Slow Velocity Research Articles

The helium and carbon isotopic signature of Ocean island basalts: Insights from Fogo volcano (Cape Verde archipelago)

Fluid inclusions (FI) entrapped in phenocrysts carried by Ocean Island Basalts (OIB) contain key information on volatiles’ abundance and origin in their mantle sources. Here, we add new piece of knowledge to our understanding of volatile geochemistry in global OIB magmas, by presenting new noble gas (He-Ne-Ar) and carbon (C) isotope results for olivine- and clinopyroxene-hosted FI from enclaves, lavas, tephra and volcanic gas samples from Fogo, the only frequently active volcano at the Cape Verde archipelago (eastern Atlantic Ocean). FI, together with crater fumaroles, constrain the Fogo 3He/4He signature at 7.14–8.44 Rc/Ra (where RC is the air-corrected 3He/4He isotope ratio, and Ra is the same ratio in air), which is within the typical MORB (Mid-Ocean Ridge Basalt) mantle. The carbon isotopic ratio (δ13C vs. Pee Dee Belemnite) of CO2 in FI and fumaroles range from -6.04 to -4.41 ‰. We identify systematic variations of δ13C and He/Ar* with FI entrapment pressure (estimated from a combination of host mineral barometry and FI microthermometry), from which we develop a model for volatile degassing in the mantle-to-crustal magma storage system. The model predicts a crustal-like signature for carbon (δ13C of -0.4 ± 1.0 ‰) in primary melts formed by mantle melting at ∼2200 MPa (∼77 km) and a source He/Ar* ratio of 0.90–0.24, which are indicative of variably depleted mantle metasomatized by carbon enriched melts/fluids from a crustal component. We also use our results to characterise regional (in the Cape Verde and Canary archipelagos) and global trends in C and He isotope composition from OIB. From a comparison with the few other OIB localities for which δ13C are available, we propose that a carbon enriched crustal component could be recurrent at a global scale in OIB magmatism, although often masked by isotope fractionation during magmatic degassing. We additionally find that, at regional scale, He isotopes in OIB scale inversely correlate with the degree of partial melting of the mantle beneath individual islands’ (inferred from the La/Yb ratio of erupted basalts). More widely, our results corroborate previously established global relationships between OIB He isotopic signature, plume buoyancy flux and overlying plate velocity. In this interpretation, the MORB-like 3He/4He (8 ± 1 Ra) at Fogo reflects a combination of (i) low to medium magma productivity, (ii) relatively low plume buoyancy flux (∼1.1 Mg/s), and (iii) slow average velocity (∼3 cm/yr) of the overlying plate.

Exploring Responsiveness to Highly Challenging Balance and Gait Training in Parkinson's Disease.

Exercise potentially improves gait, balance, and habitual physical activity in Parkinson's disease (PD). However, given the heterogeneous nature of the disease, it is likely that people respond differently to exercise interventions. Factors determining responsiveness to exercise interventions remain unclear. To address this uncertainty, we explored the responsiveness to our highly challenging balance and gait intervention (HiBalance) in people with PD. Thirty-nine participants with mild-moderate PD who underwent the HiBalance intervention from our randomized controlled trial were included. We defined response in three domains: (1) balance based on Mini-BESTest, (2) gait based on gait velocity, and (3) physical activity based on accelerometry-derived steps per day. In each domain, we explored three responsiveness levels: high, low, or non-responders according to the change from pre- to post-intervention. Separate Random Forests for each responder domain classified these responsiveness levels and identified variable importance. Only the Random Forest for the balance domain classified all responsiveness levels above the chance level indicated by a Cohen's kappa of "slight" agreement. Variable importance differed among the responsiveness levels. Slow gait velocity indicated high responders in the balance domain but showed low probabilities for low and non-responders. For low and non-responders, fall history or no falls, respectively, were more important. Among three responder domains and responsiveness levels, we could moderately classify responders in the balance domain, but not for the gait or physical activity domain. This can guide inclusion criteria for balance-targeted, personalized intervention studies in people with PD.

