Objective:To summarize the clinical features,imaging manifestations,therapeutic options,and prognosis of the primary angiitis of the central nervous system(PACNS)and to explore the role of high-resolution magnetic res...Objective:To summarize the clinical features,imaging manifestations,therapeutic options,and prognosis of the primary angiitis of the central nervous system(PACNS)and to explore the role of high-resolution magnetic resonance imaging(HR-MRI)in the PACNS diagnosis and treatment.Methods:One patient with PACNS treated by HR-MRI was retrospectively analyzed and summarized by combining relevant literature.Results:The patient was a young female who was hospitalized with progressive cerebral infarction and multiple intracranial arterial stenosis.HR-MRI indicated vasculitic changes.After excluding other diseases,hormone shock combined with immunosuppression was given,followed by long-term rehabilitation treatment.The patient’s condition tended to stabilize,and the prognosis was satisfactory.Conclusion PACNS is challenging to diagnose and is characterized by poor prognosis and easy recurrence.HR-MRI plays an important role in the clinical diagnosis and treatment adjustment for PACNS.展开更多
Mitochondria serve as the powerhouse of cells,respond to cellular demands and stressors,and play an essential role in cell signaling,differentiation,and survival.Aberrant mitochondria function has been linked to diver...Mitochondria serve as the powerhouse of cells,respond to cellular demands and stressors,and play an essential role in cell signaling,differentiation,and survival.Aberrant mitochondria function has been linked to diverse and complex human diseases such as neurodegenerative diseases,cancers,myopathies,premature aging,and metabolic syndromes(Nunnari and Suomalainen,2012).展开更多
Neurological disorders are diseases of the central and peripheral nervous systems.These disorders include Alzheimer's disease,epilepsy,brain tumor,and cerebrovascular diseases(stroke,migraine and other headache diso...Neurological disorders are diseases of the central and peripheral nervous systems.These disorders include Alzheimer's disease,epilepsy,brain tumor,and cerebrovascular diseases(stroke,migraine and other headache disorders,multiple sclerosis,Parkinson's disease,and neuroinfections).展开更多
Myelinated axons of the peripheral and central nervous system(PNS&CNS)are divided into molecularly distinct excitable domains,including the axon initial segment(AIS)and nodes of Ranvier.The AIS is composed of a d...Myelinated axons of the peripheral and central nervous system(PNS&CNS)are divided into molecularly distinct excitable domains,including the axon initial segment(AIS)and nodes of Ranvier.The AIS is composed of a dense network of cytoskeletal proteins,cell adhesion molecules,and voltage gated ion channels and is located at the proximal most region of the axon(Koleand Stuart, 2012).展开更多
Clinical use of corticosteroid (CS) therapy and links with adverse neurological effects CS therapy is widely used in clinical practice worldwide, with ad- ministration of high and multiple doses prescribed for a ran...Clinical use of corticosteroid (CS) therapy and links with adverse neurological effects CS therapy is widely used in clinical practice worldwide, with ad- ministration of high and multiple doses prescribed for a range of disease and injury. Notably, CS have been used since the 1950's for the treatment of suspected respiratory distress syndrome, in order to accelerate lung maturation in premature babies, as also in antenatal therapy to pregnant women at risk of preterm birth (Haddad et al., 1956; Shinwell and Eventov-Friedman, 2009; Bonanno and Wapner, 2012). High dose immunosuppressive CS therapy is also widely used in the treatment of multiple sclerosis (MS) and spinal cord injury (Bracken, 2012; Burton et al., 2012). However,展开更多
Scientists conclude that a combination of treatments involving rehabilitation,drug delivery,surgery and cell transplantation are necessary to achieve significant progress in regenerating the injured central nervous sy...Scientists conclude that a combination of treatments involving rehabilitation,drug delivery,surgery and cell transplantation are necessary to achieve significant progress in regenerating the injured central nervous system(CNS).展开更多
