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The aim of this study is to determine whether the initial symptom associates with motor progression in spinocerebellar ataxias (SCAs). SCAs are clinically heterogeneous and the initial presentation may represent different subtypes of SCA with different motor progression. We studied 317 participants with SCAs1, 2, 3, and 6 from the Clinical Research Consortium for SCAs (CRC-SCA) and repeatedly measured the severity of ataxia for 2 years. SCA patients were divided into gait-onset and non-gait-onset (speech, vision, and hand dexterity) groups based on the initial presentation. In addition to demographic comparison, we employed regression models to study ataxia progression in these two groups after adjusting for age, sex, and pathological CAG repeats. The majority of SCA patients had gait abnormality as an initial presentation. The pathological CAG repeat expansions were similar between the gait-onset and non-gait-onset groups. In SCA1, gait-onset group progressed slower than non-gait-onset group, while gait-onset SCA6 group progressed faster than their counterpart. In addition, the disease presented 9 years later for SCA2 gait-onset group than non-gait-onset group. Initial symptoms of SCA3 did not influence age of onset or disease progression. The initial symptom in each SCA has a different influence on age of onset and motor progression. Therefore, gait and non-gait-onset groups of SCAs might represent different subtypes of the diseases.

BACKGROUND: Postural tremor can sometimes occur in spinocerebellar ataxias (SCAs). However, the prevalence and clinical characteristics of postural tremor in SCAs are poorly understood, and whether SCA patients with postural tremor have different ataxia progression is not known. METHODS: We studied postural tremor in 315 patients with SCA1, 2, 3, and 6 recruited from the Clinical Research Consortium for Spinocerebellar Ataxias (CRC-SCA), which consists of 12 participating centers in the United States, and we evaluated ataxia progression in these patients from January 2010 to August 2012. RESULTS: Among 315 SCA patients, postural tremor was most common in SCA2 patients (SCA1, 5.8%; SCA2, 27.5%; SCA3, 12.4%; SCA6, 16.9%; p = 0.007). SCA3 patients with postural tremor had longer CAG repeat expansions than SCA3 patients without postural tremor (73.67 ± 3.12 vs. 70.42 ± 3.96, p = 0.003). Interestingly, SCA1 and SCA6 patients with postural tremor had a slower rate of ataxia progression (SCA1, β = -0.91, p < 0.001; SCA6, β = -1.28, p = 0.025), while SCA2 patients with postural tremor had a faster rate of ataxia progression (β = 1.54, p = 0.034). We also found that the presence of postural tremor in SCA2 patients could be influenced by repeat expansions of ATXN1 (β = -1.53, p = 0.037) and ATXN3 (β = 0.57, p = 0.018), whereas postural tremor in SCA3 was associated with repeat lengths in TBP (β = 0.63, p = 0.041) and PPP2R2B (β = -0.40, p = 0.032). DISCUSSION: Postural tremor could be a clinical feature of SCAs, and the presence of postural tremor could be associated with different rates of ataxia progression. Genetic interactions between ataxia genes might influence the brain circuitry and thus affect the clinical presentation of postural tremor.

To meet the growing demand for integrated monitoring of complex power grid equipment, it is necessary to improve the situational awareness model of power transformers. The model is expected to assist monitoring personnel in timely identifying transformers with deteriorating trends among massive and discrete monitoring information, and to make responses in advance. However, the current transformer state awareness technology generally has the problem of single data source and poor timeliness, and still requires monitoring personnel to make artificial analysis and prediction in combination with telemetry information, which cannot fully meet the requirements of power grid equipment monitoring. This paper is based on multi-source data fusion technology, through associating and mining transformer alarm information, equipment maintenance records and power transmission and transformation online monitoring data, to extract the dimension features of transformer operation situation assessment. By constructing a multi-layer perceptron model, a transformer state transition model based on the principle of Markov chain is established, which can predict possible defects 2 h in advance and achieve good results, and determine the transformer state early warning index, providing sufficient time for monitoring personnel to deploy transformer operation and maintenance work in advance. Finally, the effectiveness of the method proposed in this paper is proved by the case of transformer crisis state in a city substation, and the method proposed in this paper has important significance for transformer state early warning. [ABSTRACT FROM AUTHOR]

Developing hole-transport materials (HTMs) with high hole mobility is critical for constructing efficient perovskite solar cells (PSCs). We present a design strategy for improving hole mobility and PSC performance using a stable zinc complex-based HTM BPZ23. Compared to its non-metal counterpart (BP21), the BPZ23 demonstrated a 59.42% increase in hole mobility, resulting in a good perovskite layer with reduced trap-assisted recombination. As a result, the power conversion efficiency of the fabricated inverted PSC using BPZ23 is up to 19.75%, which is one of the highest reported for any transition metal complex-based HTM employed in inverted PSC. This research demonstrates a simple and efficient method for increasing hole mobility in HTMs and improving the photovoltaic performance of PSCs. [ABSTRACT FROM AUTHOR]