Superionic iron hydride shapes ultralow-velocity zones at Earth’s core–mantle boundary

Seismological studies have exposed numerous ultralow velocity zones (ULVZs) exhibiting extraordinary physical attributes at Earth's core-mantle boundary, yet their compositions and origins remain controversial. Water-iron reaction can generate unique phases under lowermost-mantle conditions and likely plays a crucial role in forming ULVZs. Through first-principles molecular dynamic simulations with machine learning techniques, we determine that iron hydride, the product of water-iron reaction, is stable as a superionic phase at the core-mantle boundary. This superionic iron hydride has much slower velocities and a higher density than the ambient mantle under lowermost-mantle conditions. Accumulation of iron hydride, created through either a chemical reaction between subducted water and iron or solidification of core material entrained in the lower mantle by convection, can explain the seismic observations of ULVZs particularly those associated with subduction. This work suggests that water may have a substantial role in creating seismic heterogeneities at the core-mantle boundary.

Focal slowing of nerve conduction velocity in leprosy patients unveiled through multisegmented nerve analysis.

Leprosy is a chronic infectious disease caused by Mycobacterium leprae (M. leprae), an intracellular bacillus that systematically invades the peripheral nerves. Diagnosing leprosy neuropathy is still a defying skill, and late diagnosis and treatment are still a reality. Based on the biological characteristics of M. leprae, particularly its preference for invading the Schwann cells localized at the coldest areas of human body, we hypothesized that these areas have focal demyelination that may escape detection through standard nerve conduction studies (NCSs) protocols. Twenty-five patients with confirmed multibacillary leprosy and 14 controls were accessed. A multisegmented NCS protocol (MP) was performed, targeting short segments through the coldest areas, to identify focal areas of slowed conduction velocity. The effectiveness of this multisegmented protocol was compared to the standard protocol (SP) to detect abnormalities. All leprosy patients presented an abnormal study with the MP, contrasting to 19 with the SP. The most frequent NCS pattern was an asymmetric neuropathy with focal slowing of conduction velocity, found in 23 out of 25 leprosy patients. Significant differences favoring the proposed method were observed when comparing the MP with the SP. Notably, the MP increased the sensitivity to detect abnormalities by 122%, 133%, and 257% for the median, peroneal, and tibial nerves, respectively. MP also increases sensitivity to detect focal abnormalities in the ulnar nerve. The MP protocol significantly increases the sensitivity of NCSs to detect neurophysiological abnormalities in leprosy neuropathy.

The comparison between intratympanic gentamicin prehabilitation and postoperative virtual reality exposure to standard vestibular training in patients with vestibular schwannoma.

Resection of the vestibular schwannoma causes acute peripheral vestibular loss. The process of central compensation starts immediately afterward. The rehabilitation goal is to support this process and restore the quality of life. In this prospective single-center study, 67 consecutive patients underwent vestibular schwannoma resection (40 females, mean age 52 ± 12 years). The patients were divided into three groups: the prehabilitation with intratympanic gentamicin group, the virtual reality group (optokinetic stimulation via virtual reality goggles in the first ten days after the surgery), and the control group. All patients were examined with objective methods and completed questionnaires before the prehabilitation, before the surgery, at the hospital discharge, and after three months. Intratympanic gentamicin prehabilitation leads ipsilaterally to a significant aVOR reduction in all semicircular canals (p < 0.050), the increase of the unilateral weakness in air calorics (p = 0.026), and loss of cVEMPs responses (p = 0.017). Prehabilitation and postoperative exposure to virtual reality scenes improved the patient's perception of vertigo problems according to Dizziness Handicap Inventory (p = 0.039 and p = 0.076, respectively). These findings conform with the optokinetic testing results, which showed higher slow phase velocities at higher speeds (40deg/s) in both targeted groups compared to the control group. Preoperative intratympanic gentamicin positively affects peripheral vestibular function, influencing balance perception after VS resection. In long-term follow-up, prehabilitation and postoperative exposure to virtual reality improve patients' quality of life in the field of vertigo problems.

