Objective To assess the effectiveness and safety of progabide (PGB) for refractory epilepsy. Methods Randomized controlled trials (RCTs) on PGB treating refractory epilepsy were searched from the following databases as PubMed, EMbase, The Cochrane Library, CNKI, CBM and VIP from the date of their establishment to July 2011. The data of RCTs meeting the inclusive criteria were extracted according to Cochrane methods by two reviewers independently, and after the quality was evaluated and cross-checked, meta-analyses were conducted using RevMan 5.1 software. Results A total of seven studies involving 231 patients were included. The results of Meta-analyses showed that based on the conventional therapy, PGB was ineffective in treating refractory partial epilepsy compared with the placebo (OR=1.76, 95%CI 0.40 to 7.65, P=0.45), but it was superior to the placebo in treating refractory partial and generalized epilepsy (OR=4.46, 95%CI 2.06 to 9.65, P=0.000 1). The main adverse events of PGB were somnolence, dizziness and headache, which were mild and transient, which could turn to normal after reducing the dose of PGB and only a few patients needed to stop taking PGB. Conclusion Current studies shows that progabide may be effective in treating refractory partial and generalized epilepsy, but its effectiveness in treating refractory partial epilepsy is still unknown. The side effects of PGB are mostly mild. For the possibility of moderate selection bias existing in the quality of the included studies which may affect the authenticity of outcomes, so this conclusion still needs to be further proved by conducting more high-quality, large-scale and double-blinded RCTs.
ObjectiveTo investigate the clinical effect of Electro-Cortico-Graphy (ECOG) monitoring on refractory epilepsy caused by double pathology. MethodsA retrospective analysis was performed on 10 patients with refractory epilepsy who underwent surgical treatment in Hunan Brain Hospital from January 2020 to December 2021. The diagnosis of postoperative disease was dual pathology of medial temporal lobe sclerosis (MTS) and focal cortical dysplasia (FCD), and the effect of oral drugs was poor. All patients underwent full preoperative evaluation to determine the scope of excision of epileptogenic lesions. Cortical electrodes were used to monitor the location and scope of epileptic discharge during the operation. Epileptogenic lesions were excised, cortical heat cautery was performed, and then cortical EEG monitoring was performed to adjust the excision strategy. The patients were followed up for 24 to 48 months, and the prognosis was assessed according to the Engel scale. ResultsAmong the 10 patients, 1 patient had acute subdural hemorrhage after surgery, 1 patient had speech and naming disorders, but all of them were recovered at discharge. The other patients had no neurological defects such as intracranial infection, hemiplegia, aphasia, etc. Engel grade I was observed in 9 cases (90%) and Engel grade III was observed in 1 case (10%). ConclusionCortical electrode monitoring is safe and effective for refractory epilepsy caused by double pathological signs.
ObjectiveIn order to evaluate the efficacy, safety and tolerability of adjunctive perampanel in children with refractory epilepsy. MethodsThis study collected medical records of 34 children with refractory epilepsy, who were admitted to Children’s Hospital of Soochow University from January 2020 to January 2021. By comparing the baseline status with the status at 4, 8, 12, 24, 36, and 48 weeks of follow-up, the efficacy and adverse reactions of perampanel were evaluated. ResultsThe mean age of the patients treated with perampanel was 8.1±4.1 years. The male-to-female ratio was 1: 1. After the addition of perampanel, the average responder rate at the 4th, 8th, 12th, 24th, 36th, 48th weeks were 37.5%, 46.7%, 50.0%, 47.4%, 53.8%, 42.9%. The adverse events were reported by 32.4%, and the retention rate was 88.2%. ConclusionsPerampanel has good efficacy, safety and tolerability in the treatment of refractory epilepsy. Moreover, personalized treatment and better baseline seizure control may increase the effectiveness and retention rate of perampanel.
Objective To investigate the compliance of ketogenic diet in children with refractory epilepsy and its correlation with the curative effect, and to provide support and basis for the implementation and improvement of the long-term management of ketogenic diet in this patient population. MethodsA total of 106 children and their families who were followed up on ketogenic diet in the Department of Pediatrics of Fudan University from March 2019 to January 2022 in the Department of Ketogenic Multidisciplinary Treatment (MDT) were selected by convenience sampling method. General data questionnaire and ketogenic compliance questionnaire were used for investigation and follow-up. ResultsThe mean compliance of ketogenic diet in refractory epilepsy children was (13.27±3.68). The compliance scores of ketogenic children with different therapeutic effects and cognitive functions were significantly different. The compliance score was significantly correlated with the therapeutic effect and cognitive level, that is, the higher the therapeutic effect of ketogenic diet in children with higher compliance score, the better the cognitive improvement. ConclusionThe compliance of ketogenic diet in children with refractory epilepsy needs to be further improved. Improving the compliance of ketogenic diet is of great significance for the efficacy of ketogenic diet. Medical staff should actively develop the corresponding intervention program and follow-up management mode to further improve the treatment compliance of children's families, improve the treatment effect and improve the quality of life of children's families.
