ObjectiveTo investigate the effect of medical counseling games on ketogenic diet therapy for drug-resistant epilepsy children. MethodsA total of 98 children with drug-resistant epilepsy admitted to the neurology ward of Shenzhen Children's Hospital from January 2023 to June 2024 who were treated with ketogenic diet for the first time were selected as the study objects by random number table method, and were divided into observation group (n=49) and control group (n=49). The control group received the traditional multidisciplinary team health education mode, while the observation group received the ketogenic diet treatment based on the multidisciplinary team health education mode and participated in the customized medical counseling games intervention. The time of children reaching ketosis, the knowledge level of ketogenic diet caregivers and the retention rate of children on ketogenic diet were compared between the two groups. ResultsThe time of ketosis in observation group was earlier than that in control group (P<0.05). The knowledge level of the main caregivers of ketogenic diet and the retention rate of children with ketogenic diet at 3 months and 6 months in observation group were higher than those in control group (P<0.05). ConclusionThe use of medical counseling games in the ketogenic diet for medically refractory epilepsy is an effective therapeutic strategy that facilitates the early attainment of ketosis in children with medically refractory epilepsy, improves the knowledge of caregivers on the ketogenic diet, improves retention of children on the ketogenic diet, and serves to optimize the effectiveness of clinical outcomes, which may contribute to the quality of life of children with medically refractory epilepsy.
ObjectiveTo improve the knowledge of a rare disease named pyridoxine-dependent epilepsy.MethodsHigh-throughput sequencing and Sanger sequencing were used to validate the genes of epilepsy. Mutation gene validation was performed on two probands and their parents. Analyze clinical manifestations, electroencephalogram (EEG), imaging and prognostic features of the two probands.ResultsProbands 1, seizure onset at 4 months, progress as drug-refractory epilepsy, manifested as seizures types origin of multi-focal lesions. Head MRI and fluorodeoxyglucose-positron-based tomography (FDG-PET) were both normal. Gene detection showed that Aldehydedehydrogenase (ALDH7A1) gene has a complex heterozygous mutation contain c.1442G> and c.1046C> T.Proband 2, seizure onset at 5 months, manifested as a tonic-clonic seizure. Intermittent EEG and head MRI were both normal. Genotyping revealed ALDH7A1 gene contain a complex heterozygous mutation c.1547A> G and c.965C> T. Two cases were both seizure free by vitamin B6 therapy and gradually reduce the antiepileptic drugs.ConclusionsPyridoxine-dependent epilepsy may be late onset, some patient can be atypical and early experimental treatment can help to identify and the diagnosis should be confirmed by gene test.
ObjectiveTo evaluate the clinical efficacy and safety of magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) for mesial temporal lobe epilepsy (MTLE), and to compare its outcomes with anterior temporal lobectomy (ATL). MethodsA retrospective cohort of 120 MTLE patients treated at Beijing Tiantan Hospital between August 2022 and August 2024 was analyzed, including 31 patients who underwent MRgLITT and 89 patients who underwent ATL. All patients received comprehensive presurgical evaluations, and stereoelectroencephalography (SEEG) was performed in selected cases. Clinical outcomes at 1 year were compared between the two groups, including seizure control (Engel classification, seizure reduction rate), cognitive and memory changes, quality of life, and postoperative complications. ResultsBaseline characteristics were comparable between groups. At 1-year follow-up, Engel class I outcomes were achieved in 71.0% of patients in the MRgLITT group and 67.4% in the ATL group. Seizure reduction rates were (89.6 ± 26.2)% for MRgLITT and (87.0 ± 28.7)% for ATL, with no significant difference (P=0.92). Postoperative changes in memory, cognition, and quality of life were not significantly different between groups (all P>0.05). The incidence of complications was low and similar between MRgLITT and ATL, including hemorrhage (3.2% vs. 2.2%), infection (16.1% vs. 19.1%), and neurological deficits (3.2% vs. 2.2%). ConclusionMRgLITT provides seizure control and safety outcomes comparable to ATL when applied to carefully selected MTLE patients, with the added advantages of minimal invasiveness and faster recovery. For patients with well-localized epileptogenic foci and hippocampal sclerosis, MRgLITT represents an important alternative to open resection.