Calibrating the WSA Model in EUHFORIA Based on Parker Solar Probe Observations

We employ Parker Solar Probe (PSP) observations during the latest solar minimum period (years 2018–2021) to calibrate the version of the Wang–Sheeley–Arge (WSA) coronal model used in the European Heliospheric Forecasting Information Asset (EUHFORIA). WSA provides a set of boundary conditions at 0.1 au necessary to initiate the heliospheric part of EUHFORIA, namely, the domain extending beyond the solar Alfvénic point. To calibrate WSA, we observationally constrain four constants in the WSA semiempirical formula based on PSP observations. We show how the updated (after the calibration) WSA boundary conditions at 0.1 au are compared to PSP observations at similar distances, and we further propagate these conditions in the heliosphere according to EUHFORIA’s magnetohydrodynamic (MHD) approach. We assess the predictions at Earth based on the dynamic time-warping technique. Our findings suggest that, for the period of interest, the WSA configurations that resembled optimally the PSP observations close to the Sun were different from the ones needed to provide better predictions at Earth. One reason for this discrepancy can be attributed to the scarcity of fast solar wind velocities recorded by PSP. The calibration of the model was performed based on unexpectedly slow velocities that did not allow us to achieve generally and globally improved solar wind predictions compared to older studies. Other reasons can be attributed to missing physical processes from the heliospheric part of EUHFORIA but also the fact that the currently employed WSA relationship, as coupled to the heliospheric MHD domain, may need a global reformulation beyond that of just updating the four constant factors that were taken into account in this study.

The Frictional‐Viscous Transition in Experimentally Deformed Granitoid Fault Gouge

AbstractIn crustal faults dominated by granitoid gouges, the frictional‐viscous transition marks a significant change in strength constraining the lower depth limit of the seismogenic zone. Dissolution‐precipitation creep (DPC) may play an important role in initiating this transition, especially within polymineralic materials. Yet, it remains unclear to what extent DPC contributes to the weakening of granitoid gouge materials at the transition. Here we conducted sliding experiments on wet granitoid gouges to large displacement (15 mm), at an effective normal stress and pore fluid pressure of 100 MPa, at temperatures of 20–650°C, and at sliding velocities of 0.1–100 μm/s, which are relevant for earthquake nucleation. Gouge shear strengths were generally ∼75 MPa even at temperatures up to 650°C and at velocities &gt;1 μm/s. At velocities ≤1 μm/s, strengths decreased at temperatures ≥450°C, reaching a minimum of 37 MPa at the highest temperature and lowest velocity condition. Microstructural observations showed that, as the gouges weakened, the strain localized into thin, dense, and ultrafine‐grained (≤1 μm) principal slip zones, where nanopores were located along grain contacts and contained minute biotite‐quartz‐feldspar precipitates. The stress sensitivity exponent n decreased from a large number at 20°C to ∼2.2 at 650°C at the lowest velocities. These findings suggest that high temperature, slow velocity and small grain sizes promote DPC‐accommodated granular flow over cataclastic frictional granular flow, leading to the observed weakening and strain localization. Field observations together with extrapolation suggest that DPC‐induced weakening occurs at depths of 7–20 km depending on geothermal gradient.

Beneficial Effect of Calcium Treatment for Hyperkalemia Is Not Due to "Membrane Stabilization".

