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 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.
ObjectiveAccording to the diagnosis and treatment analysis of 5 children with Febrile infection-related epilepsy syndrome (FIRES) and literature review, to explore the therapeutic effect of Ketogenic diet (KD).MethodsA retrospective analysis of the clinical status of 5 children with KD treatment of FIRES admitted to the Department of Pediatric Neurology, West China Second Hospital of Sichuan University from August 2016 to September 2019, combined with literature data, summarized their disease characteristics, prognosis and KD treatment effects.ResultsThe 5 cases of FIRES children were (5.8±2.0) years old and had a male to female ratio of 2∶3. They were all induced by fever followed by a status epilepticus that was difficult to control with drugs. The interval between fever and first seizure was 4 to 7 days, and the prodromal symptoms were higher respiratory tract infections, dizziness, vomiting, fatigue, listlessness, loss of appetite, etc., convulsions manifested as focal or focal secondary systemic or general seizures, EEG showed slowing background rhythm, changes in multifocal epileptic discharge, early course of disease The cranial imaging examination was basically normal. As the course of the disease progressed, changes in brain atrophy gradually appeared, and abnormal signal shadows were seen in the forehead, parietal, occipital lobe, and periventricular. A variety of anti-epileptic drugs, hormones, gamma globulin, plasma exchange and other treatments have poor therapeutic effects and severe cognitive impairment. The KD treatment started to take effect within 2 weeks, and the convulsions were reduced. One case was completely controlled, and the cognitive function basically returned to normal with only mild learning disabilities; the convulsions were reduced by more than 50% in 2 cases, leaving mild to moderate cognitive impairment, The other 2 cases had poor long-term treatment effects, left intractable seizures and severe mental retardation.ConclusionFIRES is a serious epileptic encephalopathy, most of which leave severe cognitive impairment and refractory seizures. Drug therapy and prognosis are poor. KD treatment is beneficial to control seizures in children with FIRES in the acute stage.
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.
ObjectiveAnalyze and compare the differences in the efficacy and adverse reactions of various ketogenic diet (KD) in the treatment of refractory epilepsy in children.MethodsSystematic search of electronic databases, including PubMed, Embase, Ovid MEDLINE, Web of Science and the Central Register of Cochrane Controlled Trials, published in English January 2000 Relevant research from January to August 2020. Results: Finally, 11 articles were included and 781 cases were included. Meta-analysis (NMA) method was used to compare 6 classic ketogenic diets (Classic ketogenic diet, CKD), Gradual ketogenic diet initiation (GRAD-KD), and the first modified Atkins diet of 20 g carbohydrates/d (Initial 20 g of carbohydrate/day of modified Atkins diet, IMAD), modified Atkins diet (MAD), low glycemic index diet (LGID) and medium-chain fatty acid diet (Medium-chain triglyceride diet, MCT) Therapeutic effect and adverse reactions of 3, 6, and 12 months.ResultsFrom the results of the direct comparative analysis, CKD and MAD showed superior clinical efficacy in 50% seizure reduction at 3 months to CAU, and the difference was statistically significant [OR=10.58, 95%CI (3.47, 32.40), P<0.05; OR=11.31, 95%CI (5.04, 25.38), P<0.05]; the clinical efficacy of 90% seizure reduction at 3 months for MAD was superior to that of CAU with statistical significance [OR=4.95, 95%CI (1.90, 12.88), P<0.05]. The results of further network meta-analysis suggested that for the comparison of 50% seizure reduction at 3 months, IMAD, GRAD-KD, CKD, MAD, and MCT were superior to CAU, and the difference was statistically significant [OR=0.03; 95%CI (0.00, 0.30), P<0.05; OR=0.07; 95%CI (0.01, 0.76), P<0.05; OR=0.11; 95%CI (0.03, 0.35), P<0.05; OR=0.11; 95%CI (0.04, 0.35), P<0.05; OR=0.13; 95%CI (0.03, 0.67), P<0.05; OR=0.11; 95%CI (0.03, 0.35), P<0.05; OR=0.11; 95%CI (0.04, 0.35), P<0.05]. For the comparison of 90% seizure reduction at 3 months, CKD, GRAD-CK, IMAD, MAD, and MCT were superior to CAU, and the differences were statistically significant [OR=0.05; 95%CI (0.00, 0.31), P<0.05; OR=0.22; 95%CI (0.00, 0.39), P<0.05; OR=0.03; 95%CI (0.00, 0.62), P<0.05; OR=0.12; 95%CI (0.01, 0.60), P<0.05; OR=0.09; 95%CI (0.00, 0.91), P<0.05]. It is suggested in the cumulative probability plot that: the optimal clinical regimen for 50% seizure reduction at 3 months was IMAD (Rank1=0.91), the optimal clinical regimen for 50% seizure reduction at 6 months was CKD (Rank1=0.40), the optimal clinical