Neural tube defects(NTDs)are severe congenital neurodevelopmental disorders arising from incomplete neural tube closure.Although folate supplementation has been shown to mitigate the incidence of NTDs,some cases,often...Neural tube defects(NTDs)are severe congenital neurodevelopmental disorders arising from incomplete neural tube closure.Although folate supplementation has been shown to mitigate the incidence of NTDs,some cases,often attributable to genetic factors,remain unpreventable.The SHROOM3 gene has been implicated in NTD cases that are unresponsive to folate supplementation;at present,however,the underlying mechanism remains unclear.Neural tube morphogenesis is a complex process involving the folding of the planar epithelium of the neural plate.To determine the role of SHROOM3 in early developmental morphogenesis,we established a neuroepithelial organoid culture system derived from cynomolgus monkeys to closely mimic the in vivo neural plate phase.Loss of SHROOM3 resulted in shorter neuroepithelial cells and smaller nuclei.These morphological changes were attributed to the insufficient recruitment of cytoskeletal proteins,namely fibrous actin(F-actin),myosin II,and phospho-myosin light chain(PMLC),to the apical side of the neuroepithelial cells.Notably,these defects were not rescued by folate supplementation.RNA sequencing revealed that differentially expressed genes were enriched in biological processes associated with cellular and organ morphogenesis.In summary,we established an authentic in vitro system to study NTDs and identified a novel mechanism for NTDs that are unresponsive to folate supplementation.展开更多
Neurovascular interactions are crucial for the normal development of the central nervous system. To study such interactions in primary cultures, we developed a procedure to simultaneously isolate neural progenitor and...Neurovascular interactions are crucial for the normal development of the central nervous system. To study such interactions in primary cultures, we developed a procedure to simultaneously isolate neural progenitor and endothelial cell fractions from embryonic mouse brains. Depending on the culture conditions endothelial cells were found to favor maintenance of the neuroprogenitor phenotype through the production of soluble factors, or to promote neuronal differentiation of neural progenitors through direct contact. These apparently opposing effects could reflect differential cellular interactions needed for the proper development of the brain.展开更多
Objective The accurate diagnosis of the non-specific variant of dysembryoplastic neuroepithelial tumor （DNT） is very difficult because it is characterized by absence of the histological hallmark of the ＂specific gl...Objective The accurate diagnosis of the non-specific variant of dysembryoplastic neuroepithelial tumor （DNT） is very difficult because it is characterized by absence of the histological hallmark of the ＂specific glioneuronal element＂ in lesions. We herein present two cases of the non-specific form of DNT to analyze the clinical, radiological, and histological features of this unusual subtype of DNT. Methods A 16-year-old and a 23-year-old patient had been treated for pharmacoresistant epilepsy for several years before undergoing referral to the hospital for further examination and treatment. Magnetic resonance imaging （MRI） revealed that both patients had a small, well-demarcated cystic lesion within the cortex of the brain without obvious contrast enhancement or peritumoral edema. The lesions were totally resected and routinely examined using histological and immunohistochemical analysis. Results Both lesions exhibited similar histological appearances with cyst formation and mural nodule architecture. The glial nodules were mainly composed of oligodendrocyte-like components, and partly of pi^oid cells resembling pilocytic astrocytoma. The cortex adjacent to the lesion in both cases was found to have the histological features of focal cortical dysplasia （FCD） Type I. Immunohistochemically, the oligoden- drocyte-like components were diffusely positive for Syn and Olig-2, but staining for CD34, p53, and IDH1 R132H was negative. The Ki-67 （MIB-1） labeling index was low, approximately 1%. There was no 1p/19q co-deletion in either lesion by fluorescence in situ hybridization （FISH） assay. Neither patient received postoperative adjuvant treatment, and both underwent regular follow-up for at least 24 months. No signs of recurrence or epileptic attacks were observed during the follow-up period. Conclusion The non-specific variant of DNT is a diagnostic challenge for pathologists in clinical practice, and differentiation from some low-grade gliomas needs to be considered. The careful inspection of radio- logic and histopathologic findings, accompanied by analysis of patients＇ clinical manifestations, may be helpful in making an accurate diagnosis.展开更多