Hyperkalemia is a common life-threatening condition causing severe electrophysiologic derangements and arrhythmias. The beneficial effects of calcium (Ca 2+ ) treatment for hyperkalemia have been attributed to "membrane stabilization," by restoration of resting membrane potential (RMP). However, the underlying mechanisms remain poorly understood. Our objective was to investigate the mechanisms underlying adverse electrophysiologic effects of hyperkalemia and the therapeutic effects of Ca 2+ treatment. Controlled experimental trial. Laboratory investigation. Canine myocytes and tissue preparations. Optical action potentials and volume averaged electrocardiograms were recorded from the transmural wall of ventricular wedge preparations ( n = 7) at baseline (4 mM potassium), hyperkalemia (8-12 mM), and hyperkalemia + Ca 2+ (3.6 mM). Isolated myocytes were studied during hyperkalemia (8 mM) and after Ca 2+ treatment (6 mM) to determine cellular RMP. Hyperkalemia markedly slowed conduction velocity (CV, by 67% ± 7%; p < 0.001) and homogeneously shortened action potential duration (APD, by 20% ± 10%; p < 0.002). In all preparations, this resulted in QRS widening and the "sine wave" pattern observed in severe hyperkalemia. Ca 2+ treatment restored CV (increase by 44% ± 18%; p < 0.02), resulting in narrowing of the QRS and normalization of the electrocardiogram, but did not restore APD. RMP was significantly elevated by hyperkalemia; however, it was not restored with Ca 2+ treatment suggesting a mechanism unrelated to "membrane stabilization." In addition, the effect of Ca 2+ was attenuated during L-type Ca 2+ channel blockade, suggesting a mechanism related to Ca 2+ -dependent (rather than normally sodium-dependent) conduction. These data suggest that Ca 2+ treatment for hyperkalemia restores conduction through Ca 2+ -dependent propagation, rather than restoration of membrane potential or "membrane stabilization." Our findings provide a mechanistic rationale for Ca 2+ treatment when hyperkalemia produces abnormalities of conduction (i.e., QRS prolongation).

Flatbands in a bilayer surface plasmon crystal at a large twist angle due to interlayer strong coupling.

The twisted bilayer system provides an excellent platform for the study of flatbands. In this work, we propose a bilayer hexagonal boron nitride (h-BN)-like surface plasmon crystal at a large twist angle of 38.213° due to the interlayer strong coupling, in which the adjacent pillars are in different radii. We numerically and theoretically calculate the band structure while tuning the pillar radius ratio (PRR) and the interlayer separation distance. As a result, both increasing the PRR and decreasing the separation distance contribute to the transition from weak coupling to strong coupling, leading to the flatbands with slow velocity and large density of state. Consequently, the in-layer geometry as well as the separation distance offers the degree of freedom to achieve flatbands in the bilayer surface plasmon crystal. Our work provides a fundamental understanding of the band structure of the twisted bilayer photonic system, which enriches the methods to obtain flatbands at a large twist angle.

The Influence of Mallet Mass and Velocity on the Fracture Patterns in Osteotomies.

Osteotomies are routinely incorporated in rhinoplasty, however, the influence of mass, velocity, kinetic energy (KE), and momentum (p) of the mallet on fracture patterns has not been studied. An experimental sledge guillotine setup was designed simulating a mallet strike with adjustable height and mass and 2 mm-thick Sawbone blocks. KE and p were calculated using KE = ½ mass × velocity2 and p = mass × velocity formulas. Fracture lengths and angles were measured. Ten groups with varying mallet masses and drop heights were tested with 10 bones per group. Fracture length positively correlated with KE (R = 0.542, p < 0.001) and p (R = 0.508, p < 0.001). Fracture angle also positively correlated with KE (R = 0.367, p < 0.001) and p (R = 0.329, p < 0.001). In groups with similar KE, osteotomies with higher p (heavier mallet with slower velocity) had greater fracture lengths (29.31 ± 0.68 vs. 27.68 ± 2.12 mm, p = 0.013) but similar fracture angles (p = 0.189). In groups with similar p, osteotomies with higher KE (lighter hammer with faster velocity) had significantly greater fracture lengths (28.28 ± 1.28 vs. 20.45 ± 12.20 mm, p = 0.041) and greater divergent fracture angles (3.13 ± 1.97° vs. 1.40 ± 1.36°, p = 0.031). Regression modeling of the relationship between KE and fracture lengths and angles demonstrated that cubic followed by logarithmic regression models had the best fits. Osteotomy fracture patterns positively correlated with the mallet's KE more so than its p, suggesting that the mallet's velocity has an increased impact effect than its mass. Clinically, a heavier mallet with a lower velocity will likely generate a smaller fracture length and fracture angle, indicating a more controlled and ideal fracture. NA Laryngoscope, 2024.

