Background Infantile epileptic spasms syndrome(IESS)is a serious disease in infants,and it usually evolves to other epilepsy types or syndromes,especially refractory or super-refractory focal epilepsies.Although adren...Background Infantile epileptic spasms syndrome(IESS)is a serious disease in infants,and it usually evolves to other epilepsy types or syndromes,especially refractory or super-refractory focal epilepsies.Although adrenocorticotropic hormone(ACTH)is one of the first-line and effective treatment plans for IESS,it has serious side effects and is not sufficiently effective.Methods A retrospective study of the clinical outcomes of ACTH combined with magnesium sulfate(MgSO_(4))therapy for IESS in two hospital centers was conducted.The major outcome of the single and combined treatment was evaluated by changes in seizure frequency and improvements in hypsarrhythmia electroencephalography(EEG).To reduce the confounding bias between the two groups,we used SPSS for the propensity score matching(PSM)analysis.Results We initially recruited 1205 IESS patients from two Chinese hospitals and treated them with ACTH combined with MgSO_(4) and ACTH alone.Only 1005 patients were enrolled in the treatment(ACTH combined with MgSO_(4):744,ACTH:261),and both treatment plans had a more than 55% response rate.However,compared to patients treated with ACTH alone,those patients treated with ACTH combined with MgSO_(4) had better performance in terms of the seizure frequency and hypsarrhythmia EEG.After PSM,the two groups also showed significant differences in responder rate[70.8%(95% confidence interval,CI)=66.7%–74.8%)vs.53.8%(95%CI=47.4%–60.2%),P<0.001],seizure frequency(P<0.001)and hypsarrhythmia EEG resolution(P<0.001).Notably,multivariate analysis revealed that the lead time to treatment and the number of antiseizure medications taken before treatment were two factors that may affect the clinical outcome.Patients with less than 3 months of lead time responded to the treatment much better than those with>3 months(P<0.05).In addition,the overall incidence of adverse reactions in the ACTH combined with MgSO_(4) group was much lower than that in the ACTH group(31.4%vs.63.1%,P<0.001).During the treatment,only infection(P=0.045)and hypertension(P=0.025)were significantly different between the two groups,and no baby died.Conclusion Our findings support that ACTH combined with MgSO_(4) is a more effective short-term treatment protocol for patients with IESS than ACTH alone,especially for those patients with short lead times to treatment.展开更多
Background In this study,we aimed to assess the efcacy of surgical treatment in children with drug-refractory infantile epileptic spasms syndrome(IESS)and examine the factors infuencing the post-surgical outcomes.Meth...Background In this study,we aimed to assess the efcacy of surgical treatment in children with drug-refractory infantile epileptic spasms syndrome(IESS)and examine the factors infuencing the post-surgical outcomes.Methods The clinical data of 30 children(18 males and 12 females)with epileptic spasms(ES)who underwent surgery at the Epilepsy Center of Shenzhen Children’s Hospital between June 2018 and June 2020 were retrospectively analyzed.Post-surgical outcomes were evaluated using the Engel Epilepsy Surgery Outcome Scale.Scalp electroencephalography and developmental quotient were assessed preoperatively and postoperatively.Univariate analysis and exact logistic regression analyses were used to identify the factors afecting the postoperative efcacy.Results Of the 30 patients who underwent surgical resection,22(73.3%)achieved Engel’s classⅠ-Ⅱoutcomes.Additionally,motor and cognitive functions improved in 14 patients(46.7%).The development of 12(40%)patients remained at the preoperative development level.The median number of antiseizure medications taken preoperatively was 5.27(range 2-10),which decreased to 1.90(range 0-4)at the last follow-up.Seizure duration,etiology,positive positron emission tomography-magnetic resonance imaging(PET-MRI),surgery type,and lesion location were signifcantly correlated with the postoperative efcacy(P<0.05).Positive PET/MRI fndings and lesion location predicted independently the postoperative outcomes.Permanent impairments of motor or language function were rare,with only two cases reporting hydrocephalus and one reporting hemiplegia.Conclusions Surgery is an efective treatment option for children with IESS.Early referral and comprehensive preoperative evaluation are essential for identifcation of surgically treatable structural lesions.The primary surgically treatable cause is cortical malformation,followed by perinatal brain injury.Hemispheric disconnection is a preferred surgical approach.Positive PET/MRI fndings and lesion location predicted the postoperative outcomes.展开更多
