTRE. Further prospective studies are needed to confirm these preliminary data, investigating also quality of life and neurocognitive functions.

Enhancement of Parametric Effects in Polariton Waveguides Induced by Dipolar Interactions.

Exciton-polaritons are hybrid light-matter excitations arising from the nonperturbative coupling of a photonic mode and an excitonic resonance. Behaving as interacting photons, they show optical third-order nonlinearities providing effects such as optical parametric oscillation or amplification. It has been suggested that polariton-polariton interactions can be greatly enhanced by inducing aligned electric dipoles in their excitonic part. However, direct evidence of a true particle-particle interaction, such as superfluidity or parametric scattering, is still missing. In this Letter, we demonstrate that dipolar interactions can be used to enhance parametric effects such as self-phase modulation in waveguide polaritons. By quantifying these optical nonlinearities, we provide a reliable experimental measurement of the direct dipolar enhancement of polariton-polariton interactions.

Insomnia and daytime sleepiness predict 20-year mortality in older male adults: data from a population-based study.

Data regarding the possible relationship of insomnia and EDS with mortality are inconclusive. The aim of this study was to investigate the association between these sleep complaints and the risk of long-term (20 years) all-cause mortality in older adults. Between April 2000 and March 2001, 750 subjects aged 65 years and older, who resided in the seventh district of Udine, were recruited. Data on sociodemographic characteristics, past medical history, and pharmacological treatment were collected. Dementia was diagnosed using a comprehensive neurological and neuroradiological assessment. Older adults were interviewed by neuropsychologists trained in sleep disturbances in order to assess the presence of sleep complaints. Vital status was followed over 20 years until March 2020. Older male adults affected by insomnia and EDS were significantly more likely to die over the follow-up period. Indeed, males reporting poor sleep and daytime somnolence had a 60% and 48% higher chance of dying than subjects who were not affected by these sleep complaints, respectively. The HR was attenuated after adjusting for confounding variables among insomniacs, whereas that of somnolent men strengthened. Differently from men, insomnia and EDS did not have any impact on mortality in older women. In conclusion, older male adults affected by insomnia and EDS had a significant increased risk of mortality, which is independent of cancer, depression, dementia, cardiovascular diseases, and sleeping pill use.

Two-dimensional hybrid perovskites sustaining strong polariton interactions at room temperature.

Polaritonic devices exploit the coherent coupling between excitonic and photonic degrees of freedom to perform highly nonlinear operations with low input powers. Most of the current results exploit excitons in epitaxially grown quantum wells and require low-temperature operation, while viable alternatives have yet to be found at room temperature. We show that large single-crystal flakes of two-dimensional layered perovskite are able to sustain strong polariton nonlinearities at room temperature without the need to be embedded in an optical cavity formed by highly reflecting mirrors. In particular, exciton-exciton interaction energies are shown to be spin dependent, remarkably similar to the ones known for inorganic quantum wells at cryogenic temperatures, and more than one order of magnitude larger than alternative room temperature polariton devices reported so far. Because of their easy fabrication, large dipolar oscillator strengths, and strong nonlinearities, these materials pave the way for realization of polariton devices at room temperature.

Tests of a novel imaging algorithm to localize hidden objects or cavities with muon radiography.

A novel algorithm developed within muon radiography to localize objects or cavities hidden inside large material volumes was recently proposed by some of the authors (Bonechi et al. 2015 J. Instrum. 10, P02003 (doi:10.1088/1748-0221/10/02/P02003)). The algorithm, based on muon back projection, helps to estimate the three-dimensional position and the transverse extension of detected objects without the need for measurements from different points of view, which would be required to make a triangulation. This algorithm can now be tested owing to the availability of real data collected both in laboratory tests and from real-world measurements. The methodology and some test results are presented in this paper.This article is part of the Theo Murphy meeting issue 'Cosmic-ray muography'.

Reply to the 'Comment on "Metal-organic green dye: chemical and physical insight into a modified Zn-benzoporphyrin for dye-sensitized solar cells"' by R. Steer, RSC Advances, 2018, DOI: 10.1039/c8ra00213d.

