Objective:lobectomy is an effective therapy for patients with Sturge-Weber syndrome(SWS).Perioperative complications often play a critical role for SWS patients’rehabilitation.This study aimed to explore and the fact...Objective:lobectomy is an effective therapy for patients with Sturge-Weber syndrome(SWS).Perioperative complications often play a critical role for SWS patients’rehabilitation.This study aimed to explore and the factors of perioperative complications in SWS patients.Methods:we reviewed retrospectively the clinical profile of totally 60 SWS patients who received surgically treatments in Sanbo Brain Hospital,Capital Medical University,from March 2009 to April 2018.Univariate analyses were used to identify the potential predictors of perioperative complications.Results:the average hospitalization time of 60 patients was(35.57±10.79)d.After surgery,54(90.00%)patients reached Engle I level.The most common postoperative complications were fever(83.33%),motor function damage(38.33%)and hyponatremia(55.00%).Univariate analyses revealed that mental retardation,seizure types and surgery types could be the predictive factors for postoperative complications.Conclusion:postoperative complications are common in SWS patients.Prediction of the severity can help doctors know what kind of special care SWS patients need to help them for further rehabilitation.展开更多
Researchers have widely acknowledged the therapeutic value of epilepsy surgery for drug-resistant epilepsy.None-theless,there is a substantial gap in the surgical treatment for appropriate candidates owing to several ...Researchers have widely acknowledged the therapeutic value of epilepsy surgery for drug-resistant epilepsy.None-theless,there is a substantial gap in the surgical treatment for appropriate candidates owing to several factors,particularly in the population of young children.To standardize the protocols of preoperative evaluation and sur-gery of young children for epilepsy surgery,the China Association Against Epilepsy has appointed an expert task force to standardize the protocols of preoperative evaluation and surgery in pediatric epilepsy patients.It adopted the modifed Delphi method and performed two rounds of surveys through an anonymous inquiry among 75 experts from four subgroups including pediatric neurologists,epileptologists,pediatric epilepsy surgeons,and functional neurosurgeons.The survey contents contained:(1)the participants,comprising children aged≤6 years;(2)adopted DRE definition proposed by the International League Against Epilepsy in 2010;and(3)investigated epilepsy surgery,principally referring to curative epilepsy surgeries.The neuromodulation therapies were excluded because of the differences in treatment mechanisms from the above-mentioned surgeries.According to the Delphi process,a con-sensus was achieved for most aspects by incorporating two rounds of surveys including preoperative assessment,sur-gical strategies and techniques,and perioperative and long-term postoperative management,despite controversial opinions on certain items.We hope the results of this consensus will improve the level of surgical treatment and man-agement of intractable epilepsy in young children.展开更多
Rasmussen's encephalitis(RE) is a rare and severe progressive epileptic syndrome with unknown etiology. Infection by viruses, including human cytomegalovirus(HCMV), has been speculated to be a potential trigger fo...Rasmussen's encephalitis(RE) is a rare and severe progressive epileptic syndrome with unknown etiology. Infection by viruses, including human cytomegalovirus(HCMV), has been speculated to be a potential trigger for RE. However, no viral antigens have been detected in the brains of patients with RE; thus, a possible clinical linkage between viral infections and RE has not been firmly established. In this study, we evaluated the expression of HCMV pp65 antigen in brain sections from 26 patients with RE and 20 non-RE patients by immunohistochemistry and in situ hybridization, and assessed the associations between HCMV infection and clinical parameters.Elevated expression of HCMV pp65 protein and DNA was observed in 88.5%(23/26) and 69.2%(18/26) of RE cases, respectively. In the non-RE group, HCMV pp65 antigen was detected only in two cases(10%), both of which were negative for DNA staining. Additionally, the intensity of HCMV pp65 staining was correlated with a shorter duration of the prodromal stage, younger age of seizure onset, and more severe unilateral cortical atrophy. Elevated expression of HCMV pp65 was observed in RE brain tissue and was correlated with the clinical features of RE disease. In summary, our results suggested that HCMV infection may be involved in the occurrence and progression of RE disease. Thus, further studies are needed to determine whether early treatment with anti-HCMV antibodies could modulate the course of RE.展开更多
