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Prognostic value of time to generalization in 71 Chinese patients with sporadic amyotrophic lateral sclerosis
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作者 Qiong-Hua Sun Yan-Ran Li +3 位作者 Wen-Jie Lan Fei Yang Fang Cui Xu-Sheng Huang 《中华医学杂志:英文版》 SCIE CAS CSCD 2019年第9期1023-1027,共5页
Background:It is important to determine prognostic factors for the outcome of amyotrophic lateral sclerosis (ALS) at an early stage.The time taken for symptoms to spread from spinal or bulbar regions to both (time to ... Background:It is important to determine prognostic factors for the outcome of amyotrophic lateral sclerosis (ALS) at an early stage.The time taken for symptoms to spread from spinal or bulbar regions to both (time to generalization;TTG) is considered a strong predictor of survival;however,this has rarely been studied in Asian populations.The aim of this retrospective study was to evaluate potential factors affecting prognosis in Chinese patients with sporadic ALS,with a focus on the association between TTG and overall survival.Methods:Seventy-one patients with sporadic ALS who were hospitalized at Chinese PLA General Hospital from 2009 to 2016 were followed up until December 2017.Survival analysis was performed using univariate Kaplan-Meier log-rank and multivariate Cox proportional hazards models.The clinical data of the patients were recorded and analyzed.Variables studied were age at symptom onset,sex,site of symptom onset,diagnostic latency,TTG,diagnostic category,ALS Functional Rating Scale-revised score,percent predicted forced vital capacity (FVC%),and disease progression rate (DPR) at diagnosis.Results:The mean age at onset was 54 (SD = 10.2) years,and the median survival time from symptom onset was 41 months (95% confidence interval:34–47).By univariate analysis,factors independently affecting survival were age at symptom onset (Log rank = 15.652,P<0.0001),TTG (Log rank = 14.728,P<0.0001),diagnostic latency (Log rank = 11.997,P = 0.001),and DPR (Log rank = 6.50,P = 0.011).In the Cox multivariate model,TTG had the strongest impact on survival time (hazard ratio = 0.926,P = 0.01).Conclusions:TTG can be used as an effective indicator of prognosis in patients with sporadic ALS. 展开更多
关键词 Amyotrophic LATERAL SCLEROSIS Time to generalisation (TTG) Prognosis Survival
Fungal-contaminated grass and well water and sporadic amyotrophic lateral sclerosis 预览
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作者 Peter William French Russell Ian Ludowyke Gilles J.Guillemin 《中国神经再生研究:英文版》 SCIE CAS CSCD 2019年第9期1490-1493,共4页
Fungi are important infectious disease-causing agents,but are often overlooked as environmental factors in disease.We review several lines of evidence that point to a potential fungal origin of sporadic amyotrophic la... Fungi are important infectious disease-causing agents,but are often overlooked as environmental factors in disease.We review several lines of evidence that point to a potential fungal origin of sporadic amyotrophic lateral sclerosis(ALS),the most common form of motor neurone disease.Approximately 90%cases of ALS are sporadic,and the aetiology of sporadic ALS is still unknown.We have previously postulated that grass or soil-associated fungal infections may be a leading cause of sporadic ALS.Herein we extend this proposal to water-associated fungi.A wide variety of fungi have been reported in drinking water including Acremonium,Alternaria,Aspergillus,Cladosporium,Fusarium,Penicillium and Trichoderma.Some of these are known to produce neurotoxic mycotoxins.Despite this,drinking water is not routinely monitored for fungal contamination.Fungal contamination could explain the close correlation between distribution of well water and cases of sporadic ALS in the United States.We propose several mechanisms by which an opportunistic fungal infection from environmental exposure(to water,soil or plants)can lead to long term neuronal degradation resulting in the hallmarks of ALS.If confirmed,the association between fungal infection and sporadic ALS could lead to novel treatment strategies for this progressive and fatal disease. 展开更多
关键词 amyotrophic lateral SCLEROSIS FUNGI motor neurone disease mycotoxins NEUROTOXINS ALS well water SPORADIC ALS
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Post-Mortem MRI and Histopathology in Neurologic Disease:A Translational Approach
