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Mice with type I interferon signaling deficiency are prone to epilepsy upon HSV-1 infection

Virologica Sinica 2024年第2期39卷 251-263页

作者： Wei Yang Chong-Yang Tang Dong-Ying Fan Yi-Song Wang Pei-Gang Wang Jing An Guo-Ming Luan Beijing Key Laboratory of epilepsy Sanbo Brain HospitalCapital Medical UniversityBeijing100093China Beijing Institute for Brain Disorders Capital Medical UniversityBeijing100093China Department of neurosurgery Epilepsy CenterSanbo Brain HospitalCapital Medical UniversityBeijing100093China Department of Microbiology School of Basic Medical SciencesCapital Medical UniversityBeijing100069China Chinese Institute for Brain Research Beijing102206China

Viral encephalitis continues to be a significant public health *** our previous study,we discovered a lower expression of antiviral factors,such as IFN-β,STING and IFI16,in the brain tissues of patients with Rasmussen's encephalitis(RE),a rare chronic neurological disorder often occurred in children,characterized by unihemispheric brain ***,a higher cumulative viral score of human herpes viruses(HHVs)was also found to have a significant positive correlation with the unihemispheric atrophy in *** I IFNs(IFN-I)signaling is essential for innate anti-infection response by binding to IFN-α/βreceptor(IFNAR).In this study,we infected WT mice and IFNAR-deficient A6 mice with herpes simplex virus 1(HSV-1)via periocular injection to investigate the relationship between IFN-I signaling and HHVs-induced brain *** all mice exhibited typical viral encephalitis lesions in their brains,HSV-induced epilepsy was only observed in A6 *** gene expression matrix,functional enrichment analysis and protein-protein interaction network revealed four gene models that were positively related with HSV-induced ***,ten key genes with the highest scores were *** together,these findings indicate that intact IFN-I signaling can effectively limit HHVs induced neural symptoms and brain lesions,thereby confirming the positive correlation between IFN-I signaling repression and brain atrophy in RE and other HHVs encephalitis.

关键词： IFN-I signaling Human herpes viruses(HHVs) Rasmussen's encephalitis(RE)

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Safety and efficacy of a novel responsive neurostimulation system in China for drug-refractory focal epilepsy:The first-in-man study

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Chinese Medical Journal 2024年第12期137卷 1486-1488页

作者： Kailiang Wang Yongzhi Shan Penghu Wei Liankun Ren Liang Chen Junming Zhu Guoguang Zhao Department of neurosurgery Xuanwu HospitalCapital Medical UniversityBeijing 100053China International Neuroscience Institute(China-INI) Clinical Research Center for EpilepsyCapital Medical UniversityBeijing 100053China China National Medical Center for Neurological Diseases Beijing 100053China Department of Neurology Xuanwu HospitalCapital Medical UniversityBeijing 100053China Department of neurosurgery Huashan HospitalFudan UniversityShanghai 200032China Department of neurosurgery The Second Affiliated Hospital of Zhejiang University School of MedicineHangzhouZhejiang 310009China

To the Editor:Recent studies have characterized drugresistant epilepsy(DRE)as essentially a neural network disease.[1]Detection and disconnection of this pathological epileptic connectome or network can greatly improve seizure *** team also proposes the development of the newly emerging branch of epileptic networks neurosurgery(ENN).Responsive neurostimulation(RNS)is an ENN-oriented emerging treatment option without the resection of the seizure-onset zone or epileptic focus and aims to control the seizure or other epileptic manifestations by modulating or disrupting the network’s key nodes(epileptic hubs)in a self-responsive way.

关键词： epilepsy stimulation drug

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Local field potentials,spiking activity,and receptive fields in human visual cortex

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Science China(Life Sciences) 2024年第3期67卷 543-554页

作者： Lu Luo Xiongfei Wang Junshi Lu Guanpeng Chen Guoming Luan Wu Li Qian Wang Fang Fang School of Psychological and Cognitive Sciences and Beijing Key Laboratory of Behavior and Mental Health Peking UniversityBeijing 100871China School of Psychology Beijing Sport UniversityBeijing 100084China Department of neurosurgery Sanbo Brain HospitalCapital Medical UniversityBeijing 100093China Beijing Key Laboratory of epilepsy Epilepsy CenterSanbo Brain HospitalCapital Medical UniversityBeijing 100093China IDG/McGovern Institute for Brain Research Peking UniversityBeijing 100871China Peking-Tsinghua Center for Life Sciences Peking UniversityBeijing 100871China Beijing Institute for Brain Disorders Beijing 100069China State Key Laboratory of Cognitive Neuroscience and Learning and IDG/McGovern Institute for Brain Research Beijing Normal UniversityBeijing 100875China