Background The goal of surgery in the treatment of intrinsic cerebral tumors is to resect the maximum tumor volume,and to spare the eloquent areas. However, it is difficult to discover the eloquent areas intraoperativ...Background The goal of surgery in the treatment of intrinsic cerebral tumors is to resect the maximum tumor volume,and to spare the eloquent areas. However, it is difficult to discover the eloquent areas intraoperatively due to individual anatomo-functional variability both for sensori-motor and language functions. Consequently, the surgery of intrinsic cerebral tumors frequently results in poor extent of resection or permanent postoperative deficits, or both, and remains a difficult problem for neurosurgeons.Methods From January 2003 to January 2010, 112 patients with neuroepithelial tumors in/close to the eloquent areas were operated on under awake anesthesia with the intraoperative direct electrical stimulation for functional mapping of the eloquent areas. The extent of the tumors was verified by intraoperative ultrasonography. The maximal resection of the tumors and minimal damage of the eloquent areas were the surgical goal of all patients.Results Totally 356 cortical sites in 99 patients were detected for motor response by intraoperative direct electrical stimulation, 50 sites in 16 patients for sensory, 72 sites in 48 patients for language. Sixty-six patients （58.9%） achieved total resection, 34 （30.4%） subtotal and 12 （10.7%） partial. Fifty-eight patients （51.8%） had no postoperative deficit, while 37 patients （33.0%） had transitory postoperative paralysis, 26 patients （23.2%） with transitory postoperative language disturbance and 3 patients （2.7%） with permanent neurological deficits. No patient complained of pain recollection following operation.Conclusions Awake anesthesia, intraoperative direct electrical stimulation and ultrasonography are three core techniques for the resection of intrinsic cerebral tumors near the eloquent areas. This new concept allows an improvement in the quality of surgery for neuroepithelial tumors in/adjacent to eloquent areas.展开更多
Background Dysembryoplastic neuroepithelial tumor （DNT） is a rare benign neoplasm of the central nervous system affecting young people. A correct preoperative diagnosis is helpful for planning surgical strategies an...Background Dysembryoplastic neuroepithelial tumor （DNT） is a rare benign neoplasm of the central nervous system affecting young people. A correct preoperative diagnosis is helpful for planning surgical strategies and improving prognosis. The purpose of this study was to characterize DNTs using magnetic resonance imaging （MRI） and magnetic resonance spectroscopy （MRS） and to analyze the value of these two techniques in the diagnosis of DNTs. Methods MR images of 13 patients with DNTs were reviewed retrospectively; and five of the patients also underwent MRS. Tumors were confirmed by surgery. The distribution, extension and signal features of the lesions were assessed, and the MRS results were analyzed. Results All tumors were supratentorial. The cortex was the main area involved, with nine tumors located in the temporal lobe, three in the frontal lobe, and one on the boundary between the temporal and occipital lobes. All cases had decreased signal intensity on Tl-weighted MR images and increased signal intensity on T2-weighted images. On fluid attenuated inversion recovery weighted images, the hyperintense ＂ring sign＂ and internal septation of the lesion were seen in 9 cases. Eight tumors had well-demarcated borders. Peritumoral edema or mass effect was absent in all cases. A contrast enhancement examination was performed in 9 cases. Contrast enhancement was absent in five cases, and four cases showed significant enhancement. The MRS showed a low N-acetylaspartate peak and a lack of elevated choline-containing component （Cho） or Cho-Cr ratio （Cho/Cr） in five patients. Conclusions The MRI findings of DNTs were stereotypical. The combination of MRI and MRS techniques were helpful in making a correct presurgical diagnosis.展开更多
Clinical data have shown that survival rates vary considerably among brain tumor patients,according to the type and grade of the tumor.Metabolite profiles of intact tumor tissues measured with high-resolution magic-an...Clinical data have shown that survival rates vary considerably among brain tumor patients,according to the type and grade of the tumor.Metabolite profiles of intact tumor tissues measured with high-resolution magic-angle spinning proton nuclear magnetic resonance spectroscopy (HRMAS 1H NMRS) can provide important information on tumor biology and metabolism.These metabolic fingerprints can then be used for tumor classification and grading,with great potential value for tumor diagnosis.We studied the metabolic characteristics of 30 neuroepithelial tumor biopsies,including two astrocytomas (grade I),12 astrocytomas (grade II),eight anaplastic astrocytomas (grade III),three glioblastomas (grade IV) and five medulloblastomas (grade IV) from 30 patients using HRMAS 1H NMRS.The results