Retrospective analysis of nystagmus characteristics and clinical applications of positional testing in patients with cupulolithiasis of the posterior semicircular canal in benign paroxysmal positional vertigo.

This study aimed to investigate the characteristics of positional nystagmus in patients with cupulolithiasis of the posterior semicircular canal-benign paroxysmal positional vertigo (PC-BPPV-cu) to improve clinical diagnostic accuracy. This study retrospectively analyzed 128 cases of PC-BPPV-cu and 128 cases of canalolithiasis of BPPV (PC-BPPV-ca). General data, intensity, distribution, and the correlation of positional nystagmus were compared between the two groups. Compared to the PC-BPPV-ca group, more cases from the PC-BPPV-cu group initially presented in the emergency department (P < 0.05). The most frequent positional nystagmus induced by PC-BPPV-cu was torsional-upbeat nystagmus, characterized by the upper pole of the affected eye beating toward the lower ear and vertically upward (387 cases, 59.7%). It was followed by torsional-downbeat nystagmus, characterized by the upper pole of the unaffected eye beating toward the lower ear and vertically downward (164 cases, 25.3%). The former represented posterior canal excitatory nystagmus (PC-EN), while the latter represented posterior canal inhibitory nystagmus (PC-IN). In the PC-BPPV-cu group, PC-EN was most easily caused by the Half Dix-Hallpike (HH) maneuver on the affected side, while PC-IN was most easily induced by a face-down position (FDP) on the unaffected side at approximately 45° angle (45° FDP). The vertical slow phase velocity (v-SPV) of positional nystagmus was more potent in the affected HH than in other positions with PC-EN (all P < 0.05); the v-SPV of positional nystagmus was greater in the 45° FDP than in different positions with PC-IN (all P < 0.05); the v-SPV of the affected Dix-Hallpike (DH) maneuver in the PC-BPPV-ca group was significantly greater than that of the affected HH maneuver in the PC-BPPV-cu group (P < 0.05). The a priori analysis showed that the strongest correlation with HH positional nystagmus was observed in the affected side roll test, followed by the DH maneuver. In the PC-BPPV-cu group, the HH maneuver most easily induced PC-EN on the affected side, and PC-IN was most easily induced by the 45° FDP. In some cases of PC-BPPV-cu, significant nystagmus was not observed to be induced in the DH position on the affected side; however, vertical rotation nystagmus was induced in the roll-test position on the affected side. In such cases, PC-BPPV-cu diagnosis should be considered, and HH and 45° FDP tests should be conducted to support the diagnosis.

Noradrenergic cardiac denervation is associated with gait velocity in Parkinson disease: a dual ligand PET study.

Preliminary data suggest that gait abnormalities in Parkinson disease (PD) may be associated with sympathetic cardiac denervation. No kinematic gait studies were performed to confirm this observation. We aimed to correlate spatiotemporal kinematic gait parameters with cardiac sympathetic denervation as determined by cardiac [11C]HED PET in PD. Retrospective database analysis of 27 PD patients with cardiac sympathetic denervation. All patients underwent spatiotemporal kinematic gait assessment (medication 'off' state), cardiac [11C]HED and dopaminergic brain [11C]DTBZ PET scans. We employed a hierarchical regression approach to examine associations between the extent of cardiac denervation, dopaminergic nigrostriatal neurodegeneration, and three gait parameters - velocity, step length and cadence. More extensive cardiac denervation was associated with slower velocity (estimate: -1.034, 95% CI [-1.65, -0.42], p = 0.002), shorter step length (estimate: -0.818, 95% CI [-1.43, -0.21], p = 0.011) and lower cadence (estimate: -0.752, 95% CI [-1.28, -0.23], p = 0.007) explaining alone 30% (Adjusted-R²: 0.297), 20% (Adjusted-R²: 0.202) and 23% (Adjusted-R²: 0.227) of the variability, respecivetly. These associations remained independent of striatal dopaminergic impairment and confounding factors such as age, Hoehn and Yahr (HY) stages, peripheral neuropathy, cognition, and autonomic symptoms. In contrast, striatal dopaminergic denervation was significantly associated with step length (estimate: 0.883, 95% CI [0.29, 1.48], p = 0.005), explaining about 24% of the variability but was dependent of HY stage. More severe cardiac noradrenergic denervation was associated with lower gait velocity, independent of striatal dopaminergic denervation and HY stage, impacting both step length and cadence. These results suggest independent contributions of the peripheral autonomic system degeneration on gait dynsfunction in PD.