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作者 Laura E.Jonkman Boyd Kenkhuis +1 位作者 Jeroen J.G.Geurts Wilma D.J.van de Berg 《神经科学通报:英文版》 SCIE CAS CSCD 2019年第2期229-243,共15页
In this review, combined post-mortem brain magnetic resonance imaging(MRI) and histology studies are highlighted, illustrating the relevance of translational approaches to de?ne novel MRI signatures of neuropathologic... In this review, combined post-mortem brain magnetic resonance imaging(MRI) and histology studies are highlighted, illustrating the relevance of translational approaches to de?ne novel MRI signatures of neuropathological lesions in neuroin?ammatory and neurodegenerative disorders. Initial studies combining post-mortem MRI and histology have validated various MRI sequences,assessing their sensitivity and speci?city as diagnostic biomarkers in neurologic disease. More recent studies have focused on de?ning new radiological(bio)markers and implementing them in the clinical(research) setting. By combining neurological and neuroanatomical expertise with radiological development and pathological validation,a cycle emerges that allows for the discovery of novel MRI biomarkers to be implemented in vivo. Examples of this cycle are presented for multiple sclerosis, Alzheimer’s disease, Parkinson’s disease, and traumatic brain injury.Some applications have been shown to be successful, while others require further validation. In conclusion, there is much to explore with post-mortem MRI and histology studies, which can eventually be of high relevance for clinical practice. 展开更多
关键词 MRI Histology Biomarkers Multiple SCLEROSIS Alzheimer’s DISEASE Parkinson’s DISEASE
Transcriptional dysregulation in neurodegenerative diseases:who tipped the balance of Yin Yang 1 in the brain? 预览
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作者 Zhefan Stephen Chen Ho Yin Edwin Chan 《中国神经再生研究:英文版》 SCIE CAS CSCD 2019年第7期1148-1151,共4页
Yin Yang 1(YY1)is a multi-functional transcription factor that regulates gene expression in a range of cell types,including neurons.It controls neuronal differentiation,as well as neuronal specification and migration ... Yin Yang 1(YY1)is a multi-functional transcription factor that regulates gene expression in a range of cell types,including neurons.It controls neuronal differentiation,as well as neuronal specification and migration during the development of the mammalian nervous system.Besides,YY1 also mediates the transcription of genes that are required for neuronal survival.An impairment of the transcriptional function of YY1 causes neuronal death.This review summarizes recent research findings that unveil the dysfunction of YY1 in multiple neurodegenerative disorders.The expression of disease proteins perturbs the function of YY1 via distinct molecular mechanisms,including recruitment to protein aggregates,protein degradation and aberrant nuclear/cytoplasmic shuttling.Understanding the pathogenic roles of YY1 will further broaden our knowledge of the disease mechanisms in distinct neurodegenerative disorders. 展开更多
关键词 Alzheimer’s disease amyotrophic lateral SCLEROSIS neurodegeneration PROTEIN aggregates recruitment PROTEIN degradation SUBCELLULAR localization TRANSCRIPTIONAL regulation YIN Yang 1
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Presence of antibodies against low-density lipoprotein receptor-related protein 4 and impairment of neuromuscular junction in a Chinese cohort of amyotrophic lateral sclerosis
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作者 Lin Lei Xin-Ming Shen +8 位作者 Shu-Yan Wang Yan Lu Suo-Bin Wang Hai Chen Zheng Liu Ya-Sheng Ouyang Jian-Ying Duo Yu-Wei Da Zhi-Guo Chen 《中华医学杂志:英文版》 SCIE CAS CSCD 2019年第12期1487-1489,共3页
Amyotrophic lateral sclerosis (ALS) is a heterogeneous disorder characterized by a loss of upper and lower motor neurons with neither clear pathogenesis nor effective treatment. Thus, potential biomarkers are needed t... Amyotrophic lateral sclerosis (ALS) is a heterogeneous disorder characterized by a loss of upper and lower motor neurons with neither clear pathogenesis nor effective treatment. Thus, potential biomarkers are needed to classify the disease and find new drug targets. Previous studies have shown that auto-antibodies against the low-density lipoprotein receptor-related protein 4 (LRP4), called the anti-LRP4 antibodies, are found in ~23% patients of Greek and Italian ALS cohorts,[1] and in 10% of the American ALS population.[2] Anti-LRP4 antibodies were previously identified in myasthenia gravis (MG), the most common neuromuscular junction (NMJ) disorder, and were shown to cause NMJ abnormality in animal studies.[3] Here, we studied anti-LRP4 antibodies in Chinese patients and investigated the correlation between anti-LRP4 antibodies and abnormal neuromuscular transmission in ALS. 展开更多
