Non-motor symptoms, including depression, anxiety, sleep disturbances, pain and cognitive dysfunction, are a much more important predictor of quality of life than the severity of dystonia. To assess the effect of Botulinum toxin on non-motor symptoms and quality of life in patients with adult-onset idiopathic focal dystonia. Patients aged > 18 years diagnosed with idiopathic focal dystonia were recruited in this longitudinal cohort study. The severity of dystonia, non-motor symptoms, and quality of life were evaluated using the BFMDRS, DNMSQuest, and EQ-5D at baseline and 1 and 3 months after botulinum toxin. 65 patients were recruited with a median age of 59 years. Blepharospasm was the most common phenomenology. 49.2

Introduction Post-surgical dysphagia is one complication particularly common in early postoperative period after Anterior cervical spine surgery (ACSS). However, the pathophysiology of dysphagia after surgery has not been well understood. This study aimed to analyse the frequency and risk factors for developing dysphagia following ACSS and find an effective program to prevent and treat. Materials and methods A prospective observational study was conducted on 50 patients undergoing ACSS from April 2021 to Oct 2022 at the Department of Orthopedics, Guru Gobind Singh Medical College and Hospital, Punjab (India). Patients were in the age group of 27 to 60 years. The indications for cervical surgeries were traumatic, degenerative, infective and neoplastic involving C2 to C7 vertebra with signs of neural compression unresponsive to conservative treatment. Data on patient age, gender, duration of surgery, intraoperative blood loss, segment operated and the number of segments operated were collected. Follow up time was 24 weeks. Results Incidence of dysphagia was 20 % (10/50) within first week which reduced to zero at completion of six months of follow-up. Dysphagia was present in 2 % (1/50) patients in age group 21–40 years and 18 % (9/50) patients in age group of 41–60 years. 14.6 % (6/41) males and 44 % (4/9) of females had dysphagia. Prevalence of dysphagia in patients with one affected segment was 9.5 % (4/42), two segments was 80 % (4/5) and three segments was 50 % (1/2). Mean duration of surgery in patients with post-operative dysphagia was 115 mins. Mean blood loss in patients with post-operative dysphagia was 171.40 mL Mean Et (endotracheal) tube cuff pressure in patients with post-operative dysphagia was 24.70 cm H2O. Within the first week of surgery, there were 10 cases out of which one was mild, six were moderate and three were severe. Conclusion Despite the fact that some inconsistency is there in the literature regarding risk factors it can be safely concluded from our study that incidence of post-operative dysphagia can be reduced by decreasing blood loss during surgery, reducing surgery time and optimizing Endotracheal tube cuff pressure during surgery.

BACKGROUND:Epicardial adipose tissue (EAT) has a contributory role in the progression of heart failure. We tested whether dapagliflozin reduces EAT in adults with type 2 diabetes (T2D) and heart failure and explored links with systemic inflammation and cardiac structure. METHODS:This analysis is based on pooled data from two phase 2, single-centre, double-blind, placebo-controlled randomised trials (REFORM and DAPA-LVH) conducted in Scotland. Exactly 122 participants with T2D and stage B or C heart failure were randomised to dapagliflozin 10 mg once daily or placebo for 12 months. Cardiac magnetic resonance imaging (CMR) was used to assess EAT. At baseline and follow-up, the inflammatory markers TNF, IL-1, IL-6, IL-10, and CRP were measured. RESULTS:At baseline, obesity was common (75% with BMI ≥30 kg/m2) and heart-failure phenotypes were balanced (HFpEF 51%, HFrEF 49%). After 12 months, dapagliflozin significantly reduced EAT independently of changes in BMI (-1.16 ± 0.18 vs. +0.36 ± 0.19 cm2, p < 0.001), BMI (-1.17 ± 0.16 vs. -0.18 ± 0.17 kg/m2, p < 0.001), and left ventricular mass (-3.53 ± 1.77 vs. +1.57 ± 1.83 g, p = 0.048) compared with placebo. CONCLUSION:Dapagliflozin shrinks EAT and LV mass independently of BMI in T2D patients with stage B/C heart failure, supporting EAT as a modifiable target of SGLT2 inhibition. The absence of parallel changes in systemic inflammation suggests primarily local mechanisms.

