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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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research progress on the electrophysiological indicators to predict the efficacy of vagus nerve stimulation for drug-refractory epilepsy

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Acta Epileptologica 2024年第1期7卷 14-20页

作者： Guangli Li Zhenguang Li Yingting Liu Hunan University of Chinese Medicine Changsha410208Hunan ProvinceChina epilepsy center of Brain Hospital of Hunan Province Changsha410007Hunan ProvinceChina

Vagus nerve stimulation(VNS)is an important treatment option for drug-refractory epilepsy(DRE),with well-established efficacy and safety in clinical practice for more than 20 ***,it is very difficult to find the optimal electrophysiological indicators for the effectiveness of VNS on DRE because the mechanism of action is *** this review,we provide an update of the potential applications of VNS outcomes in patients with drug-resistant ***(EEG)activity,event-related potentials,EEG synchronization levels,magnetoencephalographic,laryngeal muscle evoked potentials,and heart rate variability are potential biomarkers for VNS outcomes in people with DRE.

关键词： Electrophysiology Vagus nerve stimulation Drug-refractory epilepsy Efficacy Predictive indicators Electroencephalogram Event-related potential Magnetoencephalography Aryngeal muscle evoked potential Heart rate variability

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Ketogenic diet therapy in children with epilepsy caused by SLC2A1 mutations:a single-center single-arm retrospective study

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World Journal of Pediatrics 2024年第5期20卷 517-524页

作者： Ying-Yan Wang Yun-Qing Zhou Li-Juan Luo Cui-Jin Wang Nan Shen Hao Li Ji-Wen Wang Department of Neurology Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina Department of Infectious Diseases Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina Clinical research Ward Clinical Research CenterShanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina

Background This retrospective study assessed the efficacy and safety of ketogenic diet therapies in children with epilepsy caused by SLC2A1 genetic mutations and glucose transporter type 1 deficiency *** Pediatric patients with epilepsy symptoms admitted to our medical center between January 2017 and October 2021 were included if they presented with an SLC2A1 genetic mutation on whole-exome *** analyzed the patients’convulsions and treatment with antiepileptic *** patients were followed up at different time periods after ketogenic diet *** Six patients with SLC2A1 mutations were included in this *** patients had seizures of different types and frequencies,and they took antiepileptic drugs to relieve their *** were then treated with a ketogenic diet for at least four *** analyzed epilepsy control rates at 1,2,3,6,and 12 months after ketogenic diet *** patients were seizure-free within a month of receiving the diet *** patients were followed up for six months,three were followed up for 12 months after the treatment,and there was no recurrence of epilepsy during this *** antiepileptic drug withdrawal,none of the patients experienced seizure relapse when receiving ketogenic diet treatment *** severe adverse events occurred during the *** Ketogenic diet therapy is very effective and safe for the treatment of epilepsy caused by SLC2A1 ***,patients with glucose transporter type 1 deficiency syndrome caused by SLC2A1 mutations should begin ketogenic diet treatment as soon as possible.

关键词： epilepsy Glucose transporter type 1 Ketogenic diet SLC2A1

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Sirolimus can promote the disappearance of renal angiomyolipoma associated with tuberous sclerosis complex:a prospective cohort study

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World Journal of Pediatrics 2024年第6期20卷 602-610页

作者： Shuo Dun Yang-Yang Wang Lin Wan Qiu-Hong Wang Qian Lu Xiao-Yan Yang Qi Zhang Hui-Min Chen Lu-Peng Qiu Li-Ping Zou Medical School of Chinese PLA Beijing 100853China Department of Pediatrics The First Medical CenterChinese PLA General HospitalBeijing 100853China center of epilepsy Beijing Institute for Brain DisordersBeijing 100069China