Primary angiitis of the central nervous system is a rare and difficult entity. Here we represented the clinical and pathological features of a patient with little response to steroid before definite diagnosis. The 50-...Primary angiitis of the central nervous system is a rare and difficult entity. Here we represented the clinical and pathological features of a patient with little response to steroid before definite diagnosis. The 50-year-old male had a fluctuating disease course for more than 3 years. He presented visual disorders, seizure, cognitive impairment, hypersomnia, unsteady gait, dysphasia, dysphagia, and incontinence. Magnetic resonance imaging showed multiple, supratentorial and infratentorial abnormal signals, while cerebrospinal fluid and cerebral angiography were normal. Magnetic resonance spectrum showed a decrease of N-acetyl-aspartate. Brain biopsy revealed nongranulomatous lymphatic vasculitis with reactive gliosis, cicatrization, demyelination and focal hemorrhages.展开更多
Polyglutamine (polyQ) diseases are a family of neurodegenerative disorders including Huntington’s disease, spinobulbar muscular atrophy,dentatorubral-pallidoluysian atrophy and several spinocerebellar ataxias.polyQ...Polyglutamine (polyQ) diseases are a family of neurodegenerative disorders including Huntington’s disease, spinobulbar muscular atrophy,dentatorubral-pallidoluysian atrophy and several spinocerebellar ataxias.polyQ diseases are caused by abnormal expansion of CAG repeats in certain genes.The expanded CAG repeats are then translated into a series of abnormally expanded polyQ tracts.Such polyQ tracts may induce misfolding of the disease-causing proteins.The present review mainly focuses on the common characteristics of the pathogenesis of these polyQ diseases,including conformational transition of proteins and its influence on the function of these proteins,the correlation between decreased ability of proteoly-sis and late-onset polyQ diseases,and the relationship between wide expression of disease-causing proteins and selective neuronal death.展开更多
The infection of the central nervous system (CNS) by schistosome may or may not have clinical manifestations. When symptomatic, neuroschistosomiasis (NS) is one of the most severe presentations of schistosome infe...The infection of the central nervous system (CNS) by schistosome may or may not have clinical manifestations. When symptomatic, neuroschistosomiasis (NS) is one of the most severe presentations of schistosome infection. Among the NS symptoms, cerebral invasion is mostly caused by Schistosoma japonicum (S. japonicum), and the spinal cord symptoms are mainly caused by S. mansoni or S. haematobium. There are 2 main pathways by which schistosomes cause NS: egg embolism and worm migration, via either artery or vein system, especially the valveless perivertebral Batson's plexus. The adult worm migrates anomalously through the above pathways to the CNS where they lay eggs. Due to the differences in species of schistosomes and stages of infection, mechanisms vary greatly. The portal hypertension with hepatosplenic schistosomiasis also plays an important role in the pathogenesis. Here the pathways through which NS occurs in the CNS were reviewed.展开更多
Objective Cyclic nucleotide phosphodiesterase(PDE)is a critical component of the nitric oxide(NO)signaling pathway and plays critical roles in cognition and learning,Parkinson’s disease,attention deficit hyperact...Objective Cyclic nucleotide phosphodiesterase(PDE)is a critical component of the nitric oxide(NO)signaling pathway and plays critical roles in cognition and learning,Parkinson’s disease,attention deficit hyperactivity disorder, psychosis and depression.The PDEs in the brain of guinea pig have not yet been reported.The present study aimed to detect the unknown Pde cDNAs in the brain of guinea pig.Methods Reverse transcription polymerase chain reaction(RT-PCR)and sequence comparison analysis were performed to detect the expression of Pde cDNAs and to assess the identity rates of cDNA and amino acid sequences between guinea pig and human or mouse,respectvely.The RT-PCR primers were located on the conserved region of human PDE and mouse Pde cDNAs.Results Eleven novel Pde cDNAs were detected in the brain of guinea pig(Cavia porcellus),including CpPde1a,CpPde1b,CpPde2a,CpPde4a,CpPde4d,CpPde5a,CpPde6c,CpPde7b, CpPde8a,CpPde9a,and CpPde10a.The identity rates of the Pde cDNA sequences between guinea pig and human ranged from 83.8%to 94.3%,and those of the amino acid sequences ranged from 91.9%to 100%.The identity rates of Pde cDNA sequences between guinea pig and mouse ranged from 84.6%to 92.1%,and those of amino acid sequences ranged from 91.2% to 99.2%.The average identity rate of the 11 Pde cDNA sequences between guinea pig and human was significantly higher(P 0.01)than that between guinea pig and mouse.The putative partial amino acid sequences of guinea pig contained at least one of the conserved domains of human and mouse PDE proteins.Conclusion These results indicate that the brainexpressed Pde genes are identified in guinea pig,which lays the foundation for further investigating the physiological roles of PDE proteins in the brain.展开更多
基金Basic and Applied Basic Research Fund Project of Guangdong Province(2022A1515220161).