were correlated with pathological features using multivariate data analysis,including principal component analysis (PCA).There were significant differences in the levels of N-acetyl-aspartate (NAA),creatine,myo-inositol,glycine and lactate between tumors of different grades (P<0.05).There were also significant differences in the ratios of NAA/creatine,lactate/creatine,myo-inositol/creatine,glycine/creatine,scyllo-inositol/creatine and alanine/creatine (P<0.05).A soft independent modeling of class analogy model produced a predictive accuracy of 87% for high-grade (grade III-IV) brain tumors with a sensitivity of 87% and a specificity of 93%.HRMAS 1H NMR spectroscopy in conjunction with pattern recognition thus provides a potentially useful tool for the rapid and accurate classification of human brain tumor grades.展开更多
Dysembryoplastic neuroepithelial tumors ( DNTs ), which were first describedby Daumas-Duport in 1988, are one of rare benign tumors usually associated with medicallyintractable seizures which date from childhood. The ...Dysembryoplastic neuroepithelial tumors ( DNTs ), which were first describedby Daumas-Duport in 1988, are one of rare benign tumors usually associated with medicallyintractable seizures which date from childhood. The clinical, pathologic and neuroradiologicfindings of DNT have been described. Recent advances in magnetic resonance imaging ( MRI) technologyallow the acquisition of cerebral microcirculation parameters by perfusion weighted imaging ( PWI)and brain metabolic indices by MR spectroscopy (MRS) . Several studies have shown the utility of PWIand MRS can improve the diagnostic accuracy of brain tumor, we combine the two techniques toevaluate a case with DNT and suggest that wider application of these techniques is warranted.展开更多
Background:Dysembryoplastic neuroepithelial tumors(DNTs)are common causes of intractable epilepsy in pediatric epilepsy patients.The effect of surgical intervention is often limited when the tumor is located in the pr...Background:Dysembryoplastic neuroepithelial tumors(DNTs)are common causes of intractable epilepsy in pediatric epilepsy patients.The effect of surgical intervention is often limited when the tumor is located in the precentral gyrus.Furthermore,complete surgical resection is often not performed in order to avoid permanent neurological deficits.Methods:Here,we present a pediatric patient with intractable epilepsy caused by a simple DNT located in the precentral gyrus.Intracranial electrodes were implanted and used in combination with magnetic resonance imaging,video-electroencephalography and electrical cortical stimulation to assess neurological function,and where the epileptogenic zone was located.Results:The results of intracranial electrode monitoring suggested that the epileptogenic zone was located in the tumor area and that cortical function had been reorganized.We completely resected the tumor based on these findings.The patient has been seizure free after the surgery and has not had any neurological deficits.Conclusions:Simple form DNTs in the precentral gyrus can be completely resected with careful preoperative assessment of cortical function.Cortical reorganization could partly explain the functional preservation after surgery.展开更多
Dysembryoplastic neuroepithelial tumors （DNTs） are graded as neural and mixed neuroglial tumors （WHO Ⅰ）.They almost locate in superior tentorium lobe,and often in temporal lobe.It frequently associated with intra...Dysembryoplastic neuroepithelial tumors （DNTs） are graded as neural and mixed neuroglial tumors （WHO Ⅰ）.They almost locate in superior tentorium lobe,and often in temporal lobe.It frequently associated with intractable localization-related epilepsy in children and young adults.Its typical pathological features include disorganized arrangement of neuronal and glial elements without significant cytological atypia and frequent association of foci of dysplastic cortical disorganization.DNTs are generally regarded as essentially benign lesions,so surgical complete resection is the unique treatment without any need for chemotherapy and/or radiation therapy.Keywords：intraventricular dysembryoplastic neuroepithelial tumor; manifestation; treatment展开更多
Long-term epilepsy associated tumors(LEAT) represent a well known cause of focal epilepsies. Glioneuronaltumors are the most frequent histological type consisting of a mixture of glial and neuronal elements and most c...Long-term epilepsy associated tumors(LEAT) represent a well known cause of focal epilepsies. Glioneuronaltumors are the most frequent histological type consisting of a mixture of glial and neuronal elements and most commonly ariseing in the temporal lobe. Cortical dysplasia or other neuronal migration abnormalities often coexist. Epilepsy associated with LEAT is generally poorly controlled by antiepileptic drugs while, on the other hand, it is high responsive to surgical treatment. However the best management strategy of tumor-related focal epilepsies remains controversial representing a contemporary issues in epilepsy surgery. Temporo-mesial LEAT have a widespread epileptic networkwith complex epileptogenic mechanisms. By using an epilepsy surgery oriented strategy LEAT may have an excellent seizure outcome therefore surgical treatment should be offered early, irrespective of pharmacoresistance, avoiding both the consequences of uncontrolled seizures as well as the side effects of prolonged pharmacological therapy and the rare risk of malignant transformation.展开更多