CMRLCCOA: Multi-Strategy Enhanced Coati Optimization Algorithm for Engineering Designs and Hypersonic Vehicle Path Planning.

The recently introduced coati optimization algorithm suffers from drawbacks such as slow search velocity and weak optimization precision. An enhanced coati optimization algorithm called CMRLCCOA is proposed. Firstly, the Sine chaotic mapping function is used to initialize the CMRLCCOA as a way to obtain better-quality coati populations and increase the diversity of the population. Secondly, the generated candidate solutions are updated again using the convex lens imaging reverse learning strategy to expand the search range. Thirdly, the Lévy flight strategy increases the search step size, expands the search range, and avoids the phenomenon of convergence too early. Finally, utilizing the crossover strategy can effectively reduce the search blind spots, making the search particles constantly close to the global optimum solution. The four strategies work together to enhance the efficiency of COA and to boost the precision and steadiness. The performance of CMRLCCOA is evaluated on CEC2017 and CEC2019. The superiority of CMRLCCOA is comprehensively demonstrated by comparing the output of CMRLCCOA with the previously submitted algorithms. Besides the results of iterative convergence curves, boxplots and a nonparametric statistical analysis illustrate that the CMRLCCOA is competitive, significantly improves the convergence accuracy, and well avoids local optimal solutions. Finally, the performance and usefulness of CMRLCCOA are proven through three engineering application problems. A mathematical model of the hypersonic vehicle cruise trajectory optimization problem is developed. The result of CMRLCCOA is less than other comparative algorithms and the shortest path length for this problem is obtained.

Torque, Work, Power, and Muscle Activity Analysis According to Self-Selected Slow, Moderate, and Fast Angular Velocity for Knee Extension

Torque, Work, Power, and Muscle Activity Analysis According to Self-Selected Slow, Moderate, and Fast Angular Velocity for Knee Extension

Predicting heat transfer rate and system entropy based on combining artificial neural network with numerical simulation

PurposeThe purpose of this paper is to present numerical simulations for magnetohydrodynamics natural convection of a nanofluid flow inside a cavity with an H-shaped obstacle based on combining artificial neural network (ANN) with the finite element method (FEM), and predict the heat transfer rate and system entropy.Design/methodology/approachThe enclosure is assumed to be inclined. Changing the inclination angle results in a different obstacle shape, which affects the buoyancy force. Hence, different configurations of the contours of the fluid flow, isotherms and the entropy of the system are obtained. The outer walls of the cavity as well as the central part of the obstacle are kept adiabatic. The left vertical portion of the hindrance is cooled, whereas the right vertical part of the obstacle is a heated wall. Using dimensionless variables allows obtaining a dimensionless version of the governing system of equations that is solved via the consistency FEM. The coupled problem of pressure and velocity is overcome via the Increment Pressure Correction Scheme, which is known for its accuracy and stability for similar simple problems. A numerical computation is performed across a broad range of the governing parameters. A total of 304 data sets were used in the development of an ANN model. That data set was conducted from the numerical simulations. The data set underwent optimization, with 70% sets used for training the model, 15% for validation and another 15% for the testing phase. The training of the network model used the Levenberg–Marquardt training algorithm.FindingsFrom the numerical simulations, it is concluded that the H-shaped obstacle boosts heat transfer rate in comparison with the I-shaped case. Also, raising the value of the inclination angle improves the entropy of the system presented by the Bejen number. Furthermore, strength heat transfer rate is obtained via decreasing the Hartmann number while this decrease decays the values of the Bejen number for both positive and negative amounts of the nonlinear Boussinesq parameter. Slower velocity and a better heat transfer rate characterize nanofluid compared with pure fluid. Leveraging the capabilities of the ANN, the developed model adeptly forecasts the values of both the average Nusselt and Bejen numbers with a high degree of accuracy.Originality/valueA novel fusion of FEM and ANN has been tailored to forecast the heat transfer rate and system entropy of MHD natural convective flow within an inclined cavity containing an H-shaped obstacle, amid various physical influences.