Background Renal angiomyolipoma(RAML)is the most common kidney lesion in patients with tuberous sclerosis complex(TSC),affecting about 80%of *** is a benign tumor that grows over time,usually bilaterally,and can easily lead to kidney complications such as acute ***,we investigated the efficacy and safety of sirolimus in children with TSC-associated RAML and explored the factors affecting tumor disappearance under sirolimus treatment through subgroup *** A prospective cohort study was *** was initiated at 1 mg/(m^(2)×day),and dose adjustments were made by a 2-week titration period to attain a trough blood concentration of 5-10 ng/*** disappearance of RAML in chil-dren after sirolimus treatment was observed,and Cox regression was used to screen the factors affecting tumor *** One hundred and twenty-six patients who met the criteria were *** 3 months,6 months,12 months,and 24 months of follow-up,tumors disappeared in 18(14.3%),30(23.8%),39(31.0%),and 42(33.3%)children,*** disappeared in 50(39.7%)children by the last visit of each individual,and 30(60%)of them occurred within 6 *** multivariate Cox regression analysis showed that patients with a smaller maximum tumor diameter at baseline had a higher tumor disappearance ***-six(29%)patients had stomatitis during the entire treatment period,and no serious *** Sirolimus could promote the disappearance of TSC-related *** disappearance rate was correlated with the maximum diameter at baseline,and the smaller the tumor was,the higher the disappearance *** is well tolerated in the treatment of RAML associated with TSC.

关键词： Angiomyolipoma mTOR inhibitor Sirolimus Tuberous sclerosis

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PACS gene family-related neurological diseases: limited genotypes and diverse phenotypes

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World Journal of Pediatrics 2024年第1期20卷 82-91页

作者： Han Zhang Kai Gao Shuang Wang Yue-Hua Zhang Zhi-Xian Yang Ye Wu Yu-Wu Jiang Department of Pediatrics Peking University First Hospital.No.1 Xi'an Men StreetWest DistrictBeijing 100034China Bejing Key Laboratory of Molecular Diagnosis and Study on Pediatric Genetic Diseases BeijingChina Children epilepsy center Peking University First HospitalBeijingChina Key Laboratory for Neuroscience Ministry of Education/National Health and Family Planning CommissionPeking UniversityBeijingChina center of epilepsy Beijing Institute for Brain DisordersBeijingChina

Background The PACS gene family has been demonstrated to be related to intracellular vesicular *** phenotypic manifestations caused by the pathogenic variants of PACS include epilepsy,intellectual disability/developmental delay,and malformations,such as facial *** We identified seven new cases with pathogenic or likely pathogenic PACS variants using next-generation *** information obtained from these patients was analyzed along with that obtained from previously reported *** With the inclusion of the newly diagnosed cases in this study,103 cases with PACS gene family-related neurological diseases were reported,of which 43 were PACS2-related cases and the remaining were PACSI-related *** patients had seizures,which have been reported to be effectively controlled by several types of anti-seizure medications(ASMs).The most efficacious and frequently prescribed ASMs included sodium valproate(43.3%,13/30),oxcarbazepine/carbamazepine(26.7%,8/30),and levetiracetam(20%,6/30).Almost all patients had intellectual disability/developmental *** most common pathogenic missense variants were PACSI ***203Trp and PACS2 *** addition,we report a patient carrying a likely pathogenic copy number variation(CNV)(de novo heterozygous deletion of chr14:105821380-106107443,286 kilobase,destroyed part of the furin-binding region domain and the protein structure after it)with more severe and refractory late-onset *** The clinical phenotypes of the different PACS heterozygous missense variants were *** pathogenic variant sites of PACSI and PACS2 were quite limited but located in different regions.A CNV destroying part of the PACS2 gene might also be *** findings may provide an important clue for further functional studies on the pathogenic mechanism of neurological disorders related to the PACS gene family.

关键词： Developmental delay epilepsy Intellectual disability PACSI PACS2

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Pathological and Physiological High-frequency Oscillations on Electroencephalography in Patients with epilepsy

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Neuroscience Bulletin 2024年第5期40卷 609-620页

作者： Hongyi Ye Cong Chen Shennan A.Weiss Shuang Wang Department of Neurology Epilepsy CenterSecond Affiliated HospitalSchool of MedicineZhejiang UniversityHangzhou310009China Department of Neurology State University of New York DownstateBrooklynNY11203USA Department of Physiology and Pharmacology State University of New York DownstateBrooklynNY11203USA Department of Neurology New York City Health+Hospitals/Kings CountyBrooklynNY11203USA

High-frequency oscillations(HFOs)encompass ripples(80 Hz–200 Hz)and fast ripples(200 Hz–600 Hz),serving as a promising biomarker for localizing the epileptogenic zone in *** fast ripples are always pathological,while ripples may be physiological or *** physiological from pathological ripples is important not only for designating epileptogenic brain regions,but also for investigations that study ripples in the context of memory encoding,consolidation,and recall in patients with *** studies have sought to identify distinguishing features between pathological and physiological ripples over the past two *** and pathological ripples differ with respect to their spatial location,cellular mechanisms,morphology,and coupling with background electroencephalographic *** studies have demonstrated that differentiating between pathological and physiological ripples can improve surgical outcome *** this review,we summarize the characteristics,differences,and applications of pathological and physiological HFOs and discuss strategies for their clinical translation.