关键词 ANTIBODIES AGAINST amyotrophic lateral SCLEROSIS NEUROMUSCULAR junction
Protective effect of hydrogen sulfide on oxidative stress-induced neurodegenerative diseases 预览
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作者 Rubaiya Tabassum Na Young Jeong Junyang Jung 《中国神经再生研究:英文版》 SCIE CAS CSCD 2020年第2期232-241,共10页
Hydrogen sulfide is an antioxidant molecule that has a wide range of biological effects against oxidative stress. Balanced oxidative stress is also vital for maintaining cellular function in biological system, where r... Hydrogen sulfide is an antioxidant molecule that has a wide range of biological effects against oxidative stress. Balanced oxidative stress is also vital for maintaining cellular function in biological system, where reactive oxygen species are the main source of oxidative stress. When the normal redox balance is disturbed, deoxyribonucleic acid, lipid, and protein molecules are oxidized under pathological conditions, like diabetes mellitus that leads to diabetic peripheral neuropathy. In diabetes mellitus-induced diabetic peripheral neuropathy, due to hyperglycemia, pancreatic beta cell(β cell) shows resistance to insulin secretion. As a consequence, glucose metabolism is disturbed in neuronal cells which are distracted from providing proper cell signaling pathway. Not only diabetic peripheral neuropathy but also other central damages occur in brain neuropathy. Neurological studies regarding type 1 diabetes mellitus patients with Alzheimer’s disease, Parkinson’s disease, and amyotrophic lateral sclerosis have shown changes in the central nervous system because high blood glucose levels(HbA1 c) appeared with poor cognitive function. Oxidative stress plays a role in inhibiting insulin signaling that is necessary for brain function. Hydrogen sulfide exhibits antioxidant effects against oxidative stress, where cystathionine β synthase, cystathionine γ lyase, and 3-mercaptopyruvate sulfurtransferase are the endogenous sources of hydrogen sulfide. This review is to explore the pathogenesis of diabetes mellitus-induced diabetic peripheral neuropathy and other neurological comorbid disorders under the oxidative stress condition and the anti-oxidative effects of hydrogen sulfide. 展开更多
关键词 Alzheimer's DISEASE amyotrophic lateral SCLEROSIS antioxidant diabetic peripheral NEUROPATHY DNA oxidation hydrogen SULFIDE mitochondrial dysfunction oxidative stress Parkinson's DISEASE reactive oxygen species
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Genetic targeting of astrocytes to combat neurodegenerative disease 预览
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作者 Rachel Kéry Allen P. F. Chen Gregory W. Kirschen 《中国神经再生研究:英文版》 SCIE CAS CSCD 2020年第2期199-211,共13页
Astrocytes, glial cells that interact extensively with neurons and other support cells throughout the central nervous system, have recently come under the spotlight for their potential contribution to, or potential re... Astrocytes, glial cells that interact extensively with neurons and other support cells throughout the central nervous system, have recently come under the spotlight for their potential contribution to, or potential regenerative role in a host of neurodegenerative disorders. It is becoming increasingly clear that astrocytes, in concert with microglial cells, activate intrinsic immunological pathways in the setting of neurodegenerative injury, although the direct and indirect consequences of such activation are still largely unknown. We review the current literature on the astrocyte’s role in several neurodegenerative diseases, as well as highlighting recent advances in genetic manipulation of astrocytes that may prove critical to modulating their response to neurological injury, potentially combatting neurodegenerative damage. 展开更多
关键词 Alzheimer's DISEASE amyotrophic lateral SCLEROSIS GLIA immune system inflammation Parkinson's DISEASE reactive ASTROCYTE regeneration
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Study on variation trend of repetitive nerve stimulation waveform in amyotrophic lateral sclerosis
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作者 Li-Lan Fu He-Xiang Yin +1 位作者 Ming-Sheng Liu Li-Ying Cui 《中华医学杂志:英文版》 SCIE CAS CSCD 2019年第5期542-550,共9页
