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.
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 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.
Ketogenic diet (KD) is one of the effective treatments for refractory epilepsy (RE) and is recommended when anti-seizure medications (ASMs) are ineffective or less effective, inoperable or ineffective. The efficacy of the medium-chain triglyceride (MCT) ketogenic diet is as good as the classical KD (CKD), which has been demonstrated in several retrospective, prospective, and randomized studies, and MCT is more ketogenic than long-chain triglycerides, so MCTD allows more carbohydrate and protein foods, which makes MCTD more palatable than CKD more palatable. Research advances in the mechanisms and clinical efficacy associated with MCTD in the treatment of refractory epilepsy are reviewed.
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 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.
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.
Objectives To investigate the changes of serum monoamine neurotransmitters and myocardial enzymes in patients with refractory epilepsy (RE), and the possible effects on the cardiovascular system, which would contribute to provide help and guidance to the early warming and prevention to the sudden unexpected death in epilepsy (SUDEP). Methods We collected sixty patients with RE who admitted to Neurological department of First Hospital of Jilin University from December 2015 to December 2016. According to the exclusion criteria, we selected thirty-two patients into the study. The study included 21 males and 11 females patients. Epinephrine (EPI), norepinephrine (NE), dopamine (DA), 5-hydroxytryptamine (5-HT), creatine kinase isoenzyme (CKMB), lactate dehydrogenase (LDH) and hydroxybutyrate dehydrogenase (HBDH) were measured in peri-ictal period and the interictal period in the patients. All the data were analyzed by SPSS17.0 statistical software. Results ① Thirty two patients were eligiblefor this study and the maleto female ratio is 21:11; The age ranged from 15 to 85 years old, with the average age of 50.9±17.6 years old. Twelve (37.5%) were older than 60 years old and 20 (62.5%) were under 60 years old. The epilepsy history ranged from 1 year to 14 years, with an average of 3.75±3.12 years; ② Comparing the levels of monoamine neurotransmitters in peri-ictal period and the interictal period in the patients with RE, we found that the level of EPI and LDH was significantly lower than that in interictal period, while the levels of NE and DA were significantly increased; ③ The results showed that EPI, NE and DA levels in patients under 60 were higher than over 60; ④ Patients were divided into four groups according to the etiology of the disease: idiopathic epilepsy group (10 cases, 31.25%), post-encephalitic epilepsy group (7 cases, 21.88%), post-stroke epilepsy group (9 cases, 28.12%) and epilepsy after brain injury group (6 cases, 18.75%). The results showed that the levels of EPI, NE and DA in the post-strokeepilepsy group were significantly lower than those in the other three groups. The level of CKMB in the idiopathic epilepsy group was higher than that in post-stroke epilepsy and epilepsy induced by brain injury patients. Conclusions RE patients have a higher level of serum NE and DA interictal period, suggesting that seizures may increase sympathetic nervous excitability. The patients under 60 years-old with RE release more catecholamines than young patients, suggesting that the latterwith intractable epilepsy may have higher sympathetic nerve excitability. And it may be associated with the higher incidence of SUDEP in young patients. Post-stroke epilepsyrelease less catecholamine than others, suggesting that the sympathetic nervous excitability is relatively low, and it may have relatively little damage to heart.
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.
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.