The concept of receptive field(RF) is central to sensory neuroscience. Neuronal RF properties have been substantially studied in animals,while those in humans remain nearly unexplored. Here, we measured neuronal RFs with intracranial local field potentials(LFPs) and spiking activity in human visual cortex(V1/V2/V3). We recorded LFPs via macro-contacts and discovered that RF sizes estimated from lowfrequency activity(LFA, 0.5–30 Hz) were larger than those estimated from low-gamma activity(LGA, 30–60 Hz) and high-gamma activity(HGA, 60–150 Hz). We then took a rare opportunity to record LFPs and spiking activity via microwires in V1 simultaneously. We found that RF sizes and temporal profiles measured from LGA and HGA closely matched those from spiking activity. In sum, this study reveals that spiking activity of neurons in human visual cortex could be well approximated by LGA and HGA in RF estimation and temporal profile measurement, implying the pivotal functions of LGA and HGA in early visual information processing.

关键词： human visual cortex receptive field intracranial EEG local field potential spiking activity

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MPDZ variants associated with epilepsies and/or febrile seizures and the individualized genotype-phenotype correlation

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Genes & Diseases 2024年第4期11卷 124-127页

作者： Junxia Luo Yun Li Yong Lv Xin Li Bing Qin Chuanfang Cheng Xiaorong Liu Weiping Liao Jie Wang Zaifen Gao Department of epilepsy Center Children's Hospital Affiliated to Shandong UniversityJinanShandong 250022China Department of epilepsy Center Jinan Children's HospitalJinanShandong 250022China Department of Brain Function and Neuroelectrophysiology The Affiliated Nanhua HospitalHengyang Medical SchoolUniversity of South ChinaHengyangHunan 421001China Department of Pediatrics The First Affiliated Hospital of University of Science and Technology of China(Anhui Provincial Hospital)HefeiAnhui 230001China Department of Pediatrics The Second HospitalCheeloo College of MedicineShandong UniversityJinanShandong 250033China epilepsy Center and Department of neurosurgery The First Affliated Hospital of Jinan UniversityGuangzhouGuangdong 510630China Department of Cardiology The Second Afiliated Hospital of Guangzhou Medical UniversityGuangzhouGuangdong 510260China Institute of Neuroscience and Department of Neurology of the Second Affiliated Hospital of Guangzhou Medical University GuangzhouGuangdong 510260China Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China GuangzhouGuangdong 510260China

The multiple PDZ domain crumbs cell polarity complex component gene(MPDZ;MIM:603785),is highly expressed in the brain across the whole *** encodes the multiple PDZ domain protein,which is a member of the NMDAR signaling complex that may play a role in the control of AMPAR potentiation and synaptic plasticity in excitatory synapses."Previously,MPDZ variants have been demonstrated to be associated with autosomal recessive congenital hydrocephalus-2(HYC2;MIM:615219)which is commonly complicated by brain abnormalities and developmental *** were reported in only one *** association between MPDz and epilepsy requires clarification.

关键词： epilepsy febrile congenital

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Vagus nerve stimulation in cerebral stroke:biological mechanisms,therapeutic modalities,clinical applications,and future directions

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Neural Regeneration Research 2024年第8期19卷 1707-1717页

作者： Li Du Xuan He Xiaoxing Xiong Xu Zhang Zhihong Jian Zhenxing Yang Department of Anesthesiology Renmin Hospital of Wuhan UniversityWuhanHubei ProvinceChina Department of neurosurgery Renmin Hospital of Wuhan UniversityWuhanHubei ProvinceChina

Stroke is a major disorder of the central nervous system that poses a serious threat to human life and quality of *** stro ke victims are left with long-term neurological dysfunction,which adversely affects the well-being of the individual and the broader socioeconomic ***,poststroke brain dysfunction is a major and difficult area of *** nerve stimulation is a Food and Drug Administration-approved exploratory treatment option for autis m,refractory depression,epilepsy,and Alzheimer’s *** is expected to be a novel therapeutic technique for the treatment of stroke owing to its association with multiple mechanisms such as alte ring neurotransmitters and the plasticity of central neuro *** animal models of acute ischemic stroke,vagus nerve stimulation has been shown to reduce infarct size,reduce post-stroke neurological damage,and improve learning and memory capacity in rats with stroke by reducing the inflammatory response,regulating bloodbrain barrier permeability,and promoting angiogenesis and *** present,vagus nerve stimulation includes both invasive and non-invasive vagus nerve *** studies have found that invasive vagus nerve stimulation combined with rehabilitation therapy is effective in im proving upper limb motor and cognitive abilities in stroke *** clinical studies have shown that non-invasive vagus nerve stimulation,including ear/ce rvical vagus nerve stimulation,can stimulate vagal projections to the central nervous system similarly to invasive vagus nerve stimulation and can have the same *** this paper,we first describe the multiple effects of vagus nerve stimulation in stroke,and then discuss in depth its neuroprotective mechanisms in ischemic *** go on to outline the res ults of the current major clinical applications of invasive and non-invasive vagus nerve ***,we provide a more comprehensive evaluation of the advantages and disadvantages of di

