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闪光视诱发电位在克-雅病中的应用:通过单扫描评估波形以及与周期性EEG图形之间的交互作用

世界核心医学期刊文摘（神经病学分册） 2005年第8期1卷 31-31页

作者： Visani E. Agazzi P. Scaioli V. F. Panzica 江山 Department of neurophysiology C. Besta Natl. Neurol. Institute ViaCeloria 11 20133 Milan Italy

Objective: To characterise flash visual evoked potentials(FVEPs) in 20 patient s with Creutzfeldt-Jacob disease (CJD),and assess the relationships between spo ntaneous EEG patterns and the responses to individual stimuli. Methods: We analy sed the shape and time course of periodic sharp wave complexes(PSWCs) and respon ses to 1 Hz flashes. In nine patients, we applied an algorithm based on an autor egressive model with exogenous input (ARX) to estimate responses to individual r andom flashes and their interaction with PSWCs. Results:The FVEPs included P1 an d N1 components in all patients, and the P2 peak in 18. Eight patients showed gi ant FVEPs (N1-P2 > 60 V), all of whom had an MM polymorphism in codon129 of the prion protein gene; in seven cases, the presence of giant FVEPs correlated with a prominent and almost continuous periodic EEG pattern. Giant N1-P2 abnormally spread on the anterior scalp regions, and had a different waveform distribution from that of the PSWCs. In five patients with a normal or slightly enlarged ave rage N1-P2 amplitude, single sweep (ARX) analysis revealed a period of relative refractoriness following individual PSWCs. In four patients with‘giant’FVEPs, the individual responses occurred regardless of the interval between the stimul us and previous PSWC, but their amplitude had an inverse relationship with the i nterval ***: Giant responses to flash stimuli are a common findin g in CJD patients (40%of our cases). Single sweep ARX analysis showed that PSWC s were followed by a period of partial refractoriness, which prevented most of t he individual responses to flashes, but not giant FVEPs. The association between prominent spontaneous paroxysms and giant FVEPs suggests that both are due to a common hyperexcitable change favouring neuronal synchronisation. Significance: Our data contribute to clarifying the debated problem of the occurrence of giant FVEPs in CJD and their relationships with the spontaneous periodic EEG pat

关键词：克-雅病 EEG 视诱发电位交互作用朊蛋白相对不应期复合波前区输入性运算方法

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Lentiviral haematopoietic stem-cell gene therapy for early-onset metachromatic leukodystrophy:long-term results from a non-randomised,open-label,phase 1/2 trial and expanded access

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四川生理科学杂志 2021年第11期43卷 2002-2002页

作者： Francesca Fumagalli San Raffaele Telethon Institute for Gene Therapy (SR-Tiget) IRCCS San Raffaele Scientific Institute Milan Italy Pediatric Immunohematology Unit and BMT Program IRCCS San Raffaele Scientific Institute Milan Italy Units of Neurology and neurophysiology IRCCS San Raffaele Scientific Institute Milan Italy Department of Neurology ASST Papa Giovanni XXIII Bergamo Italy Neuroradiology Unit IRCCS San Raffaele Scientific Institute Milan Italy University Centre of Statistics in the Biomedical Sciences (CUSSB) Milan Italy Vita-Salute San Raffaele University Milan Italy

Background:Effective treatment for metachromatic leukodystrophy(MLD)remains a substantial unmet medical *** this study we investigated the safety and efficacy of atidarsagene autotemcel(arsa-cel)in patients with ***:This study is an integrated analysis of results from a prospective,non-randomised,phase 1/2 clinical study and expanded-access frameworks.29 paediatric patients with pre-symptomatic or early-symptomatic early-onset MLD with biochemical and molecular confirmation of diagnosis were treated with arsa-cel,a gene therapy containing an autologous haematopoietic stem and progenitor cell(HSPC)population transduced ex vivo with a lentiviral vector encoding human arylsulfatase A(ARSA)cDNA.

关键词： dystrophy diagnosis expanded

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Zingiber officinale(Ginger)Methanol Extract Abates Kidney Dysfunction in Mice Co-exposed to Sub-chronic Alcohol Intoxication and Post-traumatic Stress Disorder

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clinical Complementary Medicine and Pharmacology 2023年第4期3卷 79-90页