CTP synthase （CTPsyn） is a metabolic enzyme responsible for the de novo synthesis of the nucleotide CTE Several recent studies have shown that CTPsyn forms filamentous subcellular structures known as cytoophidia in ...CTP synthase （CTPsyn） is a metabolic enzyme responsible for the de novo synthesis of the nucleotide CTE Several recent studies have shown that CTPsyn forms filamentous subcellular structures known as cytoophidia in bacteria, yeast, fruit flies and humans. However, it remains elusive whether and how CTPsyn and cytoophidia play a role during development. Here, we show that cytoophidia are abundant in the neuroepithelial stem cells in Drosophila optic lobes. Optic lobes are underdeveloped in CTPsyn mutants as well as in CTPsyn RNAi. Moreover, overexpressing CTPsyn impairs the development of optic lobes, specifically by blocking the transition from neuro- epithelium to neuroblast. Taken together, our results indicate that CTPsyn is critical for optic lobe homeostasis in Drosophila.展开更多
Hydrogen sulfide(H2S)is considered as a protective factor against cardiovascular disorders.However,there are few reports on the effects of H2S in the central nervous system during stress or injury.Previous studies on ...Hydrogen sulfide(H2S)is considered as a protective factor against cardiovascular disorders.However,there are few reports on the effects of H2S in the central nervous system during stress or injury.Previous studies on goldfish have shown that astrocytic response occurs in the damaged and contralateral optic nerves.Glial fibrillary acidic protein(GFAP)concentration in the optic nerves of rainbow trout has not been measured previously.This study further characterized the astrocytic response in the optic nerve and the brain of a rainbow trout(Oncorhynchus mykiss)after unilateral eye injury and estimated the amount of H2S-producing enzyme cystathionineβ-synthase(CBS)in the brain of the rainbow trout.Within 1 week after unilateral eye injury,a protein band corresponding to a molecular weight of 50 kDa was identified in the ipsi-and contralateral optic nerves of the rainbow trout.The concentration of GFAP in the injured optic nerve increased compared to the protein concentration on the contralateral side.The results of a quantitative analysis of GFAP+cell distribution in the contralateral optic nerve showed the largest number of GFAP+cells and fibers in the optic nerve head.In the damaged optic nerve,patterns of GFAP+cell migration and large GFAP+bipolar activated astrocytes were detected at 1 week after unilateral eye injury.The study of H2S-producing system after unilateral eye injury in the rainbow trout was conducted using enzyme-linked immunosorbent assay,western blot analysis,and immunohistochemistry of polyclonal antibodies again st CBS in the integrative centers of the brain:telencephalon,optic tectum,and cerebellum.Enzyme-linked immunosorbent assay results showed a 1.7-fold increase in CBS expression in the rainbow trout brain at 1 week after unilateral eye injury compared with that in intact animals.In the ventricular and subventricular regions of the rainbow trout telencephalon,CBS+radial glia and neuroepithelial cells were identified.After unilateral eye injury,the number of CBS+neuroepithelial cells in the pallial and subpallial periventricular regions of the telencephalon increased.In the optic tectum,unilateral eye injury led to an increase in CBS expression in radial glial cells;simultaneously,the number of CBS+neuroepithelial cells decreased in intact animals.In the cerebellum of the rainbow trout,neuroglial interrelationships were revealed,where H2S was released,apparently,from astrocyte-like cells.The organization of H2S-producing cell complexes suggests that,the amount of glutamate produced in the rainbow trout cerebellum and its reuptake was controlled by astrocyte-like cells,reducing its excitotoxicity.In the dorsal matrix zone and granular eminences of the rainbow trout cerebellum,CBS was expressed in neuroepithelial cells.After unilateral eye injury,the level of CBS activity increased in all parts of the cerebellum.An increase in the number of H2S-producing cells was a response to oxidative stress after unilateral eye injury,and the overproduction of H2S in the cerebellum occurred to neutralize reactive oxygen species,providing the cells of the rainbow trout cerebellum with a protective effect.A structural reorganization in the dorsal matrix zone,associated with the appearance of an additional CBS+apical zone,and a decrease in the enzyme activity in the dorsal matrix zone,was revealed in the zones of constitutive neurogenesis.All experiments were approved by the Commission on Biomedical Ethics,A.V.Zhirmunsky National Scientific Center of Marine Biology(NSCMB),Far Eastern Branch,Russian Academy of Science(FEB RAS)(approval No.1)on July 31,2019.展开更多