Rasmussen's encephalitis (RE) is a rare pediatric neurological disorder, and the exact etiology is not clear. Viral infection may be involved in the pathogenesis of RE, but conflicting results have reported. In th...Rasmussen's encephalitis (RE) is a rare pediatric neurological disorder, and the exact etiology is not clear. Viral infection may be involved in the pathogenesis of RE, but conflicting results have reported. In this study, we evaluated the expression of both Epstein-Barr virus (EBV) and human herpes virus (HHV) 6 antigens in brain sections from 30 patients with RE and16 control individuals by immunohistochemistry. In the RE group, EBV and HHV6 antigens were detected in 56.7% (17/30) and 50% (15/30) of individuals, respectively. In contrast, no detectable EBV and HHV6 antigen expression was found in brain tissues of the control group. The co-expression of EBV and HHV6 was detected in 20.0% (6/30) of individuals. In particular, a 4-year-old boy had a typical clinical course, including a medical history of viral encephalitis,intractable epilepsy, and hemispheric atrophy. The co-expression of EBV and HHV6 was detected in neurons and astrocytes in the brain tissue, accompanied by a high frequency of CD8?T cells. Our results suggest that EBV and HHV6 infection and the activation of CD8?T cells are involved in the pathogenesis of RE.展开更多
Rasmussen's encephalitis(RE) is a rare pediatric neurological disorder, the etiology of which remains unclear. It has been speculated that the immunopathogenesis of RE involves damage to neurons, which eventually ...Rasmussen's encephalitis(RE) is a rare pediatric neurological disorder, the etiology of which remains unclear. It has been speculated that the immunopathogenesis of RE involves damage to neurons, which eventually leads to the occurrence of RE. Viral infection may be a critical factor in triggering RE immunopathogenesis. In this study, we analyzed the expression of Epstein-Barr virus(EBV) antigens as well as of Toll-like receptor 3(TLR3), TLR9, and downstream adapter TIRdomain-containing adapter-inducing interferon-β(TRIF) in the brain tissues of 26 patients with RE and 16 control individuals using immunohistochemistry(IHC). In the RE group, EBV antigens were detected in 53% of individuals at various expression levels. In contrast, there was no detectable EBV antigen expression in control brain tissues. Moreover, we found marked increases in the expression of TLR3, TLR9, and TRIF in the brain tissues of RE patients compared with levels in the control group. Furthermore, among RE cases, EBV expression and high TLR3 expression were associated with more severe brain atrophy. Our results suggest that the elevated expression of EBV and TLRs may be involved in RE occurrence through the activation of downstream molecules.展开更多
文摘Objective:lobectomy is an effective therapy for patients with Sturge-Weber syndrome(SWS).Perioperative complications often play a critical role for SWS patients’rehabilitation.This study aimed to explore and the factors of perioperative complications in SWS patients.Methods:we reviewed retrospectively the clinical profile of totally 60 SWS patients who received surgically treatments in Sanbo Brain Hospital,Capital Medical University,from March 2009 to April 2018.Univariate analyses were used to identify the potential predictors of perioperative complications.Results:the average hospitalization time of 60 patients was(35.57±10.79)d.After surgery,54(90.00%)patients reached Engle I level.The most common postoperative complications were fever(83.33%),motor function damage(38.33%)and hyponatremia(55.00%).Univariate analyses revealed that mental retardation,seizure types and surgery types could be the predictive factors for postoperative complications.Conclusion:postoperative complications are common in SWS patients.Prediction of the severity can help doctors know what kind of special care SWS patients need to help them for further rehabilitation.
文摘Researchers have widely acknowledged the therapeutic value of epilepsy surgery for drug-resistant epilepsy.None-theless,there is a substantial gap in the surgical treatment for appropriate candidates owing to several factors,particularly in the population of young children.To standardize the protocols of preoperative evaluation and sur-gery of young children for epilepsy surgery,the China Association Against Epilepsy has appointed an expert task force to standardize the protocols of preoperative evaluation and surgery in pediatric epilepsy patients.It adopted the modifed Delphi method and performed two rounds of surveys through an anonymous inquiry among 75 experts from four subgroups including pediatric neurologists,epileptologists,pediatric epilepsy surgeons,and functional neurosurgeons.The survey contents contained:(1)the participants,comprising children aged≤6 years;(2)adopted DRE definition proposed by the International League Against Epilepsy in 2010;and(3)investigated epilepsy surgery,principally referring to curative epilepsy surgeries.The neuromodulation therapies were excluded because of the differences in treatment mechanisms from the above-mentioned surgeries.According to the Delphi process,a con-sensus was achieved for most aspects by incorporating two rounds of surveys including preoperative assessment,sur-gical strategies and techniques,and perioperative and long-term postoperative management,despite controversial opinions on certain items.We hope the results of this consensus will improve the level of surgical treatment and man-agement of intractable epilepsy in young children.
