Distinct clinical and neuropathological features of G51D SNCA mutation cases compared with SNCA duplication and H50Q mutation

Background: We and others have described the neurodegenerative disorder caused by G51D SNCA mutation which shares characteristics of Parkinson’s disease (PD) and multiple system atrophy (MSA). The objective of this investigation was to extend the description of the clinical and neuropathological hallmarks of G51D mutant SNCA-associated disease by the study of two additional cases from a further G51D SNCA kindred and to compare the features of this group with a SNCA duplication case and a H50Q SNCA mutation case. Results: All three G51D patients were clinically characterised by parkinsonism, dementia, visual hallucinations, autonomic dysfunction and pyramidal signs with variable age at disease onset and levodopa response. The H50Q SNCA mutation case had a clinical picture that mimicked late-onset idiopathic PD with a good and sustained levodopa response. The SNCA duplication case presented with a clinical phenotype of frontotemporal dementia with marked behavioural changes, pyramidal signs, postural hypotension and transiently levodopa responsive parkinsonism. Detailed post-mortem neuropathological analysis was performed in all cases. All three G51D cases had abundant α-synuclein pathology with characteristics of both PD and MSA. These included widespread cortical and subcortical neuronal α-synuclein inclusions together with small numbers of inclusions resembling glial cytoplasmic inclusions (GCIs) in oligodendrocytes. In contrast the H50Q and SNCA duplication cases, had α-synuclein pathology resembling idiopathic PD without GCIs. Phosphorylated α-synuclein was present in all inclusions types in G51D cases but was more restricted in SNCA duplication and H50Q mutation. Inclusions were also immunoreactive for the 5G4 antibody indicating their highly aggregated and likely fibrillar state. Conclusions: Our characterisation of the clinical and neuropathological features of the present small series of G51D SNCA mutation cases should aid the recognition of this clinico-pathological entity. The neuropathological features of these cases consistently share characteristics of PD and MSA and are distinct from PD patients carrying the H50Q or SNCA duplication

First measurement of coherent ρ0 photoproduction in ultra-peripheral Xe–Xe collisions at √sNN = 5.44 TeV

The first measurement of the coherent photoproduction of ρ0 vector mesons in ultra-peripheral Xe–Xe collisions at sNN=5.44 TeV is presented. This result, together with previous HERA γp data and γ–Pb measurements from ALICE, describes the atomic number (A) dependence of this process, which is particularly sensitive to nuclear shadowing effects and to the approach to the black-disc limit of QCD at a semi-hard scale. The cross section of the Xe+Xe→ρ0+Xe+Xe process, measured at midrapidity through the decay channel ρ0→π+π−, is found to be dσ/dy=131.5±5.6(stat.)−16.9+17.5(syst.) mb. The ratio of the continuum to resonant contributions for the production of pion pairs is also measured. In addition, the fraction of events accompanied by electromagnetic dissociation of either one or both colliding nuclei is reported. The dependence on A of cross section for the coherent ρ0 photoproduction at a centre-of-mass energy per nucleon of the γA system of WγA,n=65 GeV is found to be consistent with a power-law behaviour σ(γA→ρ0A)∝Aα with a slope α=0.96±0.02(syst.). This slope signals important shadowing effects, but it is still far from the behaviour expected in the black-disc limit.publishedVersio

A(c)(+) Production and Baryon-to-Meson Ratios in pp and p-Pb Collisions at root S-NN=5.02 TeV at the LHC

The prompt production of the charm baryon \u39bc+ and the \u39bc+/D0 production ratios were measured at midrapidity with the ALICE detector in pp and p-Pb collisions at sNN=5.02 TeV. These new measurements show a clear decrease of the \u39bc+/D0 ratio with increasing transverse momentum (pT) in both collision systems in the range 2<12 GeV/c, exhibiting similarities with the light-flavor baryon-to-meson ratios p/\u3c0 and \u39b/KS0. At low pT, predictions that include additional color-reconnection mechanisms beyond the leading-color approximation, assume the existence of additional higher-mass charm-baryon states, or include hadronization via coalescence can describe the data, while predictions driven by charm-quark fragmentation processes measured in e+e- and e-p collisions significantly underestimate the data. The results presented in this Letter provide significant evidence that the established assumption of universality (colliding-system independence) of parton-to-hadron fragmentation is not sufficient to describe charm-baryon production in hadronic collisions at LHC energies

Measurement of the production cross section of prompt Xi(0)(c) baryons at midrapidity in pp collisions at root s=5.02 TeV