基金supported by the National Natural Science Foundation of China (81930121,82125008 to Y.C.C.)National Key Research and Development Program of China (2018YFA0107902 to Y.C.C.and 2018YFA0801403 to Z.B.W.)+1 种基金Major Basic Research Project of Science and Technology of Yunnan (202001BC070001 to Y.C.C.)Natural Science Foundation of Yunnan Province (202102AA100053 to Y.C.C.)。
文摘Neural tube defects(NTDs)are severe congenital neurodevelopmental disorders arising from incomplete neural tube closure.Although folate supplementation has been shown to mitigate the incidence of NTDs,some cases,often attributable to genetic factors,remain unpreventable.The SHROOM3 gene has been implicated in NTD cases that are unresponsive to folate supplementation;at present,however,the underlying mechanism remains unclear.Neural tube morphogenesis is a complex process involving the folding of the planar epithelium of the neural plate.To determine the role of SHROOM3 in early developmental morphogenesis,we established a neuroepithelial organoid culture system derived from cynomolgus monkeys to closely mimic the in vivo neural plate phase.Loss of SHROOM3 resulted in shorter neuroepithelial cells and smaller nuclei.These morphological changes were attributed to the insufficient recruitment of cytoskeletal proteins,namely fibrous actin(F-actin),myosin II,and phospho-myosin light chain(PMLC),to the apical side of the neuroepithelial cells.Notably,these defects were not rescued by folate supplementation.RNA sequencing revealed that differentially expressed genes were enriched in biological processes associated with cellular and organ morphogenesis.In summary,we established an authentic in vitro system to study NTDs and identified a novel mechanism for NTDs that are unresponsive to folate supplementation.
文摘Neurovascular interactions are crucial for the normal development of the central nervous system. To study such interactions in primary cultures, we developed a procedure to simultaneously isolate neural progenitor and endothelial cell fractions from embryonic mouse brains. Depending on the culture conditions endothelial cells were found to favor maintenance of the neuroprogenitor phenotype through the production of soluble factors, or to promote neuronal differentiation of neural progenitors through direct contact. These apparently opposing effects could reflect differential cellular interactions needed for the proper development of the brain.
文摘Objective The accurate diagnosis of the non-specific variant of dysembryoplastic neuroepithelial tumor （DNT） is very difficult because it is characterized by absence of the histological hallmark of the ＂specific glioneuronal element＂ in lesions. We herein present two cases of the non-specific form of DNT to analyze the clinical, radiological, and histological features of this unusual subtype of DNT. Methods A 16-year-old and a 23-year-old patient had been treated for pharmacoresistant epilepsy for several years before undergoing referral to the hospital for further examination and treatment. Magnetic resonance imaging （MRI） revealed that both patients had a small, well-demarcated cystic lesion within the cortex of the brain without obvious contrast enhancement or peritumoral edema. The lesions were totally resected and routinely examined using histological and immunohistochemical analysis. Results Both lesions exhibited similar histological appearances with cyst formation and mural nodule architecture. The glial nodules were mainly composed of oligodendrocyte-like components, and partly of pi^oid cells resembling pilocytic astrocytoma. The cortex adjacent to the lesion in both cases was found to have the histological features of focal cortical dysplasia （FCD） Type I. Immunohistochemically, the oligoden- drocyte-like components were diffusely positive for Syn and Olig-2, but staining for CD34, p53, and IDH1 R132H was negative. The Ki-67 （MIB-1） labeling index was low, approximately 1%. There was no 1p/19q co-deletion in either lesion by fluorescence in situ hybridization （FISH） assay. Neither patient received postoperative adjuvant treatment, and both underwent regular follow-up for at least 24 months. No signs of recurrence or epileptic attacks were observed during the follow-up period. Conclusion The non-specific variant of DNT is a diagnostic challenge for pathologists in clinical practice, and differentiation from some low-grade gliomas needs to be considered. The careful inspection of radio- logic and histopathologic findings, accompanied by analysis of patients＇ clinical manifestations, may be helpful in making an accurate diagnosis.