Background: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease involving both upper and lower neurons with no effective cure. Electrophysiological studies have found decremental responses d... Background: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease involving both upper and lower neurons with no effective cure. Electrophysiological studies have found decremental responses during low-frequency repetitive nerve stimulation (RNS) except for diffused neurogenic activities. However, the difference between ALS and generalized myasthenia gravis (GMG) in terms of waveform features is unclear. In the current study, we explored the variation trend of the amplitudes curve between ALS and GMG with low-frequency, positive RNS, and the possible mechanism is discussed preliminarily. Methods: A total of 85 ALS patients and 41 GMG patients were recruited. All patients were from Peking Union Medical College Hospital (PUMCH) between July 1,2012 and February 28,2015. RNS study included ulnar nerve, accessory nerve and facial nerve at 3 Hz and 5 Hz stimulation. The percentage reduction in the amplitude of the fourth or fifth wave from the first wave was calculated and compared with the normal values of our hospital. A 15% decrease in amplitude is defined as a decrease in amplitude. Results: The decremental response at low-frequency RNS showed the abnormal rate of RNS decline was 54.1%(46/85) in the ALS group, and the results of different nerves were 54.1 %(46/85) of the accessory nerve, 8.2%(7/85) of the ulnar nerve and 0%(0/85) of the facial nerve stimulation, respectively. In the GMG group, the abnormal rate of RNS decline was 100%(41/41) at low-frequency RNS of accessory nerves. However, there was a significant difference between the 2 groups in the amplitude after the sixth wave. Conclusions: Both groups of patients are able to show a decreasing amplitude of low-frequency stimulation RNS, but the recovery trend after the sixth wave has significant variation. It implies the different pathogenesis of NMJ dysfunction of these 2 diseases. 展开更多
关键词 Amyotrophic lateral SCLEROSIS Generalized MYASTHENIA GRAVIS NEUROMUSCULAR junction REPETITIVE nerve stimulation
MicroRNAs in blood and cerebrospinal fluid as diagnostic biomarkers of multiple sclerosis and to monitor disease progression 预览
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作者 Bridget Martinez Philip V.Peplow 《中国神经再生研究:英文版》 SCIE CAS CSCD 2020年第4期606-619,共14页
Multiple sclerosis is a chronic autoimmune disease of the central nervous system.It is the main cause of non-traumatic neurological disability in young adults.Multiple sclerosis mostly affects people aged 20–50 years... Multiple sclerosis is a chronic autoimmune disease of the central nervous system.It is the main cause of non-traumatic neurological disability in young adults.Multiple sclerosis mostly affects people aged 20–50 years;however,it can occur in young children and much older adults.Factors identified in the distribution of MS include age,gender,genetics,environment,and ethnic background.Multiple sclerosis is usually associated with progressive degrees of disability.The disease involves demyelination of axons of the central nervous system and causes brain and spinal cord neuronal loss and atrophy.Diagnosing multiple sclerosis is based on a patient’s medical history including symptoms,physical examination,and various tests such as magnetic resonance imaging,cerebrospinal fluid and blood tests,and electrophysiology.The disease course of multiple sclerosis is not well correlated with the biomarkers presently used in clinical practice.Blood-derived biomarkers that can detect and distinguish the different phenotypes in multiple sclerosis may be advantageous in personalized treatment with disease-modifying drugs and to predict response to treatment.The studies reviewed have shown that the expression levels of a large number of miRNAs in peripheral blood,serum,exosomes isolated from serum,and cerebrospinal fluid are altered in multiple sclerosis and can distinguish the disease phenotypes from each other.Further studies are warranted to independently validate these findings so that individual or pairs of miRNAs in serum or cerebrospinal fluid can be used as potential diagnostic markers for adult and pediatric multiple sclerosis and for monitoring disease progression and response to therapy. 展开更多