regimen for 50% seizure reduction at 12 months was MCT (Rank1=0.64); the optimal clinical regimen for 90% seizure reduction at 3 months was IMAD (Rank1=0.94), the optimal clinical regimen for 90% seizure reduction at 6 months was LGIT (Rank1=0.44), and the optimal clinical regimen for 90% seizure reduction at 12 months was MCT (Rank1=0.41); the optimal clinical regimen for seizure reduction at 3 months was GRAD-CK (Rank1=0.46), the optimal clinical regimen for seizure reduction at 6 months was LGIT (Rank1=0.58), and the optimal clinical regimen for seizure reduction at 12 months was CKD (Rank1=0.56). It is suggested in the benefit-risk assessment that among the three KDs (CKD, MAD, MCT) with better 50% and 90% seizure reduction at 3 months and 6 months, combining with the incidence of adverse reactions, CKD was the optimal treatment regimen (CF=0.47, CF=0.86); among the two KDs (CKD, MAD) with better seizure reduction at 3 months and 6 months, combining with the incidence of adverse reactions, CKD was the optimal treatment regimen (CF=0.45); among the two KDs (CKD, MCT) with better 50% and 90% seizure reduction at 12 months, combining with the incidence of adverse reactions, CKD was the optimal treatment regimen (CF=0.65).ConclusionsIn this study, IMAD showed the optimal clinical efficacy at 3 months and MCT at 12 months. With stable efficacy and low incidence of adverse reactions in 12 months, CKD was the optimal treatment regimen for children with refractory epilepsy after the comprehensive evaluation.
ObjectiveTo investigate the efficacy of vagus nerve stimulation (VNS) in patients with refractory magnetic resonance imaging (MRI)-negative epilepsy and to evaluate potential clinical predictors. MethodsA retrospective collection of efficacy data was conducted on 24 patients with intractable MRI-negative epilepsy treated with VNS, who were followed up for more than six months, at Beijing Tiantan Hospital and Beijing Fengtai Hospital from January 2016 to September 2023. Patients were divided into two subgroups based on their response to VNS: responders (≥50% reduction in seizure frequency) and non-responders (<50% reduction in seizure frequency). The relationship between preoperative clinical data and VNS efficacy was further analyzed to identify potential predictors of VNS efficacy. ResultsA total of 24 patients were included, with an average age of (14.26±8.39) years old. Seizure frequency was reduced by more than 50% in 37.5% of patients, and 8.3% of patients achieved seizure-free after VNS treatment. Preoperative seizure frequency and interictal epileptiform discharge type were significantly associated with VNS efficacy (P<0.05). Multivariate regression analysis showed that a monthly seizure frequency of less than 100 and focal interictal epileptiform discharges were independent predictors of VNS efficacy (P<0.05). ConclusionVNS is an effective treatment for patients with refractory MRI-negative epilepsy. Lower monthly seizure frequency and focal interictal epileptiform discharges are potential predictors of VNS efficacy. These findings provide important references for clinicians in selecting and evaluating patients for VNS treatment.
Objective To observe the efficacy and adverse reactions of pirenpanet in patients with refractory epilepsy treated with add-on therapy. MethodsForty-one patients with medically refractory epilepsy were selected from January 2021 to December 2021 in the Department of Epilepsy of Jiamusi Central Hospital, among which 25 males and 16 females. The self-control study was conducted before and after the treatment with pirenpanet. The observation period was 1 year, and the clinical efficacy, platelet, liver and kidney function and EEG discharge index were observed at 6 months and 12 months respectively. Changes in seizure frequency after treatment were divided into the following 3 groups: effective control: no seizure; effective: seizures reduced by 50% ~ 99%; Ineffective: < 50% reduction in seizures or exacerbation. Results Among the 41 patients with drug-refractory epilepsy, 8 (8/41, 19.51% P < 0.05) were seizure-free, 17 (17/41, 41.46% P < 0.05) were effective, and the total effective rate was (25/41, 60.98%); Sixteen cases (16/41, 39.02%) were ineffective. There were 31 children in 41 cases (31/41, 75.61%), of which 18 cases (18/31, 58.06%) were effective, 16 cases (16/31, 51.61%) had mental and motor retardation, and 8 cases (8/16, 50%) were effective; There were 10 adults (10/41, 24.39%) and 7 effective patients (7/10, 70%). After one year's treatment, the EEG changes of the patients were observed, and the abnormal discharge of 6 patients was reduced according to the discharge index, 2 patients had no abnormal discharge, and the other patients had no obvious EEG changes. Conclusion Pirenpanet is effective and safe as an add-on therapy for medically refractory epilepsy.
