摘要： Background The objective was to determine the association between perioperative risk factors and brain imaging abnormalities on neurologic outcome in neonates with hypoplastic left heart syndrome (HLHS) or d-Transposition of the great arteries (d-TGA) who underwent cardiac surgery including cardiopulmonary bypass. Methods A retrospective analysis of neonates with HLHS or d-TGA undergoing cardiac surgery including cardiopulmonary bypass between 2009 and 2017 was performed. Perioperative risk factors and Andropoulos' Brain Injury Scores from pre- and postoperative brain magnetic resonant images (MRI) were correlated to outcome assessments on patients between 5 and 23 months of age. Neurologic outcome was measured using the Pediatric Stroke Outcome Measure (PSOM) and Pediatric Version of the Glasgow Outcome Scale-Extended (GOS-E). Results Fifty-three neonates met our enrollment criteria (24 HLHS, 29 d-TGA). Mechanical ventilation > 12 days and DHCA > 40 min were associated with worse outcome. MRI measures of brain injuries were not associated with worse outcome by PSOM or GOS-E. Conclusion For HLHS and d-TGA patients, duration of mechanical ventilation and DHCA are associated with adverse neurologic outcome. Neonatal brain MRI commonly demonstrates acquired brain injuries, but the clinical impact of these abnormalities are not often seen before 2 years of age. Impact Acquired brain injury is common in high-risk neonates with CHD but poor neurological outcome was not predicted by severity of injury or lesion subtype. Longer stay in ICU is associated with postoperative brain injuries on MRI. Total duration of ventilation > 12 days is predictive of adverse neurological outcome scores. DHCA > 40 min is associated with adverse neurological outcome scores. Neurological outcome before 2 years of age is more affected by the clinical course than by cardiac diagnosis.

关键词： anti-oxidant   anti-cholinesterase   alpha-amylase   alpha-glucosidase   pancreatic lipase   mechanical parameters  

摘要： BACKGROUND: Obesity and type 2 diabetes mellitus are the most extended current chronic diseases and also Alzheimer pathology which is a progressive degenerative neurological disorder. Therefore, finding effective enzyme inhibitors responsible for the development of these diseases are essential. Therefore, the aim of this study was to investigate the impact of fruit puree (Cydonia oblonga,Ziziphus jujubeandMalus domestica) and pomegranate juice cultivar ('Mollar de Elche' and 'Wonderful') of dried pomegranate sheets (DS) on the inhibition of enzymes associated with metabolic (alpha-amylase, alpha-glucosidase, and pancreatic lipase activity), and neurological disorder (acetylcholinesterase and butyrylcholinesterase activity). Quality properties (colour coordinates, texture properties and sensory characteristics) of DS were also studied. In addition, it was researched the effect of storage conditions (4 months at 4 and 20 degrees C) on phenolic content. RESULTS: DS from jujube had the highest antioxidant capacity and were characterized by the highest storage stability with respect to phenolic compounds. The alpha-amylase and alpha-glucosidase half maximal inhibitory concentration (IC50, in mg mL(-1)) inhibition of DS ranged from 107 to 216 and from 55.2 to values indicating no effect, respectively. The inhibition toward pancreatic lipase (IC50 < 5 mg mL(-1)), acetylcholinesterase (ranged 9.15-22.2%) and butyrylcholinesterase (ranged 20.6-48.6%) was increased with the presence of total flavonoids and hydroxycinnamic acids content (identifying mainly in DS from quinces). It is noteworthy that none of the samples presented off-flavour notes, supporting the high quality of the products. CONCLUSION: DS can be an innovative supplement to a diet as a snack used in the prevention of neurological changes and disorders of carbohydrate and lipid metabolism. (c) 2020 Society of Chemical Industry

摘要： Pregnancy and early childhood are periods with high plasticity in neurological development. Environmental perturbations during these sensitive windows can have lifelong developmental consequences. This review summarizes key findings relevant to the effects of air pollution on neurological development. Mounting evidence suggests that exposure to air pollution, both during pregnancy and childhood, is associated with childhood developmental outcomes ranging from changes in brain structures to subclinical deficits in developmental test scores, and, ultimately, developmental disorders such as attention-deficit/hyperactivity disorders or autism spectrum disorders. Although the biological mechanisms of effects remain to be elucidated, multiple pathways are probably involved and include oxidative stress, inflammation, and/or endocrine disruption. Given the alarming global increase in developmental disorders in recent years, and increased human exposures to pollution, it is critical to reduce personal and community-level exposures through tight collaboration of interdisciplinary and multi-level bodies including community partners, physicians, industry partners, policy makers, public health practitioners, and researchers.