The transverse momentum (pT) differential cross section of the charm-strange baryon \u39e0c is measured at midrapidity (|y| < 0.5) via its semileptonic decay into e+\u39e 12\u3bde in pp collisions at s 1a = 5.02 TeV with the ALICE detector at the LHC. The ratio of the pT-differential \u39e0c-baryon and D0-meson production cross sections is also reported. The measurements are compared with simulations with different tunes of the PYTHIA 8 event generator, with predictions from a statistical hadronisation model (SHM) with a largely augmented set of charm-baryon states beyond the current lists of the Particle Data Group, and with models including hadronisation via quark coalescence. The pT-integrated cross section of prompt \u39e0c-baryon production at midrapidity is also reported, which is used to calculate the baryon-to-meson ratio \u39e0c/D0 = 0.20 \ub1 0.04 (stat.)+0.08 120.07 (syst.). These results provide an additional indication of a modification of the charm fragmentation from e+e 12 and e 12p collisions to pp collisions

Enhanced Deuteron Coalescence Probability in Jets

International audienceThe transverse-momentum (pT) spectra and coalescence parameters B2 of (anti)deuterons are measured in p-p collisions at s=13  TeV for the first time in and out of jets. In this measurement, the direction of the leading particle with the highest pT in the event (pTlead&gt;5  GeV/c) is used as an approximation for the jet axis. The event is consequently divided into three azimuthal regions, and the jet signal is obtained as the difference between the toward region, that contains jet fragmentation products in addition to the underlying event (UE), and the transverse region, which is dominated by the UE. The coalescence parameter in the jet is found to be approximately a factor of 10 larger than that in the underlying event. This experimental observation is consistent with the coalescence picture and can be attributed to the smaller average phase-space distance between nucleons in the jet cone as compared with the underlying event. The results presented in this Letter are compared to predictions from a simple nucleon coalescence model, where the phase-space distributions of nucleons are generated using pythia8 with the Monash 2013 tuning, and to predictions from a deuteron production model based on ordinary nuclear reactions with parametrized energy-dependent cross sections tuned on data. The latter model is implemented in pythia8.3. Both models reproduce the observed large difference between in-jet and out-of-jet coalescence parameters, although the almost flat trend of the B2Jet is not reproduced by the models, which instead give a decreasing trend

Measurement of the Lifetime and <math display="inline"><mi mathvariant="normal">Λ</mi></math> Separation Energy of <math display="inline"><mmultiscripts><mrow><mi mathvariant="normal">H</mi></mrow><mprescripts/><mrow><mi mathvariant="normal">Λ</mi></mrow><mn>3</mn></mmultiscripts></math>

International audienceThe most precise measurements to date of the HΛ3 lifetime τ and Λ separation energy BΛ are obtained using the data sample of Pb-Pb collisions at sNN=5.02  TeV collected by ALICE at the LHC. The HΛ3 is reconstructed via its charged two-body mesonic decay channel (HΛ3→He3+π- and the charge-conjugate process). The measured values τ=[253±11(stat)±6(syst)]  ps and BΛ=[102±63(stat)±67(syst)]  keV are compatible with predictions from effective field theories and confirm that the HΛ3 structure is consistent with a weakly bound system

Measurement of the <math display="inline"><mrow><mi>J</mi><mo>/</mo><mi>ψ</mi></mrow></math> Polarization with Respect to the Event Plane in Pb-Pb Collisions at the LHC

International audienceWe study the polarization of inclusive J/ψ produced in Pb-Pb collisions at sNN=5.02  TeV at the LHC in the dimuon channel, via the measurement of the angular distribution of its decay products. We perform the study in the rapidity region 2.5&lt;y&lt;4, for three transverse momentum intervals (2&lt;pT&lt;4, 4&lt;pT&lt;6, 6&lt;pT&lt;10  GeV/c) and as a function of the centrality of the collision for 2&lt;pT&lt;6  GeV/c. For the first time, the polarization is measured with respect to the event plane of the collision, by considering the angle between the positive-charge decay muon in the J/ψ rest frame and the axis perpendicular to the event-plane vector in the laboratory system. A small transverse polarization is measured, with a significance reaching 3.9σ at low pT and for intermediate centrality values. The polarization could be connected with the behavior of the quark-gluon plasma, formed in Pb-Pb collisions, as a rotating fluid with large vorticity, as well as with the existence of a strong magnetic field in the early stage of its formation