关键词： Ripples epilepsy Memory Sleep Surgical outcome

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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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Balloon cells in malformations of corticaldevelopment:friends or foes?

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Acta Epileptologica 2024年第2期7卷 87-99页

作者： Zili Liu Xuefeng Shen Kaomin Lin Fengpeng Wang Jin Gao Yi Yao Jianyuan Sun CAS Key Laboratory of Brain Connectome and Manipulation Shenzhen-Hong Kong Institute of Brain ScienceShenzhen Institute of Advanced Technology(SIAT)Chinese Academy of SciencesShenzhen 518055China The Brain Cognition and Brain Disease Institute SIATCASShenzhen 518055China epilepsy center Fujian Medical University Affiliated Xiamen Humanity HospitalXiamen 361003China Department of Pathology Fujian Medical University Affiliated Xiamen Humanity HospitalXiamen 361003China HH-SIAT Joint center for epilepsy research Fujian Medical University Affiliated Xiamen Humanity HospitalXiamen 361003China

Balloon cells(BCs)are specific pathological marker of cortical malformations during brain development,often associ-ated with epilepsy and development *** a large number of studies have investigated the role of BCsin these diseases,the specific function of BCs as either epileptogenic or antiepileptic remains ***,we reviewed literatures on BCs,delved into the molecular mechanisms and signaling pathways,and updated theirprofile in several ***,BCs are heterogeneous and some of them show progenitor/stem cell ***,BCs are relatively silent in electrophysiology but not completely isolated from their ***,abnormal mTOR signaling and aberrant immunogenic process have been observed within BCs-containingmalformations of cortical development(MCDs).The question whether BCs function as the evildoer or the defenderin BCs-containing MCDs is further ***,this review provides perspectives on future investigationsof the potential role of BCs in epilepsy.

关键词： Balloon cells MCD epilepsy mTOR pathway

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Anti-epileptic and Neuroprotective Effects of Ultra-low Dose NADPH Oxidase Inhibitor Dextromethorphan on Kainic Acid-induced Chronic Temporal Lobe epilepsy in Rats

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Neuroscience Bulletin 2024年第5期40卷 577-593页

作者： Jing-Jing Yang Ying-Xin Liu Yan-Fang Wang Bi-Ying Ge Ying Wang Qing-Shan Wang Sheng Li Jian-Jie Zhang Ling-Ling Jin Jau-Shyong Hong Sheng-Ming Yin Jie Zhao College of Basic Medical Sciences Dalian Medical UniversityDalian116044China Department of Neurology The First Affiliated Hospital of Dalian Medical UniversityDalian116011China National and Local Joint Engineering research center for Drug research and Development of Neurodegenerative Diseases Dalian116044China School of Public Health Dalian Medical UniversityDalian116044China Neurobiology Laboratory National Institute of Environmental Health SciencesNational Institutes of HealthResearch Triangle ParkDurhamNC27709USA

Neuroinflammation mediated by microglia and oxidative stress play pivotal roles in the development of chronic temporal lobe epilepsy(TLE).We postulated that kainic acid(KA)-Induced status epilepticus triggers microglia-dependent inflammation,leading to neuronal damage,a lowered seizure threshold,and the emergence of spontaneous recurrent seizures(SRS).Extensive evidence from our laboratory suggests that dextromethorphan(DM),even in ultra-low doses,has anti-inflammatory and neuroprotective effects in many animal models of neurodegenerative *** results showed that administration of DM(10 ng/kg per day;subcutaneously via osmotic minipump for 4 weeks)significantly mitigated the residual effects of KA,including the frequency of SRS and seizure *** addition,DM-treated rats showed improved cognitive function and reduced hippocampal neuronal *** found suppressed microglial activation-mediated neuroinflammation and decreased expression of hippocampal gp91^(phox) and p47^(phox) proteins in KA-induced chronic TLE ***,even after discontinuation of DM treatment,ultra-low doses of DM continued to confer long-term anti-seizure and neuroprotective effects,which were attributed to the inhibition of microglial NADPH oxidase 2 as revealed by mechanistic studies.

关键词： Temporal lobe epilepsy Dextromethorphan NADPH oxidase Ultra-low dose

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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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