Excessive use of synthetic fertilizer has been proven to be an important cause of greenhouse gas emissions and water pollution in many Asian countries. This article aims to provide a better understanding of rice farmers' preferences for a chemical fertilizer reduction scheme (CFRS) by integrating economic and psychometric factors. A survey of 292 farmers was conducted in Taiwan. The results show that farmers using more chemical fertilizers have a negative attitude towards implementing SAP and less trust in government and would prefer a higher entry payment over an eco-label. On the other hand, farmers using less chemical fertilizer prefer to receive an eco-label rather than incentive payments. Our findings indicate that an eco-label and higher entry payment could overcome the psychological factors that hinder farmers' engagement. This study provides policymakers with insights into farmers' decision-making, which could lead to chemical fertilizer reduction, thereby mitigating climate change. [ABSTRACT FROM AUTHOR]

Objective: Whole-course nutrition management (WNM) has been proven to improve outcomes and reduce complications. We conducted this randomized controlled trial to validate its effectiveness in patients undergoing pancreatoduodenectomy (PD). Methods: From 1 December 2020, to 30 November 2023, this single-center randomized clinical trial was conducted at the Department of Hepatobiliopancreatic Surgery in a major hospital in Beijing, China. Participants who were undergoing PD were enrolled and randomly allocated to either the WNM group or the control group. The primary outcome was the incidence of postoperative complications. Subgroup analysis in patients who were at nutritional risk was performed. Finally, a 6-month follow-up was conducted and the economic benefit was evaluated using an incremental cost-effectiveness ratio (ICER). Results: A total of 84 patients were randomly assigned (1:1) into the WNM group and the control group. The incidences of total complications (47.6% vs. 69.0%, P= 0.046), total infections (14.3% vs. 33.3%, P=0.040), and abdominal infection (11.9% vs. 31.0%, P= 0.033) were significantly lower in the WNM group. In the subgroup analysis of patients at nutritional risk, 66 cases were included (35 cases in the WNM group and 31 cases in the control group). The rate of abdominal infection (11.4% vs. 32.3%, P =0.039) and postoperative length of stay (23.1 ± 10.3 vs. 30.4 ± 17.2, P=0.046) were statistically different between the two subgroups. In the 6-month follow-up, more patients reached the energy target in the WNM group (97.0% vs. 79.4%, P=0.049) and got a higher daily energy intake (1761.3 ± 339.5 vs. 1599.6 ± 321.5, P=0.045). The ICER suggested that WNM saved 31 511 Chinese Yuan (CNY) while reducing the rate of total infections by 1% in the intention-to-treat (ITT) population and saved 117 490 CNY in patients at nutritional risk, while WNM saved 31 511 CNY while reducing the rate of abdominal infections by 1% in the ITT population and saved 101 359 CNY in patients at nutritional risk. Conclusion: In this trial, whole-course nutrition management was associated with fewer total postoperative complications, total and abdominal infections, and was cost-effective, especially in patients at nutritional risk. It seems to be a favorable strategy for patients undergoing PD. [ABSTRACT FROM AUTHOR]

Steamed ginseng water (SGW) is a by-product of the repeated thermal processing of red ginseng, which is characterized by a high bioactive content, better skin care activity, and a large output. However, its value has been ignored, resulting in environmental pollution and resource waste. In this study, UHPLC-Q-Exactive-MS/MS liquid chromatography–mass spectrometry and multivariate statistical analysis were conducted to characterize the compositional features of the repeated thermaltreated SGW. The antioxidant activity (DPPH, ABTS, FRAP, and OH) and chemical composition (total sugars, total saponins, and reducing and non-reducing sugars) were comprehensively evaluated based on the entropy weighting method. Four comparison groups (groups 1 and 3, groups 1 and 5, groups 1 and 7, and groups 1 and 9) were screened for 37 important common difference markers using OPLS-DA analysis. The entropy weight method was used to analyze the weights of the indicators; the seventh SGW sample was reported to have a significant weight. The results of this study suggest that heat treatment time and frequency can be an important indicator value for the quality control of SGW cycling operations, which have great potential in antioxidant products. [ABSTRACT FROM AUTHOR]

Objective: Essential tremor is among the most prevalent neurological diseases. Diagnosis is based entirely on neurological evaluation. Historically, there were few postmortem brain studies, hindering attempts to develop pathologically based criteria to distinguish essential tremor from control brains. However, an intensive effort to bank essential tremor brains over recent years has resulted in postmortem studies involving >200 brains, which have identified numerous degenerative changes in the essential tremor cerebellar cortex. Although essential tremor and controls have been compared with respect to individual metrics of pathology, there has been no overarching analysis to derive a combination of metrics to distinguish essential tremor from controls. We asked whether there is a constellation of pathological findings that separates essential tremor from controls, and how well that constellation performs. Methods: Analyses included 100 essential tremor brains from the essential tremor centralized brain repository and 50 control brains. A standard tissue block from the cerebellar cortex was used to quantify 11 metrics of pathological change. Three supervised classification algorithms were investigated, with data divided into training and validation samples. Results: Using three different algorithms, we illustrate the ability to correctly predict a diagnosis of essential tremor, with sensitivity and specificity >87%, and in the majority of situations, >90%. We also provide a web‐based application that uses these metric values, and based on specified cutoffs, determines the likely diagnosis. Interpretation: These analyses set the stage for use of pathologically based criteria to distinguish clinically diagnosed essential tremor cases from controls, at the time of postmortem. [ABSTRACT FROM AUTHOR]