关键词： cerebral stroke neuroplasticity non-invasive vagus nerve stimulation rehabilitation vagus nerve stimulation

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Theta Oscillations Support Prefrontal-hippocampal Interactions in Sequential Working Memory

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Neuroscience Bulletin 2024年第2期40卷 147-156页

作者： Minghong Su Kejia Hu Wei Liu Yunhao Wu Tao Wang Chunyan Cao Bomin Sun Shikun Zhan Zheng Ye Institute of Psychology Chinese Academy of SciencesBeijing 100101China Department of Psychology University of Chinese Academy of SciencesBeijing 100049China Department of neurosurgery Center for Functional NeurosurgeryRuijin HospitalShanghai Jiao Tong University School of MedicineShanghai 200025China Institute of Neuroscience Center for Excellence in Brain Science and Intelligence TechnologyChinese Academy of SciencesShanghai 200031China

The prefrontal cortex and hippocampus may support sequential working memory beyond episodic memory and spatial *** stereoelectroencephalography(SEEG)study investigated how the dorsolateral prefrontal cortex(DLPFC)interacts with the hippocampus in the online processing of sequential *** patients with epilepsy(eight women,age 27.6±8.2 years)completed a line ordering task with SEEG recordings over the DLPFC and the *** showed longer thinking times and more recall errors when asked to arrange random lines clockwise(random trials)than to maintain ordered lines(ordered trials)before recalling the orientation of a particular ***,the ordering-related increase in thinking time and recall error was associated with a transient theta power increase in the hippocampus and a sustained theta power increase in the DLPFC(3–10 Hz).In particular,the hippocampal theta power increase correlated with the memory precision of line ***,theta phase coherences between the DLPFC and hippocampus were enhanced for ordering,especially for more precisely memorized ***,the theta band DLPFC→hippocampus influence was selectively enhanced for ordering,especially for more precisely memorized *** study suggests that theta oscillations may support DLPFC-hippocampal interactions in the online processing of sequential information.

关键词： Sequential working memory Hippocampus Dorsolateral prefrontal cortex Theta oscillations Stereoelectroencephalography(SEEG) Phase coherence Granger causality

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Allogeneic mesenchymal stem cells may be a viable treatment modality in cerebral palsy

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World Journal of Clinical Cases 2024年第9期12卷 1585-1596页

作者： Osman Boyalı Serdar Kabatas ErdinçCivelek Omer Ozdemir Yeliz Bahar-Ozdemir Necati Kaplan Eyüp Can Savrunlu Erdal Karaöz Department of neurosurgery University of Health Sciences TurkeyGaziosmanpaşa Training and Research HospitalIstanbul 34360Turkey Center for Stem Cell&Gene Therapy Research and Practice University of Health Sciences TurkeyIstanbul 34360Turkey Department of Physical Medicine and Rehabilitation Health Sciences University Sultan Abdulhamid Han Training and Research HospitalIstanbul 34668Turkey Department of neurosurgery Istanbul Rumeli UniversityÇorlu Reyap HospitalTekirdağ59860Turkey Department of neurosurgery Nevşehir State HospitalNevşehir 50300Turkey Center for Regenerative Medicine and Stem Cell Research&Manufacturing(LivMedCell) Liv HospitalIstanbul 34340Turkey Department of Histology and Embryology Istinye UniversityFaculty of Medicineİstanbul 34010Turkey Center for Stem Cell and Tissue Engineering Research and Practice Istinye UniversityIstanbul 34340Turkey

BACKGROUND Cerebral palsy(CP)describes a group of disorders affecting movement,balance,and *** in motor functions constitute the main body of CP *** symptoms surface in early childhood and patients are affected for the rest of their ***,treatment involves various pharmacotherapies for different types of CP,including antiepileptics for epilepsy and Botox A for focal ***,none of these methods can provide full symptom *** has prompted researchers to look for new treatment modalities,one of which is mesenchymal stem cell therapy(MSCT).Despite being a promising tool and offering a wide array of possibilities,mesenchymal stem cells(MSCs)still need to be investigated for their efficacy and *** To analyze the efficacy and safety of MSCT in CP *** Our sample consists of four CP patients who cannot stand or walk without external *** of these cases received allogeneic MSCT six times as 1×106/kg intrathecally,intravenously,and intramuscularly using umbilical cord-derived MSCs(UC-MSC).We monitored and assessed the patients pre-and post-treatment using the Wee Functional Independence Measure(WeeFIM),Gross Motor Function Classification System(GMFCS),and Manual Ability Classification Scale(MACS)*** utilized the Modified Ashworth Scale(MAS)to measure *** We found significant improvements in MAS scores after the intervention on both *** months:Rightχ^(2)=4000,P=0.046,leftχ^(2)=4000,P=0.046;four months:Rightχ^(2)=4000,P=0.046,leftχ^(2)=4000,P=0.046;12 months:Rightχ^(2)=4000,P=0.046,leftχ^(2)=4000,P=***,there was no significant difference in motor functions based on WeeFIM results(P>0.05).GMFCS and MACS scores differed significantly at 12 months after the intervention(P=0.046,P=0.046).Finally,there was no significant change in cognitive functions(P>0.05).CONCLUSION In light of our findings,we believe that UC-MSC therapy has a positive effect on spasticity