作者： Olusegun G.Adebayo Benneth Ben-Azu Egwonor Akpofure Modo U.Emmanuel Iheanyichukwu Wopara Wadioni Aduema Lawrence Dayo Adedayo Jude Ijuo Abeje Neurosciences and Oral Physiology Unit Department of PhysiologyFaculty of Basic Medical SciencesCollege of MedicineUniversity of IbadanIbadan 200005Nigeria Department of Pharmacology Faculty of Basic Medical SciencesCollege of Health SciencesDelta State UniversityAbrakaDelta StateNigeria Department of Medicine Evercare HospitalLekkiLagos State 105102Nigeria Department of Biochemistry Faculty of Basic Medical SciencesPAMO University of Medical SciencesPort-HarcourtRiver State 500272Nigeria Department of Biochemistry Faculty of SciencesRiver State UniversityPort HarcourtRiver State 500004Nigeria Department of Physiology Faculty of Basic Medical SciencesBayelsa Medical UniversityYenagoaBayelsa State 178Nigeria neurophysiology Division Physiology ProgrammeCollege of Health ScienceBowen UniversityIwo 232101Nigeria

Background:Increasing number of people globally gives in to indiscriminate consumption of excess alcohol as a coping mechanism to relieve any form of physical or psychological ***,ethnomedicinal use of Zingiber officinale Roscoe(Ginger)have been shown to exhibit broad range of pharmacological benefits but no data has reported the phytotherapeutic treatment of Zingiber officinale methanol extract(MEZO)on alcohol-use disorder(AUD)and post-traumatic stress disorder(PTSD)-induced oxidative and inflammatory stress relevant to disruption of kidney functions in animal ***:To investigate the protective effect of MEZO on kidney-oxidative and inflammatory biomarkers in sub-chronic alcohol exacerbation of PTSD symptoms in ***:Male Swiss mice were administered 30%ethanol for two weeks and thereafter introduced to single pro-longed stress to induce AUD and PTSD respectively prior to post-treatment with MEZO and vitamin *** of oxidative stress,inflammatory cytokines,kidney functions,HPA-axis signaling molecules,vasodilator substance,and histopathology of the kidney were ***:Sub-chronic alcohol intoxication heightened PTSD-induced oxido-inflammatory stress,altered the kidney function indices and HPA-axis,and reduced nitric oxide production,which were ameliorated by the phytother-apeutic treatment with ***,severe degeneration and atrophy of renal tubules were ***,MEZO interventions strongly abated all these ***:Herein,the study shows that phytotherapeutic treatment with MEZO prevents the damaging effects of co-exposure to sub-chronic alcohol intoxication and PTSD.

关键词： Zingiber officinale Kidney Alcohol Single prolonged stress Oxidative stress

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Is Autonomic Nervous System Involved in the Epileptogenesis in Preterm Neonates?

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Maternal-Fetal Medicine 2023年第3期5卷 173-181页

作者： Raffaele Falsaperla Giovanna Vialiti Janette Mailo Giovanni Corsello Martino Ruggieri Neonatal Intensive Care Unit San Marco HospitalUniversity Hospital Policlinico-“G.Rodolico-San Marco”Via Azeglio CiampiCatania 95100Italy Unit of Pediatrics and Pediatric Emergency University Hospital Policlinico-“G.Rodolico-San Marco”Via Azeglio CiampiCatania 95100Italy Division of Pediatric Neurology University of AlbertaStollery Children’s Hospital8440-112 St NWEdmontonAlbertaCanada Department of Maternal and Child Health University of PalermoVia del Vespro 129Palermo 90127Italy Unit of Rare Diseases of the Nervous System in Childhood Department of Clinical and Experimental MedicineSection of Pediatrics and Child NeuropsychiatryUniversity of CataniaCatania 95124Italy

Autonomic nervous system dysfunction has been described with focal and generalized epileptic seizures;occurring during their ictal,interictal,or postictal *** League Against Epilepsy Seizure Classification Manual defines autonomic seizures as a distinct alteration of autonomic nervous system function involving cardiovascular,pupillary,gastrointestinal,sudomotor,vasomotor,and thermoregulatory *** seizures represent a great challenge for neonatologists and neurophysiologists;and distinguishing between ictal and non-ictal autonomic changes in neonates is rarely straightforward,especially in the premature *** avoid overdiagnosis and overtreatment,International League Against Epilepsy and the American clinical neurophysiology Society currently require electrographic correlation for any seizure diagnosis,including preterm *** is very little scientific evidence about the pathophysiology of autonomic *** data reporting on their incidence,clinical features,and diagnostic pathway is also *** this paper,we hypothesize that in the developing brain of preterm neonates,seizures involving deeper autonomic networks and subcortical structures might not propagate sufficiently to the cortex,and therefore the association of the seizures with specific ictal electrographic changes on surface electroencephalogram might be *** propose considering autonomic seizures in the differential diagnosis of unexplained autonomic changes in neonates,especially preterm neonates,even in the absence of clear initial electrographic *** autonomic changes could therefore be thought of as a“seizure alarm”in this population.

关键词： Premature Apnea Autonomic seizures Brain Neonatal epilepsy Neonates

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