OBJECTIVE:To assess chemokine receptor CXCR4 expression in lung parenchyma and on peripheral immune cells in systemic sclerosis-related interstitial lung disease (SSc-ILD) patients. METHODS:SSc-ILD patients underwent 68Ga- CPCR4 Trifluoroacetate positron emission tomography (PET) scan, SUVmean in different lung regions and architecturally abnormal areas, and receiver operating characteristic (ROC) curves were analysed. CXCR4 expression on peripheral immune cells using flow cytometer was studied and correlated with the different lung regions. In addition, subset analysis of CXCR4 expression by clinical subset (early, progressive, stable), ILD pattern and anti-Scl-70 positivity were done. RESULTS:On PET, SSc-ILD patients showed higher median SUVmean uptake of CXCR4 in the whole lung (0.56; P < 0.0001), different lung regions and architecturally abnormal areas than controls. The highest area under curve (AUC) was observed in dorsobasal regions (AUC-0.91; P < 0.0001) and reticular with architecturally distorted areas (AUC-0.95; P < 0.0001). The progressive subset had higher whole lung median SUVmean (0.73) than early (0.49; P < 0.0001) and stable (0.45; P < 0.0001) subsets, and AUC than early and stable subsets. Usual interstitial pneumonia pattern ILD showed higher CXCR4 uptake compared with non-specific interstitial pneumonia (P = 0.0032). Additionally, a trend for higher uptake was noted in anti-Scl70 positive patients as compared with anti-Scl70 negative ones. SSc-ILD patients had higher CD4+CXCR4+T cells (P = 0.0003) and CD8+CXCR4+T cells (P = 0.0013) and showed moderate to strong association on correlation with the lung parenchymal regions. CONCLUSION:In SSc-ILD, CXCR4 expression is upregulated in both lung parenchyma and peripheral T cells, significantly in progressive and UIP subsets. CXCR4 expression is a potential tool for activity assessment and prognostication.

Developing and assessing subgrid-scale models for characterizing atmospheric and wind farm turbulence is one of the key research areas within the wind energy community. This article presents the interaction of atmospheric and wind farm turbulence using scale-adaptive large-eddy simulation. Atmospheric turbulence has been incorporated by employing the stochastic forcing method to linearized Navier-Stokes equations, which interacted with a staggered cluster of utility-scale 41 wind turbines. The effect of atmospheric turbulence on wind turbine wakes was characterized by comparing scale-adaptive large-eddy simulation results with three reference data obtained from three other subgrid-scale models: Smagorinsky model, Deardorff's one-equation turbulence kinetic energy model, and dynamic Deardorff model. The results suggest that vortex-stretching and strain skewness can accelerate wake recovery because scale-adaptive large-eddy simulation captured more than 90% of the turbulence kinetic energy, outperforming the other three models. The atmospheric turbulence in a wind farm has been characterized by considering mean vertical profiles, wake recovery, turbulence statistics, wavelet energy spectra, and power production. Finally, the interaction between atmospheric turbulence and wind turbines was evaluated through joint probability distribution of the second and the third invariant of velocity gradient and strain rate tensors and that of vortex-stretching and strain skewness. The results highlight the importance of considering vortex-stretching and strain skewness in turbine design, siting decisions, and wind farm layout optimization.

An indigenously designed High Energy Pulse LIDAR (HEPL) system was re -integrated and installed at ARIES, Nainital, for probing the atmosphere and to analyse the Mie scatterers. After the characterization of the Photomultiplier tube and Discriminator, about 50 quality checked valid measurements were investigated. Reasonably good signals were obtained upto similar to.5 km for 30 m altitude resolution, similar to 5 km for 150 m, and similar to 8 km for 300 m resolutions in the vertical, which were utilized to investigate the aerosol and cloud profiles. The system was able to capture the clouds at various altitudes under different atmospheric conditions. Elevated aerosol layers were also detected at about 3 and 3.5 km over the observation site. Such unique measurements from the data void complex Himalayan terrain highlight the need for continuous monitoring of the aerosol loading caused by transport of pollutants from continental locations and far-off distances that affect the air quality and Himalayan glaciers as well.