文摘Background The goal of surgery in the treatment of intrinsic cerebral tumors is to resect the maximum tumor volume,and to spare the eloquent areas. However, it is difficult to discover the eloquent areas intraoperatively due to individual anatomo-functional variability both for sensori-motor and language functions. Consequently, the surgery of intrinsic cerebral tumors frequently results in poor extent of resection or permanent postoperative deficits, or both, and remains a difficult problem for neurosurgeons.Methods From January 2003 to January 2010, 112 patients with neuroepithelial tumors in/close to the eloquent areas were operated on under awake anesthesia with the intraoperative direct electrical stimulation for functional mapping of the eloquent areas. The extent of the tumors was verified by intraoperative ultrasonography. The maximal resection of the tumors and minimal damage of the eloquent areas were the surgical goal of all patients.Results Totally 356 cortical sites in 99 patients were detected for motor response by intraoperative direct electrical stimulation, 50 sites in 16 patients for sensory, 72 sites in 48 patients for language. Sixty-six patients （58.9%） achieved total resection, 34 （30.4%） subtotal and 12 （10.7%） partial. Fifty-eight patients （51.8%） had no postoperative deficit, while 37 patients （33.0%） had transitory postoperative paralysis, 26 patients （23.2%） with transitory postoperative language disturbance and 3 patients （2.7%） with permanent neurological deficits. No patient complained of pain recollection following operation.Conclusions Awake anesthesia, intraoperative direct electrical stimulation and ultrasonography are three core techniques for the resection of intrinsic cerebral tumors near the eloquent areas. This new concept allows an improvement in the quality of surgery for neuroepithelial tumors in/adjacent to eloquent areas.
文摘Background Dysembryoplastic neuroepithelial tumor （DNT） is a rare benign neoplasm of the central nervous system affecting young people. A correct preoperative diagnosis is helpful for planning surgical strategies and improving prognosis. The purpose of this study was to characterize DNTs using magnetic resonance imaging （MRI） and magnetic resonance spectroscopy （MRS） and to analyze the value of these two techniques in the diagnosis of DNTs. Methods MR images of 13 patients with DNTs were reviewed retrospectively; and five of the patients also underwent MRS. Tumors were confirmed by surgery. The distribution, extension and signal features of the lesions were assessed, and the MRS results were analyzed. Results All tumors were supratentorial. The cortex was the main area involved, with nine tumors located in the temporal lobe, three in the frontal lobe, and one on the boundary between the temporal and occipital lobes. All cases had decreased signal intensity on Tl-weighted MR images and increased signal intensity on T2-weighted images. On fluid attenuated inversion recovery weighted images, the hyperintense ＂ring sign＂ and internal septation of the lesion were seen in 9 cases. Eight tumors had well-demarcated borders. Peritumoral edema or mass effect was absent in all cases. A contrast enhancement examination was performed in 9 cases. Contrast enhancement was absent in five cases, and four cases showed significant enhancement. The MRS showed a low N-acetylaspartate peak and a lack of elevated choline-containing component （Cho） or Cho-Cr ratio （Cho/Cr） in five patients. Conclusions The MRI findings of DNTs were stereotypical. The combination of MRI and MRS techniques were helpful in making a correct presurgical diagnosis.