关键词 clinically isolated syndrome CSF disease progression EXOSOMES humans microRNA multiple sclerosis peripheral blood PHENOTYPES serum
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Unfolded protein response in myelin disorders 预览
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作者 Wensheng Lin Sarrabeth Stone 《中国神经再生研究:英文版》 SCIE CAS CSCD 2020年第4期636-645,共10页
Activation of the unfolded protein response in response to endoplasmic reticulum stress preserves cell viability and function under stressful conditions.Nevertheless,persistent,unresolvable activation of the unfolded ... Activation of the unfolded protein response in response to endoplasmic reticulum stress preserves cell viability and function under stressful conditions.Nevertheless,persistent,unresolvable activation of the unfolded protein response can trigger apoptosis to eliminate stressed cells.Recent studies show that the unfolded protein response plays an important role in the pathogenesis of various disorders of myelin,including multiples sclerosis,Charcot-Marie-Tooth disease,Pelizaeus-Merzbacher disease,vanishing white matter disease,spinal cord injury,tuberous sclerosis complex,and hypoxia-induced perinatal white matter injury.In this review we summarize the current literature on the unfolded protein response and the evidence for its role in the pathogenesis of myelin disorders. 展开更多
关键词 AXON ER multiples sclerosis MYELIN OLIGODENDROCYTE Schwann cell spinal cord injury UPR
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Relationship between MRI perfusion and clinical severity in multiple sclerosis 预览
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作者 Maria Marcella Laganà Laura Pelizzari Francesca Baglio 《中国神经再生研究:英文版》 SCIE CAS CSCD 2020年第4期646-652,共7页
Perfusion alterations within several brain regions have been shown in multiple sclerosis patients using different magnetic resonance imaging(MRI)techniques.Furthermore,MRI-derived brain perfusion metrics have been inv... Perfusion alterations within several brain regions have been shown in multiple sclerosis patients using different magnetic resonance imaging(MRI)techniques.Furthermore,MRI-derived brain perfusion metrics have been investigated in association with multiple sclerosis phenotypes,physical disability,and cognitive impairment.However,a review focused on these aspects is still missing.Our aim was to review all the studies investigating the relationship between perfusion MRI and clinical severity during the last fifteen years to understand the clinical relevance of these findings.Perfusion differences among phenotypes were observed both with 1.5T and 3T scanners,with progressive multiple sclerosis presenting with lower perfusion values than relapsing-remitting multiple sclerosis patients.However,only 3T scanners showed a statistically significant distinction.Controversial results about the association between MRI-derived perfusion metrics and physical disability scores were found.However,the majority of the studies showed that lower brain perfusion and longer transit time are associated with more severe physical disability and worse cognitive performances. 展开更多
关键词 brain perfusion cerebral blood flow COGNITION DISABILITY magnetic resonance imaging MRI multiple sclerosis PHENOTYPES progressive relapsing remitting
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Characteristics and advantages of adenoassociated virus vector-mediated gene therapy for neurodegenerative diseases 预览
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作者 Yuan Qu Yi Liu +2 位作者 Ahmed Fayyaz Noor Johnathan Tran Rui Li 《中国神经再生研究:英文版》 SCIE CAS CSCD 2019年第6期931-938,共8页
Common neurodegenerative diseases of the central nervous system are characterized by progressive damage to the function of neurons,even leading to the permanent loss of function.Gene therapy via gene replacement or ge... Common neurodegenerative diseases of the central nervous system are characterized by progressive damage to the function of neurons,even leading to the permanent loss of function.Gene therapy via gene replacement or gene correction provides the potential for transformative therapies to delay or possibly stop further progression of the neurodegenerative disease in affected patients.Adeno-associated virus has been the vector of choice in recent clinical trials of therapies for neurodegenerative diseases due to its safety and efficiency in mediating gene transfer to the central nervous system.This review aims to discuss and summarize the progress and clinical applications of adeno-associated virus in neurodegenerative disease in central nervous system.Results from some clinical trials and successful cases of central neurodegenerative diseases deserve further study and exploration. 展开更多
