关键词： Bystander CPR   Cardiac arrest   Good neurological recovery   Response time interval   Survival discharge  

摘要： Background: This study intended to find out how association between response time interval (RTI) and good neurological outcome is affected by bystander CPR. We hypothesized that bystander CPR will ensure positive effect in relationship between RTI and clinical outcome. Methods: A retrospective, observational study was made with Pan-Asian Resuscitation Outcome Study data from January 2009 to December 2016. Six cities from four Asian countries were selected. EMS-treated, non-traumatic witnessed out-of-hospital cardiac arrest (OHCA) cases were included. General demographic data, prehospital cardiac arrest details and clinical outcome were collected and analyzed according to whether bystander CPR was performed. Good neurological outcome and survival discharge were primary and secondary outcomes. Results: A total of 13,245 OHCA cases were analyzed. Median EMS response time intervals were 6 min, regardless of bystander CPR. Dividing into RTI time range by 3 min, good neurological outcome and survival discharge were only significant in 3 to 6 minutes group (adjusted odds ratio [AOR] 1.42, 1.17-1.73 95% confidence interval [CI] and AOR 1.31, 1.15-1.51 95% CI) in non-bystander CPR group but in bystander CPR group significant RTI time range was 3 to 9 min (AOR 2.02, 1.82, 1.62-2.52, 1.48-2.25 95% CI for primary, AOR 1.66, 1.43, 1.41-1.96, 1.22-1.67 95% CI for secondary). Conclusions: As response time interval increased, slower deterioration of good neurological outcome and survival discharge was shown in cardiac arrest patients with bystander CPR performed. If bystander CPR is provided, RTI time range showing significant neurological outcome and survival improvement seems to be relatively lengthened. (c) 2020 Elsevier Inc. All rights reserved.

关键词： Anti-neuronal antibodies   Immunocytochemistry   Autoimmune encephalitis   Pediatric neurology  

摘要： Background: Anti-neuronal autoantibodies have been reported as the cause of several neurologic disorders other than encephalitis. Unfortunately, data are mostly based on serum analysis. Predictions about pathogenicity are thus limited. To determine the presence of so far unidentified autoantibody-derived neuroreactivity we analyzed cerebrospinal fluid (CSF) of children with neurological disorders other than encephalitis. Patients and methods: We did a retrospective analysis of CSF from 254 children with various neurologic diseases other than encephalitis and searched for reactivity against neuronal surface antigens by immunofluorescence on unfixed murine brain sections (tissue-based assay, TBA) and by commercial cell-based assays (CBA). A semi-quantitative fluorescence score classified our results and we described the clinical course of all positive patients with strong neuroreactivity. Results: Strong anti-neuronal IgG immunoreactivity of unknown antigen specificity was detected in CSF samples of 10 pediatric patients (4%, n = 10/254) with unsolved neurological disorders. CSF inflammatory markers were elevated. Most patients did not or only partly recover. Five screening-positive patients presented with a combination of headache and visual impairment due to optic nerve atrophy. Our data suggest to consider inflammatory, autoantibody-related etiologies, especially in cases without definite diagnoses. Conclusions: We present an overview of CSF neuroreactivity in children with neurological disorders other than encephalitis, indicating the presence of unidentified anti-neuronal autoantibodies. As TBA enables screening for unknown autoantibodies, we suggest this method as a second step if commercial CBAs do not yield a result. Further studies are necessary to characterize such antibodies, evaluate pathogenicity, and answer the question whether positive CSF neuroreactivity should prompt an immunotherapeutic approach. (c) 2020 European Paediatric Neurology Society. Publis

关键词： Spinal cord injury   magnetic resonance imaging   Electromyography   Nerve conduction   ASIA score  

摘要： Background To investigate the relationship between neurological deficit and subsequent recovery as assessed by ASIA score and findings of electrodiagnostic study in acute spinal cord injury (SCI) patients. Methods Thirty-five patients with acute SCI presenting within 48 h of injury were clinically evaluated for the level, extent, and severity of SCI according to the ASIA standards in a tertiary-level care center. Electrodiagnostic studies of bilateral two motor (tibial and peroneal), one sensory (sural) nerves, and five muscles [iliopsoas, vastus medialis, tibialis anterior, gastrocnemius, and extensor hallucis longus (EHL)] were conducted and repeated at 3 months and 6 months. Results The neurological recovery was highly significant (p < 0.001) at 6 months. The difference in mean amplitude was statistically significant (p < 0.05) for all the nerves;mean conduction velocity significant for peroneal and sural nerves, and with no significant difference in mean latency. The differences in mean recruitment of motor unit potential (MUP) and mean peak-to-peak amplitude were highly significant (p < 0.001). Statistically significant kappa agreement between neurological recovery according to ASIA score and nerve conduction velocity was found for right tibial nerve (K = 0.324);electromyography finding of recruitment of MUP with right and left tibialis anterior (k = 0.400) and left EHL (k = 0.407);peak-to-peak amplitude with right tibialis anterior (k = 0.211), right gastrocnemius (k = 0.390), and right EHL (k = 0.211). Conclusions There is a strong relationship between electrodiagnostic findings and ASIA scoring to predict neurological deficit and subsequent recovery after acute traumatic SCI. Serial neurologic evaluation by ASIA score and electrodiagnostic studies may help in designing customized rehabilitation programs for the patients according to the expected neurological recovery;and evaluating future research in the field of SCI with more scientific authenticity.