A recurrent missense variant in ITPR3 causes demyelinating Charcot-Marie-Tooth with variable severity

Abstract Charcot-Marie-Tooth (CMT) disease is a neuromuscular disorder affecting the peripheral nervous system. The diagnostic yield in demyelinating CMT (CMT1) is typically ∼80-95%, of which at least 60% is due to the PMP22 gene duplication. The remainder of CMT1 is more genetically heterogeneous. We used whole exome and whole genome sequencing data included in the GENESIS database to investigate novel causal genes and mutations in a cohort of ∼2,670 individuals with CMT neuropathy. A recurrent heterozygous missense variant p.Thr1424Met in the recently described CMT gene ITPR3, encoding IP3R3 (inositol 1,4,5-trisphosphate receptor 3) was identified. This previously reported p.Thr1424Met change was present in 33 affected individuals from nine unrelated families from multiple populations, representing an unusual recurrence rate at a mutational hotspot, strengthening the gene-disease relationship (GnomADv4 allele frequency 1.76e-6). Sanger sequencing confirmed the co-segregation of the CMT phenotype with the presence of the mutation in autosomal dominant and de novo inheritance patterns, including a four-generation family with multiple affected second-degree cousins. Probands from all families presented with slow nerve conduction velocities, matching the diagnostic category of CMT1. Remarkably, we observed a uniquely variable clinical phenotype for age at onset and phenotype severity in p.Thr1424Met carrying patients, even within families. Finally, we present data supportive of a dominant-negative effect of the p.Thr1424Met mutation with associated changes in protein expression in patient-derived cells.

Mapping Motor Cortical Network Excitability and Connectivity Changes in De Novo Parkinson's Disease.

Transcranial magnetic stimulation-electroencephalography (TMS-EEG) has demonstrated decreased excitability in the primary motor cortex (M1) and increased excitability in the pre-supplementary motor area (pre-SMA) in moderate-advanced Parkinson's disease (PD). The aim was to investigate whether these abnormalities are evident from the early stages of the disease, their behavioral correlates, and relationship to cortico-subcortical connections. Twenty-eight early, drug-naive (de novo) PD patients and 28 healthy controls (HCs) underwent TMS-EEG to record TMS-evoked potentials (TEPs) from the primary motor cortex (M1) and the pre-SMA, kinematic recording of finger-tapping movements, and a 3T-MRI (magnetic resonance imaging) scan to obtain diffusion tensor imaging (DTI) reconstruction of white matter (WM) tracts connecting M1 to the ventral lateral anterior thalamic nucleus and pre-SMA to the anterior putamen. We found reduced M1 TEP P30 amplitude in de novo PD patients compared to HCs and similar pre-SMA TEP N40 amplitude between groups. PD patients exhibited smaller amplitude and slower velocity in finger-tapping movements and altered structural integrity in WM tracts of interest, although these changes did not correlate with TEPs. M1 hypoexcitability is a characteristic of PD from early phases and may be a marker of the parkinsonian state. Pre-SMA hyperexcitability is not evident in early PD and possibly emerges at later stages of the disease. © 2024 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

Local climate change velocities and evolutionary history explain multidirectional range shifts in a North American butterfly assemblage.

