Background:Surgical treatment for patients with adult-onset Rasmussen’s encephalitis(A-RE)is rarely reported.We investigated the clinical and surgical features of two patients with A-RE who underwent functional hemis...Background:Surgical treatment for patients with adult-onset Rasmussen’s encephalitis(A-RE)is rarely reported.We investigated the clinical and surgical features of two patients with A-RE who underwent functional hemispherectomy.Case presentation:The data of clinical manifestations,neuroimaging,surgical treatment and surgical outcomes of two patients with A-RE was reviewed.The two patients initially presented with recurrent partial seizures or secondly generalized tonic clonic seizures.Gradually,the patients showed unilateral limb paralysis as well as chronic focal epileptic status.Both patients underwent functional hemispherectomy and achieved seizure freedom in the followup.The contralateral neurological deficits improved gradually after rehabilitation and were acceptable for the selfcare of daily living.The living quality improved prominently after surgery.Conclusions:Despite the risk of hemiplegia,functional hemispherectomy may be a choice for patients with A-RE for favorable seizure control and improved quality of life in selected patients.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, 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
基金supported by National Natural Science Foundation of China(Grant No.81771395).
文摘Background:Surgical treatment for patients with adult-onset Rasmussen’s encephalitis(A-RE)is rarely reported.We investigated the clinical and surgical features of two patients with A-RE who underwent functional hemispherectomy.Case presentation:The data of clinical manifestations,neuroimaging,surgical treatment and surgical outcomes of two patients with A-RE was reviewed.The two patients initially presented with recurrent partial seizures or secondly generalized tonic clonic seizures.Gradually,the patients showed unilateral limb paralysis as well as chronic focal epileptic status.Both patients underwent functional hemispherectomy and achieved seizure freedom in the followup.The contralateral neurological deficits improved gradually after rehabilitation and were acceptable for the selfcare of daily living.The living quality improved prominently after surgery.Conclusions:Despite the risk of hemiplegia,functional hemispherectomy may be a choice for patients with A-RE for favorable seizure control and improved quality of life in selected patients.
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.