关键词 nerve REGENERATION central nervous system gene therapy NEURODEGENERATIVE DISEASE viral vector ADENO-ASSOCIATED virus Alzheimer’s DISEASE Parkinson’s DISEASE Huntington’s DISEASE amyotrophic lateral SCLEROSIS spinal muscular atrophy neural REGENERATION
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Lessons from glaucoma:rethinking the fluid-brain barriers in common neurodegenerative disorders 预览
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作者 Francisco Javier Carreras 《中国神经再生研究:英文版》 SCIE CAS CSCD 2019年第6期962-966,共5页
Glaucoma has been recently characterized as a member of the group of anoikis-related diseases.Anoikis,a form of apoptosis,can be triggered by the unfastening of adherent junctions present in astrocytes.In those areas ... Glaucoma has been recently characterized as a member of the group of anoikis-related diseases.Anoikis,a form of apoptosis,can be triggered by the unfastening of adherent junctions present in astrocytes.In those areas of the central nervous system in which the soma of the neurons or their axons and dendrites are metabolically dependent on the activity of astrocytes,a derangement of the lactate shuttle caused by a separation between the plasma membranes of neurons and astrocytes would result in metabolic impairment of the neurons themselves.In glaucoma,the triggering event has been attributed to the posterior deviation of aqueous humor towards the astrocyte-rich prelaminar tissue of the optic nerve head.The mean calcium content in the aqueous is able to interfere with calcium-dependent adherent junctions and induce anoikis of the astrocytes.As the cerebrospinal fluid has a similar base calcium concentration,a shunt of cerebrospinal fluid through the cerebral parenchyma would be able to interfere in the astrocytic architecture with dire consequences to the metabolically dependent neurons.Here the similitude between glaucoma,amyotrophic lateral sclerosis and Alzheimer’s disease are discussed and the concept of the break in the fluid-brain barrier,as an event separated from the blood-brain barrier,is stressed. 展开更多
关键词 fluid-brain barriers blood-brain barrier CEREBROSPINAL FLUID aqueous humor calcium ion GLAUCOMA amyotrophic lateral SCLEROSIS Alzheimer’s disease
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Malignancy of renal angiomyolipoma from tuberous sclerosis complex with TSC2 mutation
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作者 Yu-Jing Huang Zong-Pei Jiang +4 位作者 Yu-Ping Chen Jin-Quan Wu Jia-Li Huang Yan-Qiang Chen Meng-Jun Liang 《中华医学杂志:英文版》 SCIE CAS CSCD 2019年第1期103-105,共3页
To the Editor:Tuberous sclerosis complex (TSC),with the birth incidence of 1:6000,[1] is an autosomal dominant inherited,multi-system disorder characterized by cellular hyperplasia and tissue dysplasia,among which,ren... To the Editor:Tuberous sclerosis complex (TSC),with the birth incidence of 1:6000,[1] is an autosomal dominant inherited,multi-system disorder characterized by cellular hyperplasia and tissue dysplasia,among which,renal angiomyolipoma (AML) is one common comorbidity.However,malignancy of renal AML is rare.Herein,we shared a case of malignancy of renal AML from TSC in a young man. 展开更多
关键词 MALIGNANCY RENAL ANGIOMYOLIPOMA tuberous SCLEROSIS COMPLEX TSC2 MUTATION
Primary lung mucosa-associated lymphoid tissue lymphoma accompanied by multiple sclerosis:case report and molecular diagnosis
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作者 Ke-Ke Yu Lei Zhu +2 位作者 Ji-Kai Zhao Rui-Ying Zhao Yu-Chen Han 《中华医学杂志:英文版》 SCIE CAS CSCD 2019年第13期1625-1628,共4页
To the Editor:Mucosa-associated lymphoid tissue(MALT)lymphoma arises at a wide range of different extranodal sides,including the stomach(70%),lung(14%),ocular adnexa(12%),thyroid(4%),and small intestine.[1,2]Most lung... To the Editor:Mucosa-associated lymphoid tissue(MALT)lymphoma arises at a wide range of different extranodal sides,including the stomach(70%),lung(14%),ocular adnexa(12%),thyroid(4%),and small intestine.[1,2]Most lung MALT lymphoma are marginal zone B-cell lymphoma with proliferation of small irregular monocytoid B cells around and between reactive lymphoid follicles,with extensive invasion of the lung parenchyma,forming lymphoepithelial lesions.Immunohistochemical analysis showed the positive staining for CD20 and Bcl-2 and negative staining for CD3,CD5,CD23,and CD10 in the neoplastic cells. 展开更多