ObjectiveTo investigate the association between mTOR pathway and pharmacoresistance of Sprague-Dawley rat epilepsy model kindled by coriaria lactone. MethodsA kindling model of pharmacoresistant temporal lobe epilepsy was developed by injecting Sprague-Dawley (SD) rats with coriaria lactone (CL) (1.75 mg/kg, every 84 h). Normal SD rats were injected with normal sodium (NS) served as control group. Rats with five or more consecutive stage 5 seizures were included in kindled group. Immunohistochemistry was used to detect the levels of P-S6 in both groups. ResultsThe expressions of P-S6 in CA1 and CA3 were significantly higher compared with control group, and were mainly in astrocytes (P < 0.001). In addition, the expression of P-S6 in DG area was significantly higher than that in control group, with more granular cell and neuron (P < 0.001). ConclusionsThe mTOR pathway may be correlated with the drug resistance of refractory lobe epilepsy kindled by coriaria lactone.
ObjectiveTo observe and analyze the efficacy and adverse reactions of Lacosamide (LCM) in the treatment of refractory epilepsy in children and adolescents. MethodsA retrospective cohort study was conducted on 85 patients with refractory epilepsy, with 50 males and 35 females, aged 0.5 ~ 15 years with an average age of (6.90±3.61) years, who were treated in the Department of Neurology of Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, from January 2020 to March 2023. A self-controlled study was conducted by oral LCM add on treatment, and follow-up was performed to compare and observe the efficacy as well as the adverse reactions before and after the use of LCM. ResultsBy self-control, after 12 months of follow-up after addition of LCM treatment, compared with baseline, the frequency of seizures decreased after 3, 6 and 12 months of treatment, the differences were statistically significant (P<0.05), and the effective rate of analysis after 3, 6 and 12 months of addition of treatment were 36.47%, 42.35% and 41.18%, respectively. There were 22 cases without seizure after 12 months of LCM treatment, and the seizure-free rate was 25.88%. Enrolled patients used a variety of antiseizure medications at baseline, and the three drugs used by the most patients were sodium valproate in 54 cases (63.53%), levetiracetam in 41 cases (48.24%) and oxcarbazepine in 24 cases (28.24%) respectively. After addition of LCM, a total of 10 cases experienced adverse reactions, such as dizziness, headache, nausea, etc. The incidence of adverse reactions was 11.76%. The retention rate at 12 months after adding LCM was 63.5%. ConclusionsThe addition of LCM in the treatment of refractory epilepsy in children and adolescents can effectively improve the frequency of seizures, with fewer adverse reactions and higher retention rates.
ObjectiveTo investigate the efficacy of vagus nerve stimulation (VNS) in the treatment of refractory epilepsy in children and the key factors affecting its efficacy. MethodsThe clinical data of 22 children with drug-resistant epilepsy who received VNS treatment in the Second People's Hospital of Hunan Province from January 2016 to April 2023 were analyzed. The average seizure reduction rate, effective rate and McHugh grade were used to evaluate the efficacy of VNS after at least 1 year follow-up. Patients with an attack reduction rate of ≥50% were defined as respondents, and Mann-Witney U test and χ2 test were used, respectively, to conduct univariate and multifactor Logistics regression analysis with statistically significant indicators (P<0.05). ResultsAmong the 22 patients, the average attack reduction rate was 12.66% at 1 month, 26.10% at 3 months, 37.47% at 6 months, 48.18% at 9 months and 54.38% at 12 months. The effective rate was 5.00%, 9.00%, 36.00%, 50.00% and 68.00%, respectively. 12 months after operation, there were 3 cases of grade I, 12 cases of grade II, 7 cases of grade III, and 0 cases of grade V. Unifactorial and multivariate Logistic regression analysis showed that the curative effect of epilepsy in children was related to the seizure type, among which the curative effect of general seizure was better than that of focal seizure (OR=0.062, P=0.014), and the curative effect of myoclonic seizure and tonic seizure was better than that of other types in general seizure. ConclusionThe clinical effect of VNS in the treatment of refractory epilepsy in children is time cumulative, and the surgical effect of myoclonic seizures and tonic seizures in general seizures is better.