关键词： Cerebral palsy Mesenchymal stem cell Transplantation Wharton’s jelly Muscle spasticity

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Astrocyte chloride,excitatory-inhibitory balance and epilepsy

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Neural Regeneration Research 2024年第9期19卷 1887-1887页

作者： Verena Untiet Maiken Nedergaard Alexei Verkhratsky Division of Glial Disease and Therapeutics Center for Translational NeuromedicineUniversity of CopenhagenCopenhagenDenmark Division of Glial Disease and Therapeutics Center for Translational NeuromedicineDepartment of NeurosurgeryUniversity of Rochester Medical CenterRochesterNYUSA Faculty of Biology Medicine and HealthThe University of ManchesterManchesterUK Achucarro centre for Neuroscience IKERBASQUEBasque Foundation for ScienceBilbaoSpain

Excitation and inhibition are at the core of brain function and *** sustain the activity of neuronal networks over time and space,glutamatergic excitation is balanced by GABAergic *** equipoise of excitation and inhibition,known as the excitation/inhibition(E/I)balance,is crucial for proper brain *** E/I balance is highly dynamic and shifts across different brain states:wakefulness primarily augments excitatory activity,while sleep promotes a decrease in excitation and an increase in inhibition(Bridi et al.,2020).Neuronal activity during various brain states is primarily regulated by neurotransmitters(Schiemann et al.,2015),alongside non-synaptic mechanisms that operate on a slower *** non-synaptic mechanisms are many,with the ionic composition of the extracellular space playing a significant role;altering extracellular ion concentrations affects sleep,arousal,electroencephalogram patterns,and behavioral states(Ding et al.,2016).

关键词： epilepsy excitatory excitation

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Bilateral globus pallidus internus-deep brain stimulation in a 5-year-old boy with SGCE-related myoclonus dystonia syndrome

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Pediatric Investigation 2024年第2期8卷 154-156页

作者： Xiaojuan Tian Changhong Ding Ming Liu Lifang Dai Zixing Xie Tie Fang Department of Neurology Neurological CenterBeijing Children’s HospitalCapital Medical UniversityNational Center For Children’s HealthBeijingChina Department of Functional neurosurgery Neurological CenterBeijing Children’s HospitalCapital Medical UniversityNational Center For Children’s HealthBeijingChina

To the editor:Myoclonus-dystonia syndrome(MDS)(OMIM15990)is a heritable disorder characterized by early onset subcortical myoclonic jerks and/or less prominent *** leads to disability and is often refractory to medical treatment.1 Deep brain stimulation(DBS)for the treatment of refractory MDS has been proven effective in adults for reducing both myoclonic jerks and dystonia.1,2,3 Here,we describe the application of bilateral globus pallidus internus(GPi)DBS in a 5-year-old boy diagnosed with MDS.

关键词： globus editor Deep

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Combined Oxidative Phosphorylation Deficiency-20-Exome as a Diagnostic Implement

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Journal of Biosciences and Medicines 2024年第6期12卷 7-12页

作者： Paulo Roberto Matos-Neto Lucas Antonissen Lima Verde Airton Ferreira da Ponte-Filho Luís Eduardo Oliveira Matos Amandha Espavier Trés Paulo Roberto Lacerda Leal Gerardo Cristino-Filho Regina Coeli de Carvalho Porto Carneiro Faculty of Medicine Federal University of Cear Sobral Cear Brazil Department of neurosurgery Federal University of Cear Sobral Cear Brazil Department of Neuropediatrics Federal University of Cear Sobral Cear Brazil

Mitochondrial disorders are phenotypically varied, with serious clinical repercussions. Among them, there is the deficiency of combined oxidative phosphorylation of type 20, which occurs due to a defect in the VARS2 gene. This article presents a case of a 2-year-old female with progressive myoclonic epilepsy and psychomotor regression, with refractoriness to multiple anticonvulsants. The diagnosis was only made after the examination was carried out. Therefore, this article highlights the aspects of this rare disease and the importance of the exome for the diagnosis of rare conditions.

关键词： Oxidative Phosphorylation epilepsy Exome Mitochondrial Defect VARS2

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