关键词： White matter   Myelination   Neurological disorders   Alzheimer's disease   Huntington's disease   Multiple sclerosis  

摘要： The central nervous system is simply divided into two distinct anatomical regions based on the color of tissues, i.e. the gray and white matter. The gray matter is composed of neuronal cell bodies, glial cells, dendrites, immune cells, and the vascular system, while the white matter is composed of concentrated myelinated axonal fibers extending from neuronal soma and glial cells, such as oligodendrocyte precursor cells (OPCs), oligodendrocytes, astrocytes, and microglia. As neuronal cell bodies are located in the gray matter, great attention has been focused mainly on the gray matter regarding the understanding of the functions of the brain throughout the neurophysiological areas, leading to a scenario in which the function of the white matter is relatively underestimated or has not received much attention. However, increasing evidence shows that the white matter plays highly significant and pivotal functions in the brain based on the fact that its abnormalities are associated with numerous neurological diseases. In this review, we will broadly discuss the pathways and functions of myelination, which is one of the main processes that modulate the functions of the white matter, as well as the manner in which its abnormalities are related to neurological disorders.

关键词： Lung cancer   Overall survival   EGFR plus   Personal status   Spine metastases  

摘要： Background: For patients with non-small cell lung cancer (NSCLC), the spinal column is the most common site for bone metastasis. Studies that assess survival prognostic factors associated with specific lung spinal metastases (SpM) are weak and required the incorporation of genotype mutations. Methods: A prospective French national multicenter database of patients treated for SpM between January 2014 and 2017.818 lung SpM were diagnosed over the course or at the time of diagnosis of 210 consecutive patients with NSCLC. Results: Median overall survival (OS) time for all patients from the lung SpM event was 5.9 months (SD 0.609). For 122 patients (61%), lung tumor and SpM were diagnosed synchronously. In univariate analysis, good World Health Organisation (WHO) status (p < 0.0001), ambulatory status (Frankel score) (p < 0.0001), the absence of spine epiduritis (p < 0.0001), immunotherapy after SpM diagnosis (p < 0.0001), ALK gene rearrangement (p < 0.0001) and EGFR mutation (p < 0.0001) were associated with longer survival, whereas spine surgery showed no association (0.141). Cox multivariate proportional hazard model identified that EGFR + status (HR: 0.339, 95% CI 0.166-0.693;p = 0.003), good WHO status (p < 0.0001) and good neurological status (Frankel E;p < 0.001 and D;p = 0.018) were associated with higher median OS. Whereas the other factors, including ALK + status, epiduritis and immunotherapy were not independent prognostic factors of survival. Conclusion: Survival in SpM must be prognosticated from general health performance status: clinical (WHO) and neurological (Frankel) as well as the EGFR mutation status. Immunotherapy, surgery and epiduritis have not demonstrated prognostic value. Therefore, surgical prognostic scoring algorithms should incorporate genotype subtypes in NSCLC cancers to adapt surgical treatment.

关键词： Cell signaling   Scaffold proteins   MAP kinase   C-RAF   RAF1   KRAS   NF-kB   Wnt   LC3   Mitophagy   Cancer   Drug discovery   Inflammation   Cardiac diseases   Alzheimer disease   Parkinson disease   Autoimmune disease   Osteoporosis   Osteoclastogenesis   Cardio-oncology   Melanogenesis  

摘要： Over the last three decades, the scaffold proteins prohibitins-1 and -2 (PHB1/2) have emerged as key signaling proteins regulating a myriad of signaling pathways in health and diseases. Small molecules targeting PHBs display promising effects against cancers, osteoporosis, inflammatory, cardiac and neurodegenerative diseases. This review provides an updated overview of the various classes of PHB ligands, with an emphasis on their mechanism of action and therapeutic potential. We also describe how these ligands have been used to explore PHB signaling in different physiological and pathological settings.