关键词 PRIMARY LUNG multiple SCLEROSIS IMMUNOHISTOCHEMICAL
The next-generation sphingosine-1 receptor modulator BAF312(siponimod) improves cortical network functionality in focal autoimmune encephalomyelitis 预览
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作者 Petra Hundehege Manuela Cerina +13 位作者 Susann Eichler Christian Thomas Alexander M. Herrmann Kerstin Goel Thomas Müntefering Juncal Fernandez-Orth Stefanie Bock Venu Narayanan Thomas Budde Erwin-Josef Speckmann Heinz Wiendl Anna Schubart Tobias Ruck Sven G. Meuth 《中国神经再生研究:英文版》 SCIE CAS CSCD 2019年第11期1950-1960,共11页
Autoimmune diseases of the central nervous system(CNS) like multiple sclerosis(MS) are characterized by inflammation and demyelinated lesions in white and grey matter regions. While inflammation is present at all stag... Autoimmune diseases of the central nervous system(CNS) like multiple sclerosis(MS) are characterized by inflammation and demyelinated lesions in white and grey matter regions. While inflammation is present at all stages of MS, it is more pronounced in the relapsing forms of the disease, whereas progressive MS(PMS) shows significant neuroaxonal damage and grey and white matter atrophy. Hence, disease-modifying treatments beneficial in patients with relapsing MS have limited success in PMS. BAF312(siponimod) is a novel sphingosine-1-phosphate receptor modulator shown to delay progression in PMS. Besides reducing inflammation by sequestering lymphocytes in lymphoid tissues, BAF312 crosses the blood-brain barrier and binds its receptors on neurons, astrocytes and oligodendrocytes. To evaluate potential direct neuroprotective effects, BAF312 was systemically or locally administered in the CNS of experimental autoimmune encephalomyelitis mice with distinct grey-and white-matter lesions(focal experimental autoimmune encephalomyelitis using an osmotic mini-pump). Ex-vivo flow cytometry revealed that systemic but not local BAF312 administration lowered immune cell infiltration in animals with both grey and white matter lesions. Ex-vivo voltage-sensitive dye imaging of acute brain slices revealed an altered spatio-temporal pattern of activation in the lesioned cortex compared to controls in response to electrical stimulation of incoming white-matter fiber tracts. Here, BAF312 administration showed partial restore of cortical neuronal circuit function. The data suggest that BAF312 exerts a neuroprotective effect after crossing the blood-brain barrier independently of peripheral effects on immune cells. Experiments were carried out in accordance with German and EU animal protection law and approved by local authorities(Landesamt für Natur, Umwelt und Verbraucherschutz Nordrhein-Westfalen;87-51.04.2010.A331) on December 28, 2010. 展开更多
关键词 multiple SCLEROSIS FOCAL experimental autoimmune ENCEPHALOMYELITIS CORTICAL grey MATTER white MATTER BAF312 neuroaxonal damage neuroprotection
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系统性硬化症误诊为消化系统疾病10例分析 预览
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作者 赖姝婕 陈东风 +1 位作者 崔红利 匡怡 《临床误诊误治》 2019年第3期4-8,共5页
目的探讨系统性硬化症(systemic sclerosis,SSc)的发病机制及临床特点,分析误诊原因及防治措施。方法回顾性分析误诊为消化系统疾病的10例SSc的临床资料。结果本组均有不同程度的消化道症状,均首诊误诊,误诊时间2周~5年,误诊为反流性食... 目的探讨系统性硬化症(systemic sclerosis,SSc)的发病机制及临床特点,分析误诊原因及防治措施。方法回顾性分析误诊为消化系统疾病的10例SSc的临床资料。结果本组均有不同程度的消化道症状,均首诊误诊,误诊时间2周~5年,误诊为反流性食管炎3例,慢性胃炎、十二指肠球部溃疡各2例,食管癌、慢性肠炎、药物性肝损伤各1例,均予相应处理,症状稍好转或病情反复发作或未见好转。后进一步完善相关医技检查,结合临床症状,确诊为SSc,予糖皮质激素及对症支持治疗后病情好转出院。随访2年,其中2例失访,1例因严重肺部感染去世,7例病情较稳定。结论SSc早期起病隐匿,常以消化系统症状为首发表现,临床医生对该病认识不足及忽略系统查体是误诊的主要原因。 展开更多
关键词 硬化症 系统性 误诊 食管炎 消化性 胃炎
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Amyotrophic lateral sclerosis: a complex syndrome that needs an integrated research approach 预览
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作者 Javier Riancho Francisco J. Gil-Bea +1 位作者 Ana Santurtun Adolfo López de Munaín 《中国神经再生研究:英文版》 SCIE CAS CSCD 2019年第2期193-196,共4页