Behavioral scoring based on clinical observations remains the gold standard for screening,diagnosing,and evaluating infantile epileptic spasm syndrome(IESS).The accurate identification of seizures is crucial for clini...Behavioral scoring based on clinical observations remains the gold standard for screening,diagnosing,and evaluating infantile epileptic spasm syndrome(IESS).The accurate identification of seizures is crucial for clinical diagnosis and assessment.In this study,we propose an innovative seizure detection method based on video feature recognition of patient spasms.To capture the temporal characteristics of the spasm behavior presented in the videos effectively,we incorporate asymmetric convolutions and convolution–batch normalization–ReLU(CBR)modules.Specifically within the 3D-ResNet residual blocks,we split the larger convolutional kernels into two asymmetric 3D convolutional kernels.These kernels are connected in series to enhance the ability of the convolutional layers to extract key local features,both horizontally and vertically.In addition,we introduce a 3D convolutional block attention module to enhance the spatial correlations between video frame channels efficiently.To improve the generalization ability,we design a composite loss function that combines cross-entropy loss with triplet loss to balance the classification and similarity requirements.We train and evaluate our method using the PLA IESS-VIDEO dataset,achieving an average seizure recognition accuracy of 90.59%,precision of 90.94%,and recall of 87.64%.To validate its generalization capability further,we conducted external validation using six different patient monitoring videos compared with assessments by six human experts from various medical centers.The final test results demonstrate that our method achieved a recall of 0.6476,surpassing the average level achieved by human experts(0.5595),while attaining a high F1-score of 0.7219.These findings have substantial significance for the long-term assessment of patients with IESS.展开更多
基金funded by the Capital’s Funds for Health Improvement and Research(No.2022-1-5081)National Key Research and Development Program of China(No.2016YFC1000707).
文摘Background Infantile epileptic spasms syndrome(IESS)is a serious disease in infants,and it usually evolves to other epilepsy types or syndromes,especially refractory or super-refractory focal epilepsies.Although adrenocorticotropic hormone(ACTH)is one of the first-line and effective treatment plans for IESS,it has serious side effects and is not sufficiently effective.Methods A retrospective study of the clinical outcomes of ACTH combined with magnesium sulfate(MgSO_(4))therapy for IESS in two hospital centers was conducted.The major outcome of the single and combined treatment was evaluated by changes in seizure frequency and improvements in hypsarrhythmia electroencephalography(EEG).To reduce the confounding bias between the two groups,we used SPSS for the propensity score matching(PSM)analysis.Results We initially recruited 1205 IESS patients from two Chinese hospitals and treated them with ACTH combined with MgSO_(4) and ACTH alone.Only 1005 patients were enrolled in the treatment(ACTH combined with MgSO_(4):744,ACTH:261),and both treatment plans had a more than 55% response rate.However,compared to patients treated with ACTH alone,those patients treated with ACTH combined with MgSO_(4) had better performance in terms of the seizure frequency and hypsarrhythmia EEG.After PSM,the two groups also showed significant differences in responder rate[70.8%(95% confidence interval,CI)=66.7%–74.8%)vs.53.8%(95%CI=47.4%–60.2%),P<0.001],seizure frequency(P<0.001)and hypsarrhythmia EEG resolution(P<0.001).Notably,multivariate analysis revealed that the lead time to treatment and the number of antiseizure medications taken before treatment were two factors that may affect the clinical outcome.Patients with less than 3 months of lead time responded to the treatment much better than those with>3 months(P<0.05).In addition,the overall incidence of adverse reactions in the ACTH combined with MgSO_(4) group was much lower than that in the ACTH group(31.4%vs.63.1%,P<0.001).During the treatment,only infection(P=0.045)and hypertension(P=0.025)were significantly different between the two groups,and no baby died.Conclusion Our findings support that ACTH combined with MgSO_(4) is a more effective short-term treatment protocol for patients with IESS than ACTH alone,especially for those patients with short lead times to treatment.