摘要： Aims To investigate current awareness and practices of neurological prognostication in comatose cardiac arrest (CA) patients. Methods An anonymous questionnaire was distributed to 1600 emergency physicians in 75 hospitals which were selected randomly from China between January and July 2018. Results 92.1% respondents fulfilled the survey. The predictive value of brain stem reflex, motor response and myoclonus was confirmed by 63.5%, 44.6% and 31.7% respondents, respectively. Only 30.7% knew that GWR value < 1.1 indicated poor prognosis and only 8.1% know the most commonly used SSEP N20. Status epilepticus, burst suppression and suppression were considered to predict poor outcome by only 35.0%, 27.4% and 20.9% respondents, respectively. Only 46.7% knew NSE and only 24.7% knew S-100. Only a few respondents knew that neurological prognostication should be performed later than 72 hours from CA either in TTM or non-TTM patients. In practice, the most commonly used method was clinical examination (85.4%). Only 67.9% had used brain CT for prognosis and 18.4% for MRI. NSE (39.6%) was a little more widely used than S-100 beta (18.0%). However, SSEP (4.4%) and EEG (11.4%) were occasionally performed. Conclusions Neurological prognostication in CA survivors had not been well understood and performed by emergency physicians in China. They were more likely to use clinical examination rather than objective tools, especially SSEP and EEG, which also illustrated that multimodal approach was not well performed in practice.

关键词： Large arterial occlusions   Long-term prognosis   Endovascular therapy   Clinical trials  

摘要： Objectives: To clarify the relationship between early neurological symptoms and long-term functional outcomes of acute ischemic stroke, which would be beneficial for patient management and determining clinical study criteria of novel therapeutic interventions. Materials and Methods: We retrieved data from the Recovery by Endo vascular Salvage for Cerebral Ultra-acute Embolism Japan Registry 2 (RESCUE Japan Registry 2) and investigated the association between 24-and 72-hour National Institutes of Health Stroke Scale (NIHSS) and 90-day modified Rankin Scale (mRS) scores, stratified by the site of occlusion (carotid or vertebrobasilar circulatory large arterial occlusion [ACO or PCO, respectively]) and endovascular recanalization therapy (EVT) performance. We examined the correlation using Spearma?s rank correlation coefficient (rho). Predictive accuracies of 24-and 72 hour NIHSS scores for good outcomes at 90 days (defined as mRS score of 0-2) were evaluated by receiver operating characteristic (ROC) analyses and the corresponding areas under the curves (AUCs). Results: Among the 2420 patients, 1745 had ACO (971 with EVT, 774 without EVT) and 263 had PCO (127 with EVT, 136 without EVT). The 24-and 72-hour NIHSS scores were significantly associated with 90-day mRS scores and accurately predicted good outcomes (all rhos >0.76, all AUCs >0.86). In the ACO group, there were differences in rho and AUC depending on EVT performance and the time from onset to NIHSS acquisition, but no differences were observed in the PCO group. Conclusions: EVT performance and time frame should be considered when determining the criteria of novel therapeutic interventions, especially for patients with ACO.

摘要： Outpatients more often represent the most frequent neurological disorders, especially neurodegenerative and chronic disorders, allowing students to observe the natural history of the neurological diseases and the interaction between neurologists following their regular patients. In internal medicine, students with a mixed inpatient and outpatient experience felt themselves to be more responsible for patient care and more like "doctors" in the outpatient sector. In this issue of the I European Journal of Neurology i , Martins et al. [2] compared students' final assessment scores between those who learned neurology primarily from outpatients and those who learned neurology primarily from inpatients. [Extracted from the article]

关键词： cerebral palsy   enteral nutrition   gastrointestinal diseases   nutrition   paediatrics  

摘要： Objectives: The main aim of this study was to determine the impact on clinical practice of the first European Society of Gastroenterology, Hepatology, and Nutrition (ESPGHAN) position paper on the diagnosis and management of nutritional and gastrointestinal problems in children with neurological impairment (NI). Methods: In this pilot-study, a web-based questionnaire was distributed between November, 2019 and June, 2020, amongst ESPGHAN members using the ESPGHAN newsletter. Fifteen questions covered the most relevant aspects on nutritional management and gastrointestinal issues of children with NI. A descriptive analysis of responses was performed. Results: A total of 150 health professionals from 23 countries responded to the survey. A considerable variation in clinical practice concerning many aspects of nutritional and gastrointestinal management of children with NI was observed. The most frequently used method for diagnosing oropharyngeal dysfunction was the direct observation of meals with or without the use of standardised scores (n = 103). Anthropometric measurements were the most commonly used tools for assessing nutritional status (n = 111). The best treatment for gastroesophageal reflux disease (GERD) was considered to be proton pump inhibitor therapy by most (n = 116) participants. Regarding tube feeding, nearly all respondents (n = 114) agreed that gastrostomy is the best enteral access to be used for long-term enteral feeding. Fundoplication was indicated at the time of gastrostomy placement especially in case of uncontrolled GERD. Conclusions: More studies are required to address open questions on adequate management of children with NI. Identifying knowledge gaps paves the way for developing updated recommendations and improving patient care.

