In the treatment of drug-refractory epilepsy in children, surgical treatment has a good clinical effect. However, for children whose surgical site is difficult to determine and who cannot undergo resectional surgery, neuromodulation techniques are one of the treatments that can be considered. At present, new neuromodulation technologies in children mainly include transcutaneous vagus nerve stimulation (transcutaneous auricular vagus nerve stimulation, ta-VNS), deep brain stimulation (deep brain stimulation, DBS), reactive nerve stimulation (responsive neurostimulation, RNS), transcranial magnetic stimulation (transcranial magnetic stimulation, TMS), transcranial direct current stimulation (transcranial direct current stimulation, TDCS) and transcranial alternating current stimulation (transcranial alternating current stimulation, TACS). This article briefly discussed the clinical efficacy and safety of various currently available neuromodulation technologies, so as to provide a reference for the rational selection and application of neuromodulation technologies, and improve the clinical efficacy and quality of life of children with drug-refractory epilepsy.
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.
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.
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.
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.
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.
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.
ObjectiveTo understand the relationship between the anatomy and the function of the insula lobe cortex based on the stereo-electro encephalography (SEEG) by direct electric stimulation of the insula cortex performed in the patients who suffered from the refractory epilepsy. MethodsRetrospective review was performed on 12 individuals with refractory epilepsy who were diagnosed in the Department of Functional neurosurgery of RenJi Hospital from December 2013 to September 2015. We studied all the SEEG electrodes implanted in the brain with contacts in the insula cortex. Direct electric stimulation was given to gain the brain mapping of the insula. Results12 consecutive patients with refractory epilepsy were implanted SEEG electrodes into the insula cortex. In all, 176 contacts were in the insula cortex, and 154 were included. The main clinical manifestations obtained by the stimulation were somatosensory abnormalities, laryngeal constriction, dyspnea, nausea, flustered. While somatosensory symptoms were located in the posterior insula, visceral sensory symptoms distribute relatively in the anterior insula, and other symptoms were mainly in the central and anterior part. ConclusionsThe symptoms of the insula present mainly according to the anatomy, but some of them are mixed. In addition, the manifestations of the insula are usually complex and individually.
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.