基金supported by the National Natural Science Foundation of China (Grant Nos. 20573132 and 20575074)China Postdoctoral Science Foundation (Grant No. 20090450065)State Key Laboratory of Mag-netic Resonance and Atomic and Molecular Physics (Grant No. T152805)
文摘Clinical data have shown that survival rates vary considerably among brain tumor patients,according to the type and grade of the tumor.Metabolite profiles of intact tumor tissues measured with high-resolution magic-angle spinning proton nuclear magnetic resonance spectroscopy (HRMAS 1H NMRS) can provide important information on tumor biology and metabolism.These metabolic fingerprints can then be used for tumor classification and grading,with great potential value for tumor diagnosis.We studied the metabolic characteristics of 30 neuroepithelial tumor biopsies,including two astrocytomas (grade I),12 astrocytomas (grade II),eight anaplastic astrocytomas (grade III),three glioblastomas (grade IV) and five medulloblastomas (grade IV) from 30 patients using HRMAS 1H NMRS.The results were correlated with pathological features using multivariate data analysis,including principal component analysis (PCA).There were significant differences in the levels of N-acetyl-aspartate (NAA),creatine,myo-inositol,glycine and lactate between tumors of different grades (P<0.05).There were also significant differences in the ratios of NAA/creatine,lactate/creatine,myo-inositol/creatine,glycine/creatine,scyllo-inositol/creatine and alanine/creatine (P<0.05).A soft independent modeling of class analogy model produced a predictive accuracy of 87% for high-grade (grade III-IV) brain tumors with a sensitivity of 87% and a specificity of 93%.HRMAS 1H NMR spectroscopy in conjunction with pattern recognition thus provides a potentially useful tool for the rapid and accurate classification of human brain tumor grades.
文摘Dysembryoplastic neuroepithelial tumors ( DNTs ), which were first describedby Daumas-Duport in 1988, are one of rare benign tumors usually associated with medicallyintractable seizures which date from childhood. The clinical, pathologic and neuroradiologicfindings of DNT have been described. Recent advances in magnetic resonance imaging ( MRI) technologyallow the acquisition of cerebral microcirculation parameters by perfusion weighted imaging ( PWI)and brain metabolic indices by MR spectroscopy (MRS) . Several studies have shown the utility of PWIand MRS can improve the diagnostic accuracy of brain tumor, we combine the two techniques toevaluate a case with DNT and suggest that wider application of these techniques is warranted.
文摘Background:Dysembryoplastic neuroepithelial tumors(DNTs)are common causes of intractable epilepsy in pediatric epilepsy patients.The effect of surgical intervention is often limited when the tumor is located in the precentral gyrus.Furthermore,complete surgical resection is often not performed in order to avoid permanent neurological deficits.Methods:Here,we present a pediatric patient with intractable epilepsy caused by a simple DNT located in the precentral gyrus.Intracranial electrodes were implanted and used in combination with magnetic resonance imaging,video-electroencephalography and electrical cortical stimulation to assess neurological function,and where the epileptogenic zone was located.Results:The results of intracranial electrode monitoring suggested that the epileptogenic zone was located in the tumor area and that cortical function had been reorganized.We completely resected the tumor based on these findings.The patient has been seizure free after the surgery and has not had any neurological deficits.Conclusions:Simple form DNTs in the precentral gyrus can be completely resected with careful preoperative assessment of cortical function.Cortical reorganization could partly explain the functional preservation after surgery.
文摘Dysembryoplastic neuroepithelial tumors （DNTs） are graded as neural and mixed neuroglial tumors （WHO Ⅰ）.They almost locate in superior tentorium lobe,and often in temporal lobe.It frequently associated with intractable localization-related epilepsy in children and young adults.Its typical pathological features include disorganized arrangement of neuronal and glial elements without significant cytological atypia and frequent association of foci of dysplastic cortical disorganization.DNTs are generally regarded as essentially benign lesions,so surgical complete resection is the unique treatment without any need for chemotherapy and/or radiation therapy.Keywords：intraventricular dysembryoplastic neuroepithelial tumor; manifestation; treatment
文摘Long-term epilepsy associated tumors(LEAT) represent a well known cause of focal epilepsies. Glioneuronaltumors are the most frequent histological type consisting of a mixture of glial and neuronal elements and most commonly ariseing in the temporal lobe. Cortical dysplasia or other neuronal migration abnormalities often coexist. Epilepsy associated with LEAT is generally poorly controlled by antiepileptic drugs while, on the other hand, it is high responsive to surgical treatment. However the best management strategy of tumor-related focal epilepsies remains controversial representing a contemporary issues in epilepsy surgery. Temporo-mesial LEAT have a widespread epileptic networkwith complex epileptogenic mechanisms. By using an epilepsy surgery oriented strategy LEAT may have an excellent seizure outcome therefore surgical treatment should be offered early, irrespective of pharmacoresistance, avoiding both the consequences of uncontrolled seizures as well as the side effects of prolonged pharmacological therapy and the rare risk of malignant transformation.