基金supported by grants from the Shenzhen Health and Family Planning Commission(SZSM201812005)the Shenzhen Key Discipline Fund(SZXK033)the Shenzhen Science and Technology Plan Project(JCY20210324135211030).
文摘Background In this study,we aimed to assess the efcacy of surgical treatment in children with drug-refractory infantile epileptic spasms syndrome(IESS)and examine the factors infuencing the post-surgical outcomes.Methods The clinical data of 30 children(18 males and 12 females)with epileptic spasms(ES)who underwent surgery at the Epilepsy Center of Shenzhen Children’s Hospital between June 2018 and June 2020 were retrospectively analyzed.Post-surgical outcomes were evaluated using the Engel Epilepsy Surgery Outcome Scale.Scalp electroencephalography and developmental quotient were assessed preoperatively and postoperatively.Univariate analysis and exact logistic regression analyses were used to identify the factors afecting the postoperative efcacy.Results Of the 30 patients who underwent surgical resection,22(73.3%)achieved Engel’s classⅠ-Ⅱoutcomes.Additionally,motor and cognitive functions improved in 14 patients(46.7%).The development of 12(40%)patients remained at the preoperative development level.The median number of antiseizure medications taken preoperatively was 5.27(range 2-10),which decreased to 1.90(range 0-4)at the last follow-up.Seizure duration,etiology,positive positron emission tomography-magnetic resonance imaging(PET-MRI),surgery type,and lesion location were signifcantly correlated with the postoperative efcacy(P<0.05).Positive PET/MRI fndings and lesion location predicted independently the postoperative outcomes.Permanent impairments of motor or language function were rare,with only two cases reporting hydrocephalus and one reporting hemiplegia.Conclusions Surgery is an efective treatment option for children with IESS.Early referral and comprehensive preoperative evaluation are essential for identifcation of surgically treatable structural lesions.The primary surgically treatable cause is cortical malformation,followed by perinatal brain injury.Hemispheric disconnection is a preferred surgical approach.Positive PET/MRI fndings and lesion location predicted the postoperative outcomes.
基金the National Social Science Foundation of China(No.21BTQ106),the Natural Science Foundation of Beijing(No.7222187),and the Key Project of Innovation Cultivation Fund of the Seventh Medical Center of PLA General Hospital(No.qzx-2023-1)。
文摘Behavioral scoring based on clinical observations remains the gold standard for screening,diagnosing,and evaluating infantile epileptic spasm syndrome(IESS).The accurate identification of seizures is crucial for clinical diagnosis and assessment.In this study,we propose an innovative seizure detection method based on video feature recognition of patient spasms.To capture the temporal characteristics of the spasm behavior presented in the videos effectively,we incorporate asymmetric convolutions and convolution–batch normalization–ReLU(CBR)modules.Specifically within the 3D-ResNet residual blocks,we split the larger convolutional kernels into two asymmetric 3D convolutional kernels.These kernels are connected in series to enhance the ability of the convolutional layers to extract key local features,both horizontally and vertically.In addition,we introduce a 3D convolutional block attention module to enhance the spatial correlations between video frame channels efficiently.To improve the generalization ability,we design a composite loss function that combines cross-entropy loss with triplet loss to balance the classification and similarity requirements.We train and evaluate our method using the PLA IESS-VIDEO dataset,achieving an average seizure recognition accuracy of 90.59%,precision of 90.94%,and recall of 87.64%.To validate its generalization capability further,we conducted external validation using six different patient monitoring videos compared with assessments by six human experts from various medical centers.The final test results demonstrate that our method achieved a recall of 0.6476,surpassing the average level achieved by human experts(0.5595),while attaining a high F1-score of 0.7219.These findings have substantial significance for the long-term assessment of patients with IESS.