Objective To investigate the clinicalmanifestations, electroencephalogram (EEG) characteristics, surgical treatment and prognosis of epilepsy secondary to Sturge-Weber syndrome (SWS) in children.Methods The data of 7 children with epilepsysecondaryto Sturge-Weber syndrome who were treated by surgery from May 2015 to May 2020 in our Children's Epilepsy Center were retrospectively reviewed. Their demographic characteristics, seizure forms, results of EEG and cranial imaging investigations, surgical methods, postoperative pathological reports and prognosis during follow-upwere summarized and analyzed. The prognosis were evaluated byEngel classificationat the last time point during follow-up. Results Totally 7 pediatric patients were enrolled, including 1 male (16/25, 64.0%) and 6 females.All the 7 cases presented with focal seizures at the onset among whom 2 cases developed status epilepticus during the course of the disease(epilepsiapartialiscontinuain 1 case),one case had epileptic spasmsand 1 case was characterized by cluster seizures. The interictal EEG manifestations of the cases gradually deteriorated as the course of the disease prolonged, including the slow wave on the affected side gradually increases (7/7), the amplitude gradually decreases (7/7), and the physiological wave disappears (4/7). Besides, no epileptiform discharges/incidental or a few epileptiform dischargeswere found in their interictal EEGs. Four cases underwent hemispherotomy, 1 case underwent temporo-parieto-occipital disconnection and 2 cases underwent lesion resection. The cases were followed up for 6 months to 5 years, and the average follow-up duration was 79.29 months. Six cases were rated as Engel Ⅰa during the regularfollow-up. Only 1 case was rated asEngel Ⅱ and Engel Ⅲ at 3 month and 1 year after the operation. ConclusionChildren with epilepsy secondary to SWS usually present with focal seizures and have diverse seizure forms. The EEG show characteristic changes. For the caseswith drug refractory epilepsy, detailed preoperative evaluation and reasonable surgical methods can result in a better therapeutic effect.
Objective To study the efficacy and adverse events of adjunctive perampanel in children with refractory epilepsy. Methods A prospective study was carried out in 45 children with refractory epilepsy, who were treated in our hospital from January 2020 to February 2021 using perampanel as an add-on treatment, with a criteria for enrollment and the starting dose of perampanel. Follow-up would be taken at once a month. Afte 3 months would check blood routine, liver function, kidney function and humoral immunity. The EEG was reviewed after 6 months. The initial dose of perampanel was 0.04 mg/(kg·d) (the maximum didn't exceed 2 mg/d), increasing by 0.04 mg/(kg·d) every two weeks, and the maximum maintenance dose didn't exceed 6 mg/d. The efficacy and adverse reactions of perampanel were evaluated by comparing the seizure frequency and EEG results before and after a 6-month add-on therapy.ResultsAmong the 45 children,complete seizure control was achieved in 7 cases after the therapy, and the seizure attacks were reduced in 26 cases, showing a total response rate of 73.3%. After the treatment, the epileptiform discharge of 28 children was reduced, and the effective rate was 62.22%. During the observation period, all the blood routine, liver function, kidney function,and humoral immunity of the children were normal.10 cases of adverse reactions occurred after the additional treatment of perampanel, and the adverse reaction rate was 22.22%. Conclusions Perampanel has good efficacy and safety in the add-on treatment of refractory epilepsy.
ObjectiveTo observe the clinical efficacy of Xiao’er kang xian capsule added to anti-seizure medications (ASMs) in the treatment of children with refractory epilepsy and its influence on serum neuron-specific enolase (NSE) and cludter of differentiation 19+ (CD19+) levels. Methods A total of 60 children with refractory epilepsy were selected from the pediatric outpatient department and ward of Guangdong Provincial People's Hospital from February 2021 to June 2023. The study subjects were divided into two groups by numerical random method,with 30 cases in each group. The children with Xiao’er kang xian capsule added to the original treatment were the treatment group and the children without Xiao’er kang xian capsule added to the original treatment were the control group. The frequency, duration, EEG characteristics, adverse reactions and changes in serum NSE and CD19+ levels of the two groups were compared after treatment. Results Self-control before and after treatment in the treatment group: the frequency and duration of seizures were significantly reduced, with statistical difference (P<0.05). EEG discharge index in awake period and sleep period were significantly decreased, with statistical difference (P<0.05). After 6 months of treatment, comparison between the two groups of children: the seizure frequency of children in the treatment group was significantly decreased compared with the control group (P=0.03). There was a statistical difference (P<0.05), and the seizure duration in the treatment group was less than that in the control group (P=0.863), the clinical effective rate of treatment group 83.33% was higher than that of control group 63.33% (P=0.08), the effective rate of EEG in treatment group 80% was higher than that of control group 60% (P=0.091), serum NSE and CD19+ in treatment group were lower than that of control group, with no statistical difference (P>0.05). After 12 months of treatment, the frequency and duration of seizures in the treatment group were significantly decreased (P<0.05). The clinical efficacy and effectiveness of treatment group were significantly higher than that of control group (P=0.038). The incidence of adverse reactions in both groups was 16.67% (P>0.05). The effective rate of EEG in treatment group was significantly higher than that in control group (P=0.053). Serum NSE and CD19+ in treatment group were significantly lower than those in control group (P<0.05). ConclusionFor children with refractory epilepsy, the addition of Xiao’er kang xian capsule on the basis of the original treatment has obvious effect low adverse reaction and high safety. NSE and CD19+ can be used as monitoring indicators for the influence of the disease and prognosis evaluation during the treatment of children with epilepsy.