文摘Objective:To summarize the clinical features,imaging manifestations,therapeutic options,and prognosis of the primary angiitis of the central nervous system(PACNS)and to explore the role of high-resolution magnetic resonance imaging(HR-MRI)in the PACNS diagnosis and treatment.Methods:One patient with PACNS treated by HR-MRI was retrospectively analyzed and summarized by combining relevant literature.Results:The patient was a young female who was hospitalized with progressive cerebral infarction and multiple intracranial arterial stenosis.HR-MRI indicated vasculitic changes.After excluding other diseases,hormone shock combined with immunosuppression was given,followed by long-term rehabilitation treatment.The patient’s condition tended to stabilize,and the prognosis was satisfactory.Conclusion PACNS is challenging to diagnose and is characterized by poor prognosis and easy recurrence.HR-MRI plays an important role in the clinical diagnosis and treatment adjustment for PACNS.
基金Supported by an endowment to JES from Cardinal Hill Rehabilitation Hospital
文摘Mitochondria serve as the powerhouse of cells,respond to cellular demands and stressors,and play an essential role in cell signaling,differentiation,and survival.Aberrant mitochondria function has been linked to diverse and complex human diseases such as neurodegenerative diseases,cancers,myopathies,premature aging,and metabolic syndromes(Nunnari and Suomalainen,2012).
文摘Neurological disorders are diseases of the central and peripheral nervous systems.These disorders include Alzheimer's disease,epilepsy,brain tumor,and cerebrovascular diseases(stroke,migraine and other headache disorders,multiple sclerosis,Parkinson's disease,and neuroinfections).
基金supported by National Institutes of Health Grants NS069688 and NS044916, TIRR Foundationthe Dr. Miriam and Sheldon G. Adelson Medical Research Foundation
文摘Myelinated axons of the peripheral and central nervous system(PNS&CNS)are divided into molecularly distinct excitable domains,including the axon initial segment(AIS)and nodes of Ranvier.The AIS is composed of a dense network of cytoskeletal proteins,cell adhesion molecules,and voltage gated ion channels and is located at the proximal most region of the axon(Koleand Stuart, 2012).
基金funded through grants from the British Neuropathological Society,North Staffordshire Medical Institute and University of Nottingham
文摘Clinical use of corticosteroid (CS) therapy and links with adverse neurological effects CS therapy is widely used in clinical practice worldwide, with ad- ministration of high and multiple doses prescribed for a range of disease and injury. Notably, CS have been used since the 1950's for the treatment of suspected respiratory distress syndrome, in order to accelerate lung maturation in premature babies, as also in antenatal therapy to pregnant women at risk of preterm birth (Haddad et al., 1956; Shinwell and Eventov-Friedman, 2009; Bonanno and Wapner, 2012). High dose immunosuppressive CS therapy is also widely used in the treatment of multiple sclerosis (MS) and spinal cord injury (Bracken, 2012; Burton et al., 2012). However,
文摘Scientists conclude that a combination of treatments involving rehabilitation,drug delivery,surgery and cell transplantation are necessary to achieve significant progress in regenerating the injured central nervous system(CNS).
文摘Primary angiitis of the central nervous system is a rare and difficult entity. Here we represented the clinical and pathological features of a patient with little response to steroid before definite diagnosis. The 50-year-old male had a fluctuating disease course for more than 3 years. He presented visual disorders, seizure, cognitive impairment, hypersomnia, unsteady gait, dysphasia, dysphagia, and incontinence. Magnetic resonance imaging showed multiple, supratentorial and infratentorial abnormal signals, while cerebrospinal fluid and cerebral angiography were normal. Magnetic resonance spectrum showed a decrease of N-acetyl-aspartate. Brain biopsy revealed nongranulomatous lymphatic vasculitis with reactive gliosis, cicatrization, demyelination and focal hemorrhages.