The authors reply to the comment by R. P. Steer discussing the reasons for their incorrect assignment of the luminescence decay of the novel compound 5,10,15-(triphenyl),20-[ethynyl-(4-carboxy)phenyl]tetrabenzoporphyrinate Zn(ii) (PETBP). Further DFT and TDDFT calculations have been performed on the compound to investigate the possibility of a direct S2-S0 decay instead of a S2-S1 conversion with a subsequent emission to the ground state. In addition, the presence of traces of very luminescent contaminants of the ring-opened type has been considered on the grounds of calculated absorption and fluorescence spectra. The results of these investigations confirm that the S2-S0 emission reported in the commented paper is not attributable to the target molecule but rather to a neglected luminescent impurity.

Toward the Elucidation of the Competing Role of Evaporation and Thermal Decomposition in Ionic Liquids: A Multitechnique Study of the Vaporization Behavior of 1-Butyl-3-methylimidazolium Hexafluorophosphate under Effusion Conditions.

The evaporation/decomposition behavior of the imidazolium ionic liquid 1-butyl-3-methylimidazolium hexafluorophosphate (BMImPF6) was investigated in the overall temperature range 425-551 K by means of the molecular-effusion-based techniques Knudsen effusion mass loss (KEML) and Knudsen effusion mass spectrometry (KEMS), using effusion orifices of different size (from 0.2 to 3 mm in diameter). Specific effusion fluxes measured by KEML were found to depend markedly on the orifice size, suggesting the occurrence of a kinetically delayed evaporation/decomposition process. KEMS experiments revealed that other species are present in the vapor phase besides the intact ion pair BMImPF6(g) produced by the simple evaporation BMImPF6(l) = BMImPF6(g), with relative abundances depending on the orifice size-the larger the orifice, the larger the contribution of the BMImPF6(g) species. By combining KEML and KEMS results, the conclusion is drawn that in the investigated temperature range, when small effusion orifices are used, a significant part of the mass loss/volatility of BMImPF6 is due to molecular products formed by decomposition/dissociation processes rather than to evaporated intact ion pairs. Additional experiments performed by nonisothermal thermogravimetry-differential thermal analysis (TG-DTA) further support the evidence of simultaneous evaporation/decomposition, although the conventional decomposition temperature derived from TG curves is much higher than the temperatures covered in effusion experiments. Partial pressures of the BMImPF6(g) species were derived from KEMS spectra and analyzed by second- and third-law methods giving a value of ΔevapH298K° = 145.3 ± 2.9 kJ·mol-1 for the standard evaporation enthalpy of BMImPF6. A comparison is done with the behavior of the 1-butyl-3-methylimidazolium bis(trifluoromethyl)sulfonylimide (BMImNTf2) ionic liquid.

Orthogonal electronic coupling in multicentre arylamine mixed-valence compounds based on a dibenzofulvene-thiophene conjugated bridge.

Herein we present organic mixed-valence compounds with an innovative H-shape design, where four redox centres are bridged "vertically" via a dibenzofulvene backbone and "horizontally" via a bis-(dibenzofulvene)-thiophene bridge. These compounds are easily oxidized to stable highly charged radical species which show intense intervalence charge transfer transitions in the near infrared region. Interestingly, depending on the position of the arylamine substituents on the bridge, both vertical and horizontal electron transfer pathways can be optically induced.

SNCA 3'UTR genetic variants in patients with Parkinson's disease and REM sleep behavior disorder.