关键词： Global warming   Air pollution   Stroke   Neurodegenerative disorders   Oxidative stress  

摘要： Climate change is one of the biggest challenges humanity is facing in the 21st century. Two recognized sequelae of climate change are global warming and air pollution. The gradual increase in ambient temperature, coupled with elevated pollution levels have a devastating effect on our health, potentially contributing to the increased rate and severity of numerous neurological disorders. The main aim of this review paper is to shed some light on the association between the phenomena of global warming and air pollution, and two of the most common and debilitating neurological conditions: stroke and neurodegenerative disorders. Extreme ambient temperatures induce neurological impairment and increase stroke incidence and mortality. Global warming does not participate in the etiology of neurodegenerative disorders, but it exacerbates symptoms of dementia, Alzheimer?s disease (AD) and Parkinson?s Disease (PD). A very close link exists between accumulated levels of air pollutants (principally particulate matter), and the incidence of ischemic rather than hemorrhagic strokes. People exposed to air pollutants have a higher risk of developing dementia and AD, but not PD. Oxidative stress, changes in cardiovascular and cerebrovascular haemodynamics, excitotoxicity, microglial activation, and cellular apoptosis, all play a central role in the overlap of the effect of climate change on neurological disorders. The complex interactions between global warming and air pollution, and their intricate effect on the nervous system, imply that future policies aimed to mitigate climate change must address these two challenges in unison.

关键词： cardiolipin   lipids   mitochondria   mitochondrial disease   nervous system   neurodegeneration  

摘要： Over the past decade, it has become clear that lipid homeostasis is central to cellular metabolism. Lipids are particularly abundant in the central nervous system (CNS) where they modulatemembrane fluidity, electric signal transduction, and synaptic stabilization. Abnormal lipid profiles reported in Alzheimer's disease (AD), Parkinson's disease (PD), amyotrophic lateral sclerosis (ALS), and traumatic brain injury (TBI), are further support for the importance of lipid metablism in the nervous system. Cardiolipin (CL), a mitochondria-exclusive phospholipid, has recently emerged as a focus of neurodegenerative disease research. Aberrant CL content, structure, and localization are linked to impaired neurogenesis and neuronal dysfunction, contributing to aging and the pathogenesis of several neurodegenerative diseases, such as AD and PD. Furthermore, the highly tissue-specific acyl chain composition of CL confers it significant potential as a biomarker to diagnose and monitor the progression in several neurological diseases. CL also represents a potential target for pharmacological strategies aimed at treating neurodegeneration. Given the equipoise that currently exists between CL metabolism, mitochondrial function, and neurological disease, we review the role of CL in nervous system physiology and monogenic and neurodegenerative disease pathophysiology, in addition to its potential application as a biomarker and pharmacological target.

关键词： cancer therapy   channelopathy   neurological disease   SENP   SUMOylation  

摘要： Neurological diseases are relatively complex diseases of a large system;however, the detailed mechanism of their pathogenesis has not been completely elucidated, and effective treatment methods are still lacking for some of the diseases. The SUMO (small ubiquitin-like modifier) modification is a dynamic and reversible process that is catalyzed by SUMO-specific E1, E2, and E3 ligases and reversed by a family of SENPs (SUMO/Sentrin-specific proteases). SUMOylation covalently conjugates numerous cellular proteins, and affects their cellular localization and biological activity in numerous cellular processes. A wide range of neuronal proteins have been identified as SUMO substrates, and the disruption of SUMOylation results in defects in synaptic plasticity, neuronal excitability, and neuronal stress responses. SUMOylation disorders cause many neurodegenerative diseases, such as Parkinson's disease, Alzheimer's disease, and Huntington's disease. By modulating the ion channel subunit, SUMOylation imbalance is responsible for the development of various channelopathies. The regulation of protein SUMOylation in neurons may provide a new strategy for the development of targeted therapeutic drugs for neurodegenerative diseases and channelopathies.