基金supported by the following funds: the National Natural Science Foundation of China (81571275)the Beijing Municipal Natural Science Foundation (7144217)+5 种基金the Capital Applied Clinic Research Programs of Science and Technology (Z131107002213171)the Beijing Rising-star Plan of Science and Technology (Z141107001814042)the Open Research Fund of the Beijing Key Laboratory of Epilepsy Research (No. 2014DXBL02)Capital Medical University (15JL08)Scientific Research Common Program of Beijing Municipal Commission of Education (KM201610025001)Scientific Research Foundation for the Returned Overseas Chinese Scholars, State Education Ministry (2014 1685)
文摘Rasmussen's encephalitis(RE) is a rare and severe progressive epileptic syndrome with unknown etiology. Infection by viruses, including human cytomegalovirus(HCMV), has been speculated to be a potential trigger for RE. However, no viral antigens have been detected in the brains of patients with RE; thus, a possible clinical linkage between viral infections and RE has not been firmly established. In this study, we evaluated the expression of HCMV pp65 antigen in brain sections from 26 patients with RE and 20 non-RE patients by immunohistochemistry and in situ hybridization, and assessed the associations between HCMV infection and clinical parameters.Elevated expression of HCMV pp65 protein and DNA was observed in 88.5%(23/26) and 69.2%(18/26) of RE cases, respectively. In the non-RE group, HCMV pp65 antigen was detected only in two cases(10%), both of which were negative for DNA staining. Additionally, the intensity of HCMV pp65 staining was correlated with a shorter duration of the prodromal stage, younger age of seizure onset, and more severe unilateral cortical atrophy. Elevated expression of HCMV pp65 was observed in RE brain tissue and was correlated with the clinical features of RE disease. In summary, our results suggested that HCMV infection may be involved in the occurrence and progression of RE disease. Thus, further studies are needed to determine whether early treatment with anti-HCMV antibodies could modulate the course of RE.
基金supported by the National Natural Science Foundation of China(81701992,81471957,and 81671971)the Beijing Municipal Natural Science Foundation(7144217)+2 种基金the Capital Applied Clinic Research Programs of Science and Technology(Z131107002213171)the Beijing Rising-star Plan of Science and Technology(Z141107001814042)Scientific Research Common Program of Beijing Municipal Commission of Education(KM201610025001)
文摘Rasmussen's encephalitis (RE) is a rare pediatric neurological disorder, and the exact etiology is not clear. Viral infection may be involved in the pathogenesis of RE, but conflicting results have reported. In this study, we evaluated the expression of both Epstein-Barr virus (EBV) and human herpes virus (HHV) 6 antigens in brain sections from 30 patients with RE and16 control individuals by immunohistochemistry. In the RE group, EBV and HHV6 antigens were detected in 56.7% (17/30) and 50% (15/30) of individuals, respectively. In contrast, no detectable EBV and HHV6 antigen expression was found in brain tissues of the control group. The co-expression of EBV and HHV6 was detected in 20.0% (6/30) of individuals. In particular, a 4-year-old boy had a typical clinical course, including a medical history of viral encephalitis,intractable epilepsy, and hemispheric atrophy. The co-expression of EBV and HHV6 was detected in neurons and astrocytes in the brain tissue, accompanied by a high frequency of CD8?T cells. Our results suggest that EBV and HHV6 infection and the activation of CD8?T cells are involved in the pathogenesis of RE.
基金the National Natural Science Foundation of China (81471957, 81571275, 81671971, and 81701992)the Beijing Municipal Natural Science Foundation (7144217)the Scientific Research Common Program of Beijing Municipal Commission of Education (KM201610025001)
文摘Rasmussen's encephalitis(RE) is a rare pediatric neurological disorder, the etiology of which remains unclear. It has been speculated that the immunopathogenesis of RE involves damage to neurons, which eventually leads to the occurrence of RE. Viral infection may be a critical factor in triggering RE immunopathogenesis. In this study, we analyzed the expression of Epstein-Barr virus(EBV) antigens as well as of Toll-like receptor 3(TLR3), TLR9, and downstream adapter TIRdomain-containing adapter-inducing interferon-β(TRIF) in the brain tissues of 26 patients with RE and 16 control individuals using immunohistochemistry(IHC). In the RE group, EBV antigens were detected in 53% of individuals at various expression levels. In contrast, there was no detectable EBV antigen expression in control brain tissues. Moreover, we found marked increases in the expression of TLR3, TLR9, and TRIF in the brain tissues of RE patients compared with levels in the control group. Furthermore, among RE cases, EBV expression and high TLR3 expression were associated with more severe brain atrophy. Our results suggest that the elevated expression of EBV and TLRs may be involved in RE occurrence through the activation of downstream molecules.