REM sleep behavior disorder (RBD) is an early marker of Parkinson's disease (PD); however, it is still unclear which patients with RBD will eventually develop PD. Single nucleotide polymorphisms (SNPs) in the 3'untranslated region (3'UTR) of alpha-synuclein (SNCA) have been associated with PD, but at present, no data is available about RBD. The 3'UTR hosts regulatory regions involved in gene expression control, such as microRNA binding sites. The aim of this study was to determine RBD specific genetic features associated to an increased risk of progression to PD, by sequencing of the SNCA-3'UTR in patients with "idiopathic" RBD (iRBD) and in patients with PD. We recruited 113 consecutive patients with a diagnosis of iRBD (56 patients) or PD (with or without RBD, 57 patients). Sequencing of SNCA-3'UTR was performed on genomic DNA extracted from peripheral blood samples. Bioinformatic analyses were carried out to predict the potential effect of the identified genetic variants on microRNA binding. We found three SNCA-3'UTR SNPs (rs356165, rs3857053, rs1045722) to be more frequent in PD patients than in iRBD patients (p = 0.014, 0.008, and 0.008, respectively). Four new or previously reported but not annotated specific genetic variants (KP876057, KP876056, NM_000345.3:c*860T>A, NM_000345.3:c*2320A>T) have been observed in the RBD population. The in silico approach highlighted that these variants could affect microRNA-mediated gene expression control. Our data show specific SNPs in the SNCA-3'UTR that may bear a risk for RBD to be associated with PD. Moreover, new genetic variants were identified in patients with iRBD.

Elucidating the effect of the lead iodide complexation degree behind the morphology and performance of perovskite solar cells.

The inclusion of iodide additives in hybrid perovskite precursor solutions has been successfully exploited to improve the solar cell efficiency but their impact on perovskite formation, morphology and photovoltaic performance is still not clear. Here an extensive analysis of the effect of iodide additives in the solution-phase and during the perovskite film formation, as well as their effect on device performance is provided. The results demonstrate that in the solution-phase the additives promote the formation of lead poly-iodide species resulting in the disaggregation of the inorganic lead iodide framework and in the formation of smaller nuclei inducing the growth of uniform and smooth perovskite films. Most importantly, the complexation capability of different iodide additives does not only directly affect film morphology but also influences the density of defect states by varying the stoichiometry of precursors. These findings demonstrate that the fine control of the interactions of the chemical species in the solution-phase is essential for the precise control of the morphology at the nanoscale and the growth of the perovskite films with a reduced density of defect states. Therefore, the in-depth understanding of all the processes involved in the solution-phase is the first step for the development of a facile and reproducible approach for the fabrication of hybrid perovskite solar cells with enhanced photovoltaic performance.

The colored Hanbury Brown-Twiss effect.

The Hanbury Brown-Twiss effect is one of the celebrated phenomenologies of modern physics that accommodates equally well classical (interferences of waves) and quantum (correlations between indistinguishable particles) interpretations. The effect was discovered in the late thirties with a basic observation of Hanbury Brown that radio-pulses from two distinct antennas generate signals on the oscilloscope that wiggle similarly to the naked eye. When Hanbury Brown and his mathematician colleague Twiss took the obvious step to propose bringing the effect in the optical range, they met with considerable opposition as single-photon interferences were deemed impossible. The Hanbury Brown-Twiss effect is nowadays universally accepted and, being so fundamental, embodies many subtleties of our understanding of the wave/particle dual nature of light. Thanks to a novel experimental technique, we report here a generalized version of the Hanbury Brown-Twiss effect to include the frequency of the detected light, or, from the particle point of view, the energy of the detected photons. Our source of light is a polariton condensate, that allows high-resolution filtering of a spectrally broad source with a high degree of coherence. In addition to the known tendencies of indistinguishable photons to arrive together on the detector, we find that photons of different colors present the opposite characteristic of avoiding each others. We postulate that fermions can be similarly brought to exhibit positive (boson-like) correlations by frequency filtering.

Virtual unrolling and deciphering of Herculaneum papyri by X-ray phase-contrast tomography.

A collection of more than 1800 carbonized papyri, discovered in the Roman 'Villa dei Papiri' at Herculaneum is the unique classical library survived from antiquity. These papyri were charred during 79 A.D. Vesuvius eruption, a circumstance which providentially preserved them until now. This magnificent collection contains an impressive amount of treatises by Greek philosophers and, especially, Philodemus of Gadara, an Epicurean thinker of 1st century BC. We read many portions of text hidden inside carbonized Herculaneum papyri using enhanced X-ray phase-contrast tomography non-destructive technique and a new set of numerical algorithms for 'virtual-unrolling'. Our success lies in revealing the largest portion of Greek text ever detected so far inside unopened scrolls, with unprecedented spatial resolution and contrast, all without damaging these precious historical manuscripts. Parts of text have been decoded and the 'voice' of the Epicurean philosopher Philodemus is brought back again after 2000 years from Herculaneum papyri.