Species are often expected to shift their distributions either poleward or upslope to evade warming climates and colonise new suitable climatic niches. However, from 18-years of fixed transect monitoring data on 88 species of butterfly in the midwestern United States, we show that butterflies are shifting their centroids in all directions, except towards regions that are warming the fastest (southeast). Butterflies shifted their centroids at a mean rate of 4.87 km year-1. The rate of centroid shift was significantly associated with local climate change velocity (temperature by precipitation interaction), but not with mean climate change velocity throughout the species' ranges. Species tended to shift their centroids at a faster rate towards regions that are warming at slower velocities but increasing in precipitation velocity. Surprisingly, species' thermal niche breadth (range of climates butterflies experience throughout their distribution) and wingspan (often used as metric for dispersal capability) were not correlated with the rate at which species shifted their ranges. We observed high phylogenetic signal in the direction species shifted their centroids. However, we found no phylogenetic signal in the rate species shifted their centroids, suggesting less conserved processes determine the rate of range shift than the direction species shift their ranges. This research shows important signatures of multidirectional range shifts (latitudinal and longitudinal) and uniquely shows that local climate change velocities are more important in driving range shifts than the mean climate change velocity throughout a species' entire range.

Visual Fixation of Skull-Vibration-Induced Nystagmus in Patients with Peripheral Vestibulopathy.

Nystagmus induced by applying an intense vibratory stimulus to the skull (SVIN) indicates vestibular functional asymmetry. In unilateral vestibular loss, a 100 Hz bone-conducted vibration given to either mastoid immediately causes a primarily horizontal nystagmus. The test is performed in darkness to avoid visual fixation (VF) but there are no data about how much VF affects the often-intense SVIN. The aim is to analyze the amount of reduction in SVIN when VF is allowed during testing. Thus, all patients seen in a tertiary hospital for vertigo or dizziness with positive SVIN were included. SVIN was recorded for 10 s for each condition: without VF (aSVINwo) and with VF (aSVINw). We obtained an aSVINwo and an aSVINw as average slow-phase velocities (SPV) without and with VF. VF index (FISVIN) was calculated as the ratio of SPV. Among the 124 patients included, spontaneous nystagmus (SN) was found in 25% and the median slow phase velocity (mSPV) (without VF) of SN was 2.6 ± 2.4°/s. Mean FISVIN was 0.27 ± 0.29. FISVIN was 0 in 42 patients, and FISVIN between 0 and 1 was found in 82 (mean FISVIN 0.39 ± 0.02). Fixation suppression was found in all patients with SVIN in cases of peripheral vestibulopathy. FISVIN clearly delineates two populations of patients: with or without a complete visual reduction in nystagmus.

KAJIAN KESESUAIAN DAN DAYA DUKUNG FISIK KAWASAN PANTAI YANAIN DI NEGERI HULALIU KECAMATAN PULAU HARUKUKABUPATEN MALUKU TENGAH

This research aims to assess the suitability and physical carrying capacity of Yanain Beach in Hulaliu State, Pulau Haruku District, Central Maluku Regency as a tourist destination. The methodology employed in this study involved a survey, collecting both primary and secondary data through direct field observations. Primary data included visual observations and measurements at Station 1 and Station 2, as well as interviews with visitors to the beach area. Data analysis utilized tourism suitability index and physical carrying capacity of the beach area. Yanain Beach covers an area of 2.14 hectares with a sandy beach width of 18.76 meters, providing ample space for tourist activities. Additionally, it features a relatively shallow depth (1.48 meters), slow current velocity (0.0847 m/s), and high water clarity (&gt;80%), making it an attractive tourist destination. Based on the calculated values, it was concluded that Yanain Beach in Hulaliu Village is "Highly Suitable" for various tourism activities, with an average TSI score of 92.25%. Furthermore, the analysis of the beach's carrying capacity indicated that the area can accommodate 641 people per day for recreation purposes, and 407 people per day for swimming and bathing activities at the beach.