ObjectiveTuberous sclerosis complex (TSC) is a multisystem disease, which often manifests as refractory epilepsy in the nervous system and multifocality in Magnetic resonance imaging (MRI). We summarized patients with TSC whose peripheral blood gene test was negative,and analyzed their medical history, EEG, MRI and postoperative conditions. MethodsWe summarized and analyzed 205 patients with TSC diagnosed clinically and pathologically and underwent surgery from April 2008 to February 2024. 11 patients with TSC whose peripheral blood gene test was negative and underwent surgery were screened out.All patients underwent gene examination, MRI and long-range video EEG monitoring. All patients underwent detailed preoperative evaluation and direct resection surgery, intracranial electrode thermocoagulation surgery or laser surgery. ResultsOf the 11 patients with peripheral blood gene test negative, 11 (100%) patients achieved Engel Ⅰ within 1 year after surgery, and 10 (91%) patients achieved Engel Ⅰwithin 2 years. The median age of onset of 11 patients was 6 months, and 8 patients (73%) had onset less than 1 year old. All patients had multiple nodules in the brain. Except for the brain, the changes of the other organs were polycystic kidney and skin changes in 2 patients, skin changes in 8 patients, and normal in 1 patients. ConclusionTSC patients with peripheral blood gene negative who have young onset age, frequent seizures, and multiple nodules on MRI often present with refractory epilepsy. However, the postoperative effect is good, and the postoperative EEG of most patients is normal.
ObjectiveTo analyze the clinical efficacy and safety of rapamycin in the treatment of Tuberous sclerosis complex ( TSC ) complicated with refractory epilepsy, and to provide scientific basis for the clinical treatment of this disease.MethodsRetrospective analysis was performed on 22 children with TSC complicated with refractory epilepsy admitted to Henan People's Hospital from 2017 to 2019, including 11 males and 11 females who met the inclusion criteria, with an average age of (27.91±36.92) months. They were treated with antiepileptic drugs and rapamycin at the same time, and followed up for at least 1 year.To observe the change of seizure frequency before and after treatment with rapamycin.ResultsThe mean reduction rate of seizure frequency in children with tuberous sclerosis complicated with refractory epilepsy was 52.1% 6 months after the addition of rapamycin, and 51.2% 12 months after the addition of rapamycin. The number of seizure-free days could be maintained. The difference before and after the addition of rapamycin was statistically significant (P<0.05).ConclusionThe addition of rapamycin in the treatment of TSC complicated with refractory epilepsy can reduce the frequency of seizure and increase the number of days without seizure, and the adverse reactions are mild/moderate. Rapamycin has certain safety in children with regular follow-up.
Objective To assess the effectiveness and safety of flunarizine for refractory epilepsy. Methods Relevant randomized controlled trials (RCTs) were searched from the database of PubMed, EMbase, Cochrane Library, CNKI, CBM, and VIP, and the related references were traced to obtain the information. The methodological quality of included RCTs was assessed using Jadad scale and meta-analysis was performed using RevMan 5.0 software. Results A total of eight studies involving 545 patients were included. The results of meta-analyses showed that: based on the conventional therapy, compared with placebo and none-treatment, flunarizine was more effective on adults and children with refractory epilepsy (OR=2.98, 95%CI 1.88 to -4.73; OR=33.75, 95%CI 4.13 to -276.00). Major adverse events of flunarizine were fatigue, dizziness, headache, and weight gain etc. All those symptoms except for the weight gain were observed in the early stage of medication, which might get self-cured or could disappear by constant medication or reducing the dose or symptomatic treatment. Conclusion The present study shows that based on the conventional therapy, flunarizine is effective and safe for refractory epilepsy.