Fibrous scaffolds fabricated by emulsion electrospinning: from hosting capacity to in vivo biocompatibility.

Electrospinning is a versatile method for preparing functional three-dimensional scaffolds. Synthetic and natural polymers have been used to produce micro- and nanofibers that mimic extracellular matrices. Here, we describe the use of emulsion electrospinning to prepare blended fibers capable of hosting aqueous species and releasing them in solution. The existence of an aqueous and a non-aqueous phase allows water-soluble molecules to be introduced without altering the structure and the degradation of the fibers, and means that their release properties under physiological conditions can be controlled. To demonstrate the loading capability and flexibility of the blend, various species were introduced, from magnetic nanoparticles and quantum rods to biological molecules. Cellular studies showed the spontaneous adhesion and alignment of cells along the fibers. Finally, in vivo experiments demonstrated the high biocompatibility and safety of the scaffolds up to 21 days post-implantation.

Real-space collapse of a polariton condensate.

Microcavity polaritons are two-dimensional bosonic fluids with strong nonlinearities, composed of coupled photonic and electronic excitations. In their condensed form, they display quantum hydrodynamic features similar to atomic Bose-Einstein condensates, such as long-range coherence, superfluidity and quantized vorticity. Here we report the unique phenomenology that is observed when a pulse of light impacts the polariton vacuum: the fluid which is suddenly created does not splash but instead coheres into a very bright spot. The real-space collapse into a sharp peak is at odd with the repulsive interactions of polaritons and their positive mass, suggesting that an unconventional mechanism is at play. Our modelling devises a possible explanation in the self-trapping due to a local heating of the crystal lattice, that can be described as a collective polaron formed by a polariton condensate. These observations hint at the polariton fluid dynamics in conditions of extreme intensities and ultrafast times.

Amino acid supplementation in l-dopa treated Parkinson's disease patients.

BACKGROUND: The correlation between Parkinson disease and malnutrition is well established, however a protein-restricted diet is usually prescribed because of potentially negative interactions between dietary amino acids and l-dopa pharmacokinetics. This strategy could increase the risk of further nutritional deficits. METHODS: A monocentric, prospective, randomized, double-blind pilot study was performed on two groups of Parkinson-affected, protein-restricted, patients: Intervention (n = 7; amino acid supplementation twice daily) and Placebo (n = 7; placebo supplementation twice daily). At enrolment, after 3- and 6-month supplementation, neurological evaluations (UPDRS III, Hoenh-Yahr scale, l-dopa equivalent dose assessment) were performed and blood sample was collected to define insulin sensitivity (QUICKI index) and oxidative stress (oxidized and reduced glutathione). Repeated measure ANCOVA was applied to define time effect and time × treatment interaction. RESULTS: Participants were comparable at baseline for all assessed parameters. Neurological outcomes and l-dopa requirement were comparable in both group after 6-month of supplementation, without time × treatment interaction. The decrease in insulin sensitivity, as assessed by QUICKI index, observed after 6 months in both groups, was greater in Placebo than in Intervention (time effect p < 0.001; time × treatment interaction p = 0.01). Moreover, despite no changes in total erythrocyte glutathione concentrations, oxidized glutathione levels decreased by 28 ± 17% in the Intervention while increased by 55 ± 38% in Placebo (time effect p = 0.05; time × treatment interaction p = 0.05), after 6-month supplementation. CONCLUSIONS: Amino acid supplementation, assumed with shrewd temporal distribution, did not show detrimental effects on neurological and pharmacological control in protein-restricted Parkinson-affected patients, chronically treated with l-dopa. Furthermore, daily amino acid supplementation partially counteracted insulin resistance development and the loss in antioxidant availability.