文摘CTP synthase （CTPsyn） is a metabolic enzyme responsible for the de novo synthesis of the nucleotide CTE Several recent studies have shown that CTPsyn forms filamentous subcellular structures known as cytoophidia in bacteria, yeast, fruit flies and humans. However, it remains elusive whether and how CTPsyn and cytoophidia play a role during development. Here, we show that cytoophidia are abundant in the neuroepithelial stem cells in Drosophila optic lobes. Optic lobes are underdeveloped in CTPsyn mutants as well as in CTPsyn RNAi. Moreover, overexpressing CTPsyn impairs the development of optic lobes, specifically by blocking the transition from neuro- epithelium to neuroblast. Taken together, our results indicate that CTPsyn is critical for optic lobe homeostasis in Drosophila.
基金supported by a grant from the President of the Russian Federation(No.MD-4318.2015.4 to EVP)a grant from the Program for Basic Research of the Far East Branch of the Russian Academy of Sciences 2015–2017(No.15-I-6-116,section III to EVP,AAV and DKO)
文摘Hydrogen sulfide(H2S)is considered as a protective factor against cardiovascular disorders.However,there are few reports on the effects of H2S in the central nervous system during stress or injury.Previous studies on goldfish have shown that astrocytic response occurs in the damaged and contralateral optic nerves.Glial fibrillary acidic protein(GFAP)concentration in the optic nerves of rainbow trout has not been measured previously.This study further characterized the astrocytic response in the optic nerve and the brain of a rainbow trout(Oncorhynchus mykiss)after unilateral eye injury and estimated the amount of H2S-producing enzyme cystathionineβ-synthase(CBS)in the brain of the rainbow trout.Within 1 week after unilateral eye injury,a protein band corresponding to a molecular weight of 50 kDa was identified in the ipsi-and contralateral optic nerves of the rainbow trout.The concentration of GFAP in the injured optic nerve increased compared to the protein concentration on the contralateral side.The results of a quantitative analysis of GFAP+cell distribution in the contralateral optic nerve showed the largest number of GFAP+cells and fibers in the optic nerve head.In the damaged optic nerve,patterns of GFAP+cell migration and large GFAP+bipolar activated astrocytes were detected at 1 week after unilateral eye injury.The study of H2S-producing system after unilateral eye injury in the rainbow trout was conducted using enzyme-linked immunosorbent assay,western blot analysis,and immunohistochemistry of polyclonal antibodies again st CBS in the integrative centers of the brain:telencephalon,optic tectum,and cerebellum.Enzyme-linked immunosorbent assay results showed a 1.7-fold increase in CBS expression in the rainbow trout brain at 1 week after unilateral eye injury compared with that in intact animals.In the ventricular and subventricular regions of the rainbow trout telencephalon,CBS+radial glia and neuroepithelial cells were identified.After unilateral eye injury,the number of CBS+neuroepithelial cells in the pallial and subpallial periventricular regions of the telencephalon increased.In the optic tectum,unilateral eye injury led to an increase in CBS expression in radial glial cells;simultaneously,the number of CBS+neuroepithelial cells decreased in intact animals.In the cerebellum of the rainbow trout,neuroglial interrelationships were revealed,where H2S was released,apparently,from astrocyte-like cells.The organization of H2S-producing cell complexes suggests that,the amount of glutamate produced in the rainbow trout cerebellum and its reuptake was controlled by astrocyte-like cells,reducing its excitotoxicity.In the dorsal matrix zone and granular eminences of the rainbow trout cerebellum,CBS was expressed in neuroepithelial cells.After unilateral eye injury,the level of CBS activity increased in all parts of the cerebellum.An increase in the number of H2S-producing cells was a response to oxidative stress after unilateral eye injury,and the overproduction of H2S in the cerebellum occurred to neutralize reactive oxygen species,providing the cells of the rainbow trout cerebellum with a protective effect.A structural reorganization in the dorsal matrix zone,associated with the appearance of an additional CBS+apical zone,and a decrease in the enzyme activity in the dorsal matrix zone,was revealed in the zones of constitutive neurogenesis.All experiments were approved by the Commission on Biomedical Ethics,A.V.Zhirmunsky National Scientific Center of Marine Biology(NSCMB),Far Eastern Branch,Russian Academy of Science(FEB RAS)(approval No.1)on July 31,2019.