The boundary layer plays a vital role in governing the local atmospheric dynamics and meteorology, including the vertical transport of moisture, momentum, energy, and air pollutants. Hence, accurate parameterization of the boundary layer processes is essential in atmospheric models such as the regional climate models (RCMs). However, evaluations of different boundary layer schemes within the Weather Research and Forecasting (WRF) model remain nearly non-existing over complex terrains of the Himalayan region. In this regard, six different planetary boundary layer (PBL) schemes: Yonsei University (YSU), Mellor-Yamada-Nakanishi-Niino level 3 (MYNN3), Shin-Hong Scale-aware (SHSS), Mellor-Yamada-Janji ' c (MYJ), Asymmetric Convective Model version 2 (ACM2), and Quasi Normal Scale Elimination (QNSE) in the WRF model have been evaluated against observations during the Ganges Valley Aerosol Experiment (GVAX). The evaluation is carried out for clear-sky conditions during spring (March 22-27, 2012) against surface-based, balloon-borne, and radar wind profiler (RWP) observations. The MYJ, YSU, and SHSS performed well in simulating the temperature and specific humidity, whereas the ACM2 (MYNN3) shows lower (higher) bias in T2 and higher (lower) bias in Q2. Model performance is limited in reproducing wind field (r similar to 0.3) with different PBL schemes over this region. Model performance is seen to vary significantly with the choice of PBL schemes; nevertheless, all schemes could typically capture the daytime maxima in boundary layer height with some overestimation. The competing effects of synoptic versus local circulation were found to control the boundary layer evolution over the Himalayas, affecting the model performance. The model better reproduced the boundary layer evolution during strong surface flow regimes, while a prominent nocturnal peak in boundary layer height is observed during the weak mean flow conditions associated with topography-induced local circulations. The model performance is limited for reproducing such mountain meteorological features in contrast to the homogeneous terrains where such nocturnal peaks are absent. This study may serve as a reference for selecting a suitable PBL scheme for regional climate modeling studies in the future.

PURPOSE:Epstein-Barr virus (EBV) reactivation in allogeneic hematopoietic stem cell transplantation (allo-HCT) recipients can lead to significant complications including post-transplant lymphoproliferative disease. Despite progress in managing EBV reactivation in allo-HCT recipients, data on clinical characteristics and prognostic implications of EBV viral load remain limited. Here, we aim to evaluate the prevalence, identify risk factors, and assess the clinical implications of EBV-DNA positivity in allo-HCT recipients. METHODS:A retrospective audit of laboratory records for EBV load monitoring in allo-HCT recipients during the period from 2021 to 2023 was performed. EBV viral load was assessed using quantitative PCR. The medical records were reviewed for clinical features, identifying risk factors, and prognostic impact of EBV-DNA positivity. RESULTS:A total of 40 patients with a median age of 20.5 years were included. Patients were divided into two groups based on presence (>600 copies/mL) and absence of EBV-DNA. We observed EBV-DNA positivity in 16 (40%) patients with a median EBV viral load of 12,400 copies/ml. Patients with EBV-DNA positivity exhibited a higher incidence of acute graft versus host disease (p=0.039). Patients with EBV-DNA positivity tended to have poorer 1-year overall survival and disease-free survival, although the results did not reach statistical significance. CONCLUSION:Our data highlights the importance of monitoring EBV viral load in predicting the outcome in terms of overall survival in allo-HCT recipients. The development of GVHD has also surfaced as a significant element increasing the likelihood of EBV-DNA positivity. It is imperative to conduct further research and establish comprehensive protocols for the routine monitoring and management of EBV post-allo-HCT.