Amyotrophic lateral sclerosis, the most common neurodegenerative disease affecting motor neurons, lacks an effective treatment. A small fraction of amyotrophic lateral sclerosis cases have a familial origin, related t... Amyotrophic lateral sclerosis, the most common neurodegenerative disease affecting motor neurons, lacks an effective treatment. A small fraction of amyotrophic lateral sclerosis cases have a familial origin, related to mutations in causative genes, while the vast majority of amyotrophic lateral sclerosis cases are considered to be sporadic, resulting from the interaction between genes and environmental factors in predisposed individuals. During the past few years, dozens of drugs have been postulated as promising strategies for the disease after showing some beneficial effects in preclinical cellular and murine models. However, the translation into clinical practice has been largely unsuccessful and the compounds failed when were tested in clinical trials. This might be explained, at least partially, by the enormous complexity of the disease both from clinico-epidemiological and a pathogenic points of view. In this review, we will briefly comment on the complexity of the disease focusing on some recent findings, and we will suggest how amyotrophic lateral sclerosis research might be reoriented to foster the advance in the diagnostic and therapeutic questions. 展开更多
关键词 amyotrophic LATERAL SCLEROSIS ALS environment EPIDEMIOLOGY GENES PHENOTYPE therapy
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Galectin-3 prospects as a therapeutic agent for multiple sclerosis 预览
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作者 Laura Thomas Laura Andrea Pasquini 《中国神经再生研究:英文版》 SCIE CAS CSCD 2019年第8期1380-1382,共3页
Galectin-3 (Gal-3) in oligodendrocyte (OLG) differentiation: OLGs are the cells in charge of myelination in the central nervous system (CNS), allowing rapid conduction of the neural action potential and giving trophic... Galectin-3 (Gal-3) in oligodendrocyte (OLG) differentiation: OLGs are the cells in charge of myelination in the central nervous system (CNS), allowing rapid conduction of the neural action potential and giving trophic support to axons. OLGs undergo a series of changes throughout their life cycle: first, upon neural stem cell comm让ment to the OLG lineage, cells referred to as OLG precursor cells (OPC) present a bipolar morphology, have proliferative and migratory capacity and express molecular markers like platelet-derived growth factor receptor alpha and neural/glial antigen 2. 展开更多
关键词 MULTIPLE SCLEROSIS THERAPEUTIC AGENT GALECTIN-3 PROSPECTS
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The role of Epstein-Barr virus in multiple sclerosis: from molecular pathophysiology to in vivo imaging 预览
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作者 Yi Guan Dejan Jakimovski +2 位作者 Murali Ramanathan Bianca Weinstock-Guttman Robert Zivadinov 《中国神经再生研究:英文版》 SCIE CAS CSCD 2019年第3期373-386,共14页
Multiple sclerosis (MS)is a disease of the central nervous system characterized by inflammation,demyelination,and neuronal damage.Environmental and genetic factors are associated with the risk of developing MS,but the... Multiple sclerosis (MS)is a disease of the central nervous system characterized by inflammation,demyelination,and neuronal damage.Environmental and genetic factors are associated with the risk of developing MS,but the exact cause still remains unidentified.Epstein-Barr virus (EBV),vitamin D,and smoking are among the most well-established environmental risk factors in MS.Infectious mononucleosis,which is caused by delayed primary EBV infection,increases the risk of developing MS.EBV may also contribute to MS pathogenesis indirectly by activating silent human endogenous retrovirus-W.The emerging B-cell depleting therapies,particularly anti-CD20 agents such as rituximab,ocrelizumab,as well as the fully human ofatumumab,have shown promising clinical and magnetic resonance imaging benefit.One potential effect of these therapies is the depletion of memory B-cells,the primary reservoir site where EBV latency occurs. In addition,EBV potentially interacts with both genetic and other environmental factors to increase susceptibility and disease severity of MS.This review examines the role of EBV in MS pathophysiology and summarizes the recent clinical and radiological findings,with a focus on B-cells and in vivo imaging.Addressing the potential link between EBV and MS allows the better understanding of MS pathogenesis and helps to identify additional disease biomarkers that may be responsive to B-cell depleting intervention. 展开更多
关键词 EPSTEIN-BARR virus multiple SCLEROSIS MENINGEAL inflammation magnetic resonance imaging LEPTOMENINGEAL contrast enhancement MONONUCLEOSIS human endogeneous retrovirus-W B-CELLS
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