关键词： 神经解剖学   神经病学   神经外科学   教研室主任   神经生理学   上海医学院   人体解剖学   医学院院长  

摘要： 由耶鲁大学神经学、神经生理学及神经外科学教授哈尔·布鲁门菲尔德(Hal Blumenfeld)编著,空军军医大学人体解剖与组织胚胎学教研室暨梁銶琚脑研究中心主任李云庆教授,西北大学医学院院长、西北大学神经病学系主任、西北大学脑科学研究中心主任赵钢教授,复旦大学附属中山医院党委书记、复旦大学上海医学院神经病学系主任汪昕教授,以及大连医科大学人体解剖学教研室主任隋鸿锦教授共同主译的《临床神经解剖学:病例解析》已于2021年3月由天津科技翻译出版有限公司出版。

关键词： 中国医师协会   中华医学会   神经科学学会   神经退行性疾病   神经病学   老年医学   学会常务理事   认知障碍  

摘要： 上海交通大学医学院附属瑞金医院神经内科主任医师,博士生导师,中华医学会神经病学分会认知障碍学组委员,中国医师协会神经病学分会认知障碍学组委员,中华医学会老年医学分会流行病学组委员,中国老年医学学会认知障碍专业委员会常务委员,中国神经科学学会神经退行性疾病分会委员,上海市衰老与退行性疾病学会常务理事。

关键词： 雨课堂   形成性评价   床旁   神经病学  

摘要： 目的为改进神经病学教学方法单一现状,将雨课堂结合形成性评价应用于神经病学床旁教学中。方法将兰州大学第二临床医学院2016级临床医学专业农村定向班学生60名,按配对法分为应用雨课堂联合形成性评价的试验组和应用传统教学模式的对照组。制定考试的量化指标,在学期末用于评定教学效果。结果试验组学生临床思维、操作技能考核成绩高于对照组,差异有统计学意义(P<0.05),98.7%的学生对雨课堂联合形成性评价教学效果满意。结论雨课堂联合形成性评价教学模式能够显著改善神经病学床旁教学效果。

关键词： EEG   CT perfusion   Hyperacute ischemic stroke   NIHSS   Biomedical signal processing  

摘要： Brain electrical activity in acute ischemic stroke is related to the hypoperfusion of cerebral tissue as manifestation of neurovascular coupling. EEG could be applicable for bedside functional monitoring in emergency settings. We aimed to investigate the relation between hyper-acute ischemic stroke EEG changes, measured with bedside wireless-EEG, and hypoperfused core-penumbra CT-perfusion (CTP) volumes. In addition, we investigated the association of EEG and CTP parameters with neurological deficit measured by NIHSS. We analyzed and processed EEG, CTP and clinical data of 31 anterior acute ischemic stroke patients registered within 4.5 h from symptom onset. Delta/alpha ratio (DAR), (delta + theta)/(alpha + beta) ratio (DTABR) and relative delta power correlated directly (rho = 0.72;0.63;0.65, respectively), while alpha correlated inversely (rho = - 0.66) with total hypoperfused volume. DAR, DTBAR and relative delta and alpha parameters also correlated with ischemic core volume (rho = 0.55;0.50;0.59;- 0.51, respectively). The same EEG parameters and CTP volumes showed significant relation with NIHSS at admission. The multivariate stepwise regression showed that DAR was the strongest predictor of NIHSS at admission (p < 0.001). The results of this study showed that hyper-acute alterations of EEG parameters are highly related to the extent of hypoperfused tissue highlighting the value of quantitative EEG as a possible complementary tool in the evaluation of stroke severity and its potential role in acute ischemic stroke monitoring.

关键词： Precision neurorehabilitation   Precision neurology   Personalized medicine  

关键词： COVID-19 disease   SARS-CoV-2 associated neurological disease   Immune-associated genes   Lung inflammation   Cytokine storm  

摘要： Background: Long period of SARS-CoV-2 infection has been associated with psychiatric and cognitive disorders in adolescents and children. SARS-CoV-2 remains dormant in the CNS leading to neurological complications. The wide expression of ACE2 in the brain raises concern for its involvement in SARS-CoV-2 infection. Though, the mechanistic insights about blood-brain barriers (BBB) crossing by SARS-CoV-2 and further brain infection are still not clear. Moreover, the mechanism behind dormant SARS-CoV-2 infections leading to chronic neurological disorders needs to be unveiled. There is an urgent need to find out the risk factor involved in COVID-19associated neurological disease. Therefore, the role of immune-associated genes in the pathogenesis of COVID19 associated neurological diseases is presented which could contribute to finding associated genetic risk factors. Method: The search utilizing multiple databases, specifically, EMBASE, PubMed (Medline), and Google Scholar was performed. Moreover, the literature survey on the involvement of COVID-19, neuropathogenesis, and its consequences was done. Description: Persistent inflammatory stimuli may promote the progression of neurodegenerative diseases. An increased expression level of cytokine, chemokine, and decreased expression level of immune cells has been associated with the COVID-19 patient. Cytokine storm was observed in severe COVID-19 patients. The nature of SARS-CoV-2 infection can be neuroinflammatory. Genes of immune response could be associated with neurodegenerative diseases. Conclusion: The present review will provide a useful framework and help in understanding COVID-19-associated neuropathogenesis. Experimental studies on immune-associated genes in COVID-19 patients with neurological manifestations could be helpful to establish its neuropathogenesis.