A 34-year-old woman presented with insidious onset and gradually progressive cerebellar ataxia over 10 years, with generalised convulsions. On examination, there were myoclonic jerks, choreiform movements and cerebellar syndrome. Her family history suggested an autosomal dominant inheritance with anticipation. Genetic analysis for trinucleotide repeat disorders led to a diagnosis of dentatorubral-pallidoluysian atrophy (60 CAG repeats in the atrophin-1 gene). This rare spinocerebellar ataxia should be considered in the differential diagnosis of inherited ataxia when combined with seizures and chorea. Other features suggesting a repeat expansion disorder are variable phenotypes within the same family and possible anticipation.

BACKGROUND:The use of urinary sodium to guide diuretics in acute heart failure is recommended by experts and the most recent European Society of Cardiology guidelines. However, there are limited data to support this recommendation. The ENACT-HF study (Efficacy of a Standardized Diuretic Protocol in Acute Heart Failure) investigated the feasibility and efficacy of a standardized natriuresis-guided diuretic protocol in patients with acute heart failure and signs of volume overload.METHODS:ENACT-HF was an international, multicenter, open-label, pragmatic, 2-phase study, comparing the current standard of care of each center with a standardized diuretic protocol, including urinary sodium to guide therapy. The primary end point was natriuresis after 1 day. Secondary end points included cumulative natriuresis and diuresis after 2 days of treatment, length of stay, and in-hospital mortality. All end points were adjusted for baseline differences between both treatment arms.RESULTS:Four hundred one patients from 29 centers in 18 countries worldwide were included in the study. The natriuresis after 1 day was significantly higher in the protocol arm compared with the standard of care arm (282 versus 174 mmol; adjusted mean ratio, 1.64; P<0.001). After 2 days, the natriuresis remained higher in the protocol arm (538 versus 365 mmol; adjusted mean ratio, 1.52; P<0.001), with a significantly higher diuresis (5776 versus 4381 mL; adjusted mean ratio, 1.33; P<0.001). The protocol arm had a shorter length of stay (5.8 versus 7.0 days; adjusted mean ratio, 0.87; P=0.036). In-hospital mortality was low and did not significantly differ between the 2 arms (1.4% versus 2.0%; P=0.852).CONCLUSIONS:A standardized natriuresis-guided diuretic protocol to guide decongestion in acute heart failure was feasible, safe, and resulted in higher natriuresis and diuresis, as well as a shorter length of stay.

BACKGROUND:Human leukocyte antigen (HLA) allele frequencies have a known association with the pathogenesis of various autoimmune diseases.METHODS:We recruited 31 Indian patients of acquired dermal macular hyperpigmentation (ADMH) and 60 unrelated, age-and-gender-matched healthy controls. After history and clinical examination, 5 ml of blood in EDTA vials was collected. These samples were subjected to DNA extraction and the expression of HLA A, B, C, DR, DQ-A, and DQ-B was studied.RESULTS:There was a predominance of females with a gender ratio of 23 : 8 and the most common phototype was Fitzpatrick type IV (83.9%). There was a significant association of HLA A*03:01 (OR: 5.8, CI: 1.7-17.0, P = 0.005), HLA B*07:02 (OR: 5.3, CI: 1.9-14.6, P = 0.003), HLA C*07:02 (OR: 4.3, CI: 1.8-9.6, P = 0.001), HLA DRB1*10:01 (OR: 7.6, CI: 1.7-38.00, P = 0.022), and HLA DRB1*15:02 (OR: 31.0, CI: 4.4-341.8, P < 0.001) with patients compared to controls, whereas HLA DQB*03:01 was less associated with patients compared to controls (OR: 0.2, CI: 0.0-0.6, P = 0.009).CONCLUSION:Patients with ADMH are more likely to have the HLA A*03:01, HLA B 07*02, HLA C*07:02, HLA DRB1*10:01, HLA DRB1*15:02 and less likely to have the HLA DQB*03:01 allele. Larger cohort studies may thus be conducted studying these specific alleles.