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.

关键词： VZV   Neurological complications   Cerebrospinal fluid   PCR  

摘要： The clinical manifestations of neurological complications associated with varicella zoster virus (VZV) are non-specific and indistinguishable from those of other viral infections. Therefore, the definite diagnosis requires evidence of VZV infection in cerebrospinal fluid (CSF). The aim of this study was to determine the frequency of VZV DNA detection in CSF of patients with neurological diseases in order to obtain information concerning involvement of VZV infection in neuropathology in the country. This study is a retrospective survey of test results obtained from January 2015 to October 2019. During this period, 411 CSF specimens were tested for the presence of VZV DNA by nested PCR. Fisher's exact test was used to test for statistically significant difference in the frequency of VZV DNA positivity of CSF specimens from different groups. Of all 411 tested CSF samples, 11.2% were positive for VZV DNA. The highest VZV prevalence was detected in CFS from patients with meningitis-18.2%, followed by patients with cranial neuritis (15.4%), encephalitis (12.2%), Guillain-Barre syndrome (11.1%), myelitis (10%), and with other neurological syndromes (8.2%). The difference of VZV prevalence in CSF of patients according to the gender and age was not statistically significant. Our results indicated that VZV is a frequent causative agent of neurological diseases, suggesting an important role of VZV infection for neuropathology in the country. Therefore, efforts for wider application of VZV identification in CSF to facilitate faster onset of antiviral treatment and further strategies concerning varicella zoster virus vaccines in the country are needed.

关键词： Parkinson's disease   Multiple sclerosis   Narcolepsy   SARS-CoV2   COVID-19   Neuroinflammation   Risk factors   Neurodegeneration   Demyelination   Sleepiness  

摘要： SARS-CoV2 infection is responsible for a complex clinical syndrome, named Coronavirus Disease 2019 (COVID-19), whose main consequences are severe pneumonia and acute respiratory distress syndrome. Occurrence of acute and subacute neurological manifestations (encephalitis, stroke, headache, seizures, Guillain-Barre syndrome) is increasingly reported in patients with COVID-19. Moreover, SARS-CoV2 immunopathology and tissue colonization in the gut and the central nervous system, and the systemic inflammatory response during COVID-19 may potentially trigger chronic autoimmune and neurodegenerative disorders. Specifically, Parkinson's disease, multiple sclerosis and narcolepsy present several pathogenic mechanisms that can be hypothetically initiated by SARS-CoV2 infection in susceptible individuals. In this short narrative review, we summarize the clinical evidence supporting the rationale for investigating SARS-CoV2 infection as risk factor for these neurological disorders, and suggest the opportunity to perform in the future SARS-CoV2 serology when diagnosing these disorders.

摘要： It is increasingly recognized that there is significant heterogeneity in phenotype and clinical trajectories in Alzheimer's disease as well as Lewy Body and Vascular dementia. Relatively common neurodegenerative disorder, Lewy Body Dementia (LBD) presents with psychiatric features. Mood disorders are common and often precede core symptoms of LBD. Patient with LBD especially early in the course of disease are at increased risk for suicide. Psychosis is one of the most debilitating symptoms of LBD as it is an independent risk factor for nursing home placements, increased caregiver distress and mortality. Visual hallucinations are the most common psychotic manifestation in LBD. Auditory, olfactory, tactile and gustatory hallucinations and delusions are less frequently found. Global cognitive deficits including attention, frontal executive dysfunction, visuospatial abnormalities, language and verbal memory were associated with the presence of psychosis in Lewy Body Dementia. Glucocerebrosidase gene (GBA) mutations and polymorphisms found to be associated with LBD. Up to 25% of Alzheimer's disease (AD) cases do not show the typical neuropathology. Subtypes and atypical non-amnestic AD presentations have been formalized in the International Working Group (IWG) clinical diagnosis criteria. Frontal signs and symptoms are often seen in Alzheimer's disease especially in patients with early onset. In those clinical situations differential diagnosis especially distunguising between Clinical diagnosis of atypical AD has significantly been improved by the availability of biomarkers, including cerebrospinal fluid levels of Aβ42, total tau and phosphorylated tau181. MRI volumetric estimation of hippocampal and medial temporal atrophy, and amyloid brain PET imaging. We will review atypical forms of AD from these varied perspectives to help improve diagnostic confidence among clinicians. Cerebral amyloid angiopathy (CAA) is a highly prevalent pathologic finding in the elderly contrib