基金supported by the grants from the National Natural Science Foundation of China(No.30600197)the Specialized Research Fund for the Doctoral Program of Higher Education of China(No.20050285017)
文摘Polyglutamine (polyQ) diseases are a family of neurodegenerative disorders including Huntington’s disease, spinobulbar muscular atrophy,dentatorubral-pallidoluysian atrophy and several spinocerebellar ataxias.polyQ diseases are caused by abnormal expansion of CAG repeats in certain genes.The expanded CAG repeats are then translated into a series of abnormally expanded polyQ tracts.Such polyQ tracts may induce misfolding of the disease-causing proteins.The present review mainly focuses on the common characteristics of the pathogenesis of these polyQ diseases,including conformational transition of proteins and its influence on the function of these proteins,the correlation between decreased ability of proteoly-sis and late-onset polyQ diseases,and the relationship between wide expression of disease-causing proteins and selective neuronal death.
基金supported by the Science Foundation of the Department of Health, Hubei Province, China (No. XF06D43, XF2008-23)
文摘The infection of the central nervous system (CNS) by schistosome may or may not have clinical manifestations. When symptomatic, neuroschistosomiasis (NS) is one of the most severe presentations of schistosome infection. Among the NS symptoms, cerebral invasion is mostly caused by Schistosoma japonicum (S. japonicum), and the spinal cord symptoms are mainly caused by S. mansoni or S. haematobium. There are 2 main pathways by which schistosomes cause NS: egg embolism and worm migration, via either artery or vein system, especially the valveless perivertebral Batson's plexus. The adult worm migrates anomalously through the above pathways to the CNS where they lay eggs. Due to the differences in species of schistosomes and stages of infection, mechanisms vary greatly. The portal hypertension with hepatosplenic schistosomiasis also plays an important role in the pathogenesis. Here the pathways through which NS occurs in the CNS were reviewed.
基金supported by the National Natural Science Foundation of China(No.31070928,30600198)the Natural Science Foundation of Guangdong Province,China(No.06301101)the Medical Research Program of Guangdong Province,China(No.A2010259)
文摘Objective Cyclic nucleotide phosphodiesterase(PDE)is a critical component of the nitric oxide(NO)signaling pathway and plays critical roles in cognition and learning,Parkinson’s disease,attention deficit hyperactivity disorder, psychosis and depression.The PDEs in the brain of guinea pig have not yet been reported.The present study aimed to detect the unknown Pde cDNAs in the brain of guinea pig.Methods Reverse transcription polymerase chain reaction(RT-PCR)and sequence comparison analysis were performed to detect the expression of Pde cDNAs and to assess the identity rates of cDNA and amino acid sequences between guinea pig and human or mouse,respectvely.The RT-PCR primers were located on the conserved region of human PDE and mouse Pde cDNAs.Results Eleven novel Pde cDNAs were detected in the brain of guinea pig(Cavia porcellus),including CpPde1a,CpPde1b,CpPde2a,CpPde4a,CpPde4d,CpPde5a,CpPde6c,CpPde7b, CpPde8a,CpPde9a,and CpPde10a.The identity rates of the Pde cDNA sequences between guinea pig and human ranged from 83.8%to 94.3%,and those of the amino acid sequences ranged from 91.9%to 100%.The identity rates of Pde cDNA sequences between guinea pig and mouse ranged from 84.6%to 92.1%,and those of amino acid sequences ranged from 91.2% to 99.2%.The average identity rate of the 11 Pde cDNA sequences between guinea pig and human was significantly higher(P 0.01)than that between guinea pig and mouse.The putative partial amino acid sequences of guinea pig contained at least one of the conserved domains of human and mouse PDE proteins.Conclusion These results indicate that the brainexpressed Pde genes are identified in guinea pig,which lays the foundation for further investigating the physiological roles of PDE proteins in the brain.