Friedreichs ataxia (FRDA) is an inherited, progressive neurodegenerative disease that typically affects teenagers and young adults. Therapeutic strategies and disease insight have expanded rapidly over recent years, leading to hope for the FRDA population. There is currently no US FDA-approved treatment for FRDA, but advances in research of its pathogenesis have led to clinical trials of potential treatments. This article reviews emerging therapies and discusses future perspectives, including the need for more precise measures for detecting changes in neurologic symptoms as well as a disease-modifying agent.

BACKGROUND: Depression is a common comorbidity in spinocerebellar ataxias (SCAs) but its association with ataxia progression is not well understood. OBJECTIVES: To study the prevalence and influence of depressive symptoms in SCAs. METHODS: We studied 300 participants with SCA 1, 2, 3 and 6 from the Clinical Research Consortium for Spinocerebellar Ataxias (CRC-SCA) and repeatedly measured depressive symptoms by the 9-item Patient Health Questionnaire (PHQ-9) along with other clinical features including ataxia, functional status, and quality of life every 6 months for 2 years. We employed regression models to study the effects of depressive symptoms on clinical progression indexed by Scale for Assessment and Rating of Ataxia (SARA), Unified Huntingtons Disease Rating Scale Part IV (UHDRS-IV) and EQ5D after adjusting for age, sex and pathological CAG repeats. RESULTS: Comorbid depression is common in SCAs (26%). Although the baseline prevalence of depression was similar among different SCA types, suicidal ideation was more frequently reported in SCA3 (65%). Depressive symptoms were associated with SARA scores but did not significantly progress over time within 2 years or deteriorate by increased numbers of pathological CAG repeats. The effects of depression on ataxia progression varied across different SCA types. Nevertheless, depression had consistently negative and significant impact on functional status and quality of life in all SCAs, even after accounting for ataxia progression. CONCLUSIONS: Depressive symptoms are not simply the consequence of motor disability in SCAs. Comorbid depression per se contributes to different health outcomes and deserves more attention when caring patients with SCAs.

The cerebellum has been largely overlooked in Alzheimer's disease, despite increasing evidence implicating its cognitive capacities and functional networks, which interacts with cerebral cortex to subserve cognition. A study by Lin et al. has indicated that the cerebellum is part of the integrated network in amnestic mild cognitive impairment (aMCI), a prodromal state of Alzheimer's disease. The aMCI patients exhibited weaker cerebello-parietal functional connectivity but stronger cerebellar coupling with precuneus cortex, posterior cingulate gyrus, and caudate nucleus. These alterations in cerebello-cortical connectivity correlated with cognitive performance, suggesting a dynamic change of cerebello-cortical network related to cognitive change in aMCI. [ABSTRACT FROM AUTHOR]

The aim of this study was to investigate the association between drug exposure and disease severity in SCA types 1, 2, 3 and 6. The Clinical Research Consortium for Spinocerebellar Ataxias (CRC-SCA) enrolled 319 participants with SCA1, 2, 3, and 6 from 12 medical centers in the United States and repeatedly measured clinical severity by the Scale for Assessment and Rating of Ataxia (SARA), the Unified Huntington's Disease Rating Scale part IV (UHDRS-IV), and the 9-item Patient Health Questionnaire during July 2009 to May 2012. We employed generalized estimating equations in regression models to study the longitudinal effects of coenzyme Q10 (CoQ10), statin, and vitamin E on clinical severity of ataxia after adjusting for age, sex, and pathological CAG repeat number. Cross-sectionally, exposure to CoQ10 was associated with lower SARA and higher UHDRS-IV scores in SCA1 and 3. No association was found between statins, vitamin E, and clinical outcome. Longitudinally, CoQ10, statins, and vitamin E did not change the rates of clinical deterioration indexed by SARA and UHDRS-IV scores within 2 years. CoQ10 is associated with better clinical outcome in SCA1 and 3. These drug exposures did not appear to influence clinical progression within 2 years. Further studies are warranted to confirm the association.

The zero-relaxation limit of Euler–Poisson systems for ion dynamics in a slow time scale leads to drift-diffusion equations. This fact was justified in previous works. This paper concerns global error estimates between solutions of Euler–Poisson systems and that of drift-diffusion equations. In the proof, we use Sobolev inequalities and employ a multiplier related to the electric potential. [ABSTRACT FROM AUTHOR]

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