关键词： Mucolipidosis types II and III   Respiratory insufficiency   Spinal cord compression   Tetraplegia  

摘要： In mucopolysaccharidoses (MPS), spinal cord compression (SCC) resulting from glycosaminoglycan (GAG) accumulation is a critical complication that can cause significant neurological and respiratory morbidities. However, clinically similar disorders such as mucolipidosis types II and III (ML) with SCC have been scarcely reported. Herein, we report four patients with ML who had SCC. Brain MRI revealed progressive spinal canal stenosis and SCC. In addition, T2-weighted high signal changes in the cervical cord were detected in two cases. Severe cases of SCC were detected as early as 1 year of age. All cases had respiratory problems. One case showed severe hypoxia and another, severe sleep apnea. In two cases, respiratory insufficiency and tetraplegia rapidly progressed as SCC progressed. Then, the patients became bedridden and needed artificial ventilation. In addition, two of the four patients died of respiratory failure. The autopsy of one patient revealed a compressed cervical cord and marked dura mater thickening due to GAG accumulation. These findings suggest that the accumulation of substrates in the dura mater caused SCC in the patients with ML. Our cases indicate that SCC is expected to be a common and critical complication of ML and MPS. MRI evaluation of cervical involvements and careful clinical observation are required in patients with ML. © 2021 The Japanese Society of Child Neurology

关键词： Neurologic   Outcome   Prognosis   Wilson's disease  

摘要： Wilson's disease (WD) is an autosomal recessive genetic disorder of copper metabolism, and WD patients can present with neurologic symptoms. We aimed to report the general characteristics and prognosis of a Turkish series of WD patients with neurological manifestations. A total of 12,352 patients were screened from the patient database, and 53 WD patients were included. Patients were classified based on the predominant neurological syndrome type including tremor, dystonia, parkinsonism, or discrete neurological signs and were classified as having "good outcome," "stable," and "poor outcome" according to their treatment response. There were 32 male and 21 female patients, aged 20-66 years. The mean follow-up was 11.3 +/- 4.56 years. Sixty-two percent of patients presented predominantly with neurological symptoms. Neurological WD diagnosis was established after a mean time delay of 2.3 years from the WD diagnosis. The most common neurological manifestation was dystonia, followed by tremor and parkinsonism. Fifteen patients had a family history of WD. Consanguinity was present in 20 patients. Patients were treated with D-penicillamine, trientine, zinc salts, or their combinations. Besides the main treatments, 41 patients were on symptomatic treatment for neurologic symptoms. Thirty-six patients had a "good outcome," five patients were stable, and six patients had "poor outcome." Post-chelation neurological worsening was observed in 11 patients. WD should be considered in differential diagnosis in any patient with unexplained neurologic symptoms. Early diagnosis is important, and appropriate treatment should be promptly initiated to prevent progressive and irreversible damage, with good prognosis and stable disease in the majority of the patients with treatment compliance.

关键词： HMGB1   IL4   NF-kappa B   NLRP3 inflammasome   sodium houttuyfonate   traumatic brain injury  

摘要： Sodium houttuyfonate (SH) is a chemical compound synthesized by houttuynin and sodium bisulfite. As it has antinflammatory effects, SH has been widely used to treat autoimmune diseases, including post events following traumatic brain injury (TBI). Meanwhile, NOD-like receptor with pyrin domain containing-3 (NLRP3) inflammasomes in microglia may play a central role in TBI. But to date, the intracellular mechanisms involved in the anti-inflammatory effects of SH in TBI remain unknown, especially whether regulating NLRP3. To gain an insight into this possibility, we conducted cell culture and biochemical studies on the effect of SH on NLRP3 inflammasome in microglia. The results showed that SH inhibited TLR4 and NLRP3 inflammasome activation in the microglia cell. In parallel, phosphorylation of ERK and NF-kappa B p65, which play a key role in NLRP3 inflammasome formation, was decreased. Intraperitoneal injection of SH into TBI mice significantly reduced the modified neurological severity score (mNSS), as well as the degree of microglia apoptosis post-controlled cortical impact (CCI). Immunohistochemistry, Western blot analysis, and reverse-transcription polymerase chain reaction (RT-PCR) revealed that SH markedly reduced NLRP3 inflammasome activation, TLR4 activity, phosphorylation of ERK and NE-kappa B. Moreover, SH significantly inhibited microglia activation post-CCI, but effectively promoted the astrocyte activation and angiopoiesis. Taken together, our research provides evidence that SH attenuated neurological deficits post TBI through inhibiting NLRP3 inflammasome activation, via influencing the TLR4/NF-kappa B signaling pathway. These findings explain the intracellular mechanism of the anti-inflammatory activity caused by SH treatment following TBI.

关键词： epitranscriptomics   m6A   methylation   nutrient   vitamin B-12  

摘要： Introduction Vitamin B-12 deficiency presents various neurological manifestations, such as cognitive dysfunction, mental retardation, or memory impairment. However, the involved molecular mechanisms remain to date unclear. Vitamin B-12 is essential for synthesizing S-adenosyl methionine (SAM), the methyl group donor used for almost all transmethylation reactions. Here, we investigate the m6A methylation of mRNAs and their related gene expression in models of vitamin B-12 deficiency. Methods and Results This study observes two cellular models deficient in vitamin B-12 and hippocampi of mice knock-out for the CD320 receptor. The decrease in SAM levels resulting from vitamin B-12 deficiency is associated with m(6)A reduced levels in mRNAs. This is also potentially mediated by the overexpression of the eraser FTO. We further investigate mRNA methylation of some genes involved in neurological functions targeted by the m6A reader YTH proteins. We notably observe a m6A hypermethylation of Prkca mRNA and a consistently increased expression of PKC alpha, a kinase involved in brain development and neuroplasticity, in the two cellular models. Conclusion Our data show that m6A methylation in mRNA could be one of the contributing mechanisms that underlie the neurological manifestations produced by vitamin B-12 deficiency.