摘要： Background The COVID-19 pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is of a scale not seen since the 1918 influenza pandemic. Although the predominant clinical presentation is with respiratory disease, neurological manifestations are being recognised increasingly. On the basis of knowledge of other coronaviruses, especially those that caused the severe acute respiratory syndrome and Middle East respiratory syndrome epidemics, cases of CNS and peripheral nervous system disease caused by SARS-CoV-2 might be expected to be rare. Recent developments A growing number of case reports and series describe a wide array of neurological manifestations in 901 patients, but many have insufficient detail, reflecting the challenge of studying such patients. Encephalopa thy has been reported for 93 patients in total, including 16 (7%) of 214 hospitalised patients with COVID-19 in Wuhan, China, and 40 (69%) of 58 patients in intensive care with COVID-19 in France. Encephalitis has been described in eight patients to date, and Guillain-Barre syndrome in 19 patients. SARS-CoV-2 has been detected in the CSF of some patients. Anosmia and ageusia are common, and can occur in the absence of other clinical features. Unexpectedly, acute cerebrovascular disease is also emerging as an important complication, with cohort studies reporting stroke in 2-6% of patients hospitalised with COVID-19. So far, 96 patients with stroke have been described, who frequently had vascular events in the context of a pro-inflammatory hypercoagulable state with elevated C-reactive protein, D-dimer, and ferritin. Where next? Careful clinical, diagnostic, and epidemiological studies are needed to help define the manifestations and burden of neurological disease caused by SARS-CoV-2. Precise case definitions must be used to distinguish nonspecific complications of severe disease (eg, hypoxic encephalopathy and critical care neuropathy) from those caused directly or indirectly by

关键词： First-episode psychosis   global functioning   response   soft neurological signs   schizophrenia  

摘要： Background Neurological Examination Abnormalities (NES) are quantified by measuring subtle, partially localizable (cerebello-thalamo-prefrontal cortical circuit) and heritable neurological signs comprising sensory integration, motor coordination and complex motor sequencing that are associated with first-episode psychosis (FEP). A few studies have evaluated NES longitudinally and as a predictor for diagnostic and response classification, but these studies have been confounded, underpowered and divergent. We examined (1) baseline and longitudinal NES differences between diagnostic and year 1 response groups;(2) if NES predicts diagnostic and response groups and (3) relationships between clinical variables and NES measures in antipsychotic-naive FEP. Methods NES and clinical measures were obtained for FEP-schizophrenia (FEP-SZ,n= 232), FEP non-schizophrenia (FEP-NSZ,n= 117) and healthy controls (HC,n= 204). Response groups with >25% improvement in average year 1 positive and negative symptomatology scores were classified as responsive (n= 97) and <25% improvement as non-responsive (n= 95). Analysis of covariance, NES trajectory analysis and logistic regression models assessed diagnostic and response group differences. Baseline and longitudinal NES relationships with clinical variables were performed with Spearman correlations. Data were adjusted for age, sex, race, socioeconomic status and handedness. Results Cognitive perceptual (COGPER) score was better than repetitive motor (REPMOT) at differentiating FEP-SZ from FEP-NSZ and distinguishing responders from non-responders. We identified significant group-specific associations between COGPER and worse GAF, positive and negative symptomatology and some of these findings persisted at 1-year assessment. Conclusion NES are an easy to administer, bedside-elicited, endophenotypic measure and could be a cost-effective clinical tool in antipsychotic-naive FEP.

关键词： Cockayne syndrome   COFS   neonatal form   NER associated disease   XP-CS  

摘要： Nucleotide excision repair associated diseases comprise overlapping phenotypes and a wide range of outcomes. The early stages still remain under-investigated and underdiagnosed, even although an early recognition of the first symptoms is of utmost importance for appropriate care and genetic counseling. We systematically collected clinical and molecular data from the literature and from newly diagnosed NER patients with neurological impairment, presenting clinical symptoms before the age of 12 months, including foetal cases. One hundred and eighty-five patients were included, 13 with specific symptoms during foetal life. Arthrogryposis, microcephaly, cataracts, and skin anomalies are the most frequently reported signs in early subtypes. NonERCC6/CSBorERCC8/CSAgenes are overrepresented compared to later onset cohorts: 19% patients of this cohort presented variants inERCC1,ERCC2/XPD,ERCC3/XPBorERCC5/XPG. ERCC5/XPGis even the most frequently involved gene in foetal cases (10/13 cases, [4/7 families]). In this cohort, the mutated gene, the age of onset, the type of disease, severe global developmental delay, IUGR and skin anomalies were associated with earlier death. This large survey focuses on specific symptoms that should attract the attention of clinicians towards early-onset NER diagnosis in foetal and neonatal period, without waiting for the completeness of classical criteria.