ObjectiveTo analyze the effect of magnetic resonance-guided laser interstitial thermal therapy (Magnetic resonance-guided laser interstitial thermal therapy , MRgLITT) for drug resistant epilepsy (DRE). MethodsThe present study analyzed the clinical information of DRE patients treated by MRgLITT in Beijing Tiantan Hospital from August 2020 to February 2021, including the type of disease, postoperative complications, and prognosis (Engel classification) in the one year after surgery. ResultsA total of 55 patients were enrolled. There were 27 males and 28 females, with an average of (21.7±14.1) years, all of whom successfully completed the operation and were followed up for the 1 year after surgery. The diagnosis included intracranial tumors, hypothalamic hamartoma (HH), focal cortical dysplasia (FCD), cavernous malformations (CM), mesial temporal lobe epilepsy (mTLE), and idiopathic generalized epilepsy (underwent corpus callosotomy). The patients with seizure freedom accounted for 59.6% (31/52), and the average remission rate of palliative surgery was 68.6%. The short-term postoperative complications included bleeding in neurological deficit in 6 cases (10.9%), 4 cases (7.3%), and noninfectious fever in 2 cases (3.6%). No serious, long-term complications occurred. The average postoperative hospital stay was (4.7±1.6) days. ConclusionsMRgLITT is gradually mature and has a wide range of indications. This technology provides a safe and effective therapy for DRE patients.
Objective To investigate the task group’s effectiveness in language evaluation based on the task group's functional Magnetic resonance (fMRI) results’ agreement with the fixation side of the Wada language area. MethodsWe collected 90 patients with intractable epilepsy of 90 patients from December 13, 2018 to January 3, 2020 from the Epilepsy Center of Guangdong 999 Brain Hospital. We used two simple fMRI tasks. Among them, 25 patients completed the Wada experimental examination, and 8 patients completed the electrode implantation and subsequent preoperative language area mapping. Adopt block experimental design, ABBA style presentation, and use AFNI software to process fMRI data, lateralization index calculation, and multiple regression analysis. ResultsfMRI results from 90 patients showed that the results from both the sentence-completion task and the image-naming task were more stable than those from either task. The results were then compared with the results of the “gold standard” Wada test in 25 patients with fMRI-located language dominance in the hemisphere. The results showed that the accuracy of the single task was between 70% and 80%, but the accuracy of the combined results of the two tasks was 93.3%. Conclusions Compared with the results of a single task, the results of multiple fMRI tasks are more stable in the judgment of activation range and language dominance hemisphere. fMRI and Wada language area siding accuracy 93.3%, fMRI task siding valid and replicable.
With the progress of genetic testing technology, a large proportion of genes associated with epilepsy are revealed. And these genes are often linked to drug-resistant epilepsy (DRE), for which surgical treatment can be an important intervention. This article reviews the progress of surgical treatment for gene-related epilepsy and focuses on influence factors of surgical outcomes, including timing of surgery, extent of lesion resection, and preoperative assessment methods. Through early surgical intervention, some patients with specific gene mutations (eg. TSC), can significantly improve neural development and control epilepsy greatly while some patients with other gene mutations (eg. SCN1A) have less favorable outcomes. Individualized preoperative assessment, accurate localization of the epileptogenic zone, and complete resection of the lesion are key to good prognosis. Although current studies are mostly based on small sample sizes, the existing evidence provides a theoretical foundation for the advancement of epilepsy surgery toward more precise approaches.
Surgical removal or destruction of the focal brain area is the main treatment for drug-resistant epilepsy, but it is not suitable for all patients. Epileptiologists in the United States have opted for a new type of palliative therapy called responsive neurostimulation (RNS). The RNS system continuously monitors the electrical activity of the brain in the area of possible seizures and places electrodes in the epileptic area to provide electrical stimulation when abnormal discharges are detected, stopping seizures. Controlled clinical trials have demonstrated the long-term effectiveness and safety of the RNS system, with continued improvement in seizure reduction rates over time. RNS system not only has a good effect on temporal lobe epilepsy and cortical functional area epilepsy, but also can dynamically monitor cortical EEG, so as to better understand the epilepsy status of each patient and provide personalized diagnosis and treatment. In this paper, the development history, structure, advantages and disadvantages of RNS system are reviewed, and its indications as palliative treatment for drug-resistant epilepsy are discussed.
ObjectiveTo explore the prognostic factors for seizure control in focal cortical dysplasia(FCD)by analyzing the clinical features of FCD patients. MethodsWe conducted a follow-up study of patients, who were confirmed FCD by pathology after resective surgery,in Epileptic Center, Guangdong Sanjiu Brain Hospital, From January 1, 2014 to December 31, 2014. All patients were followed at least 6 months,they were divided into seizure control group(Engel class I) and seizure group(Engel classⅡ-class Ⅳ) according to surgical outcomes. Clinical features,auxiliary examinations and pathological classification were compared between two groups. Results102 patients were included, male 65 cases (63.7%), female 37 cases (36.3%), onset age 0.01~45 years old, average (10.3±8.26) years old, surgery age (3~47) years old, average (21.21±8.9) years old, all had seizure onset. 83 (81.4%) patients in seizure control group, 19 (18.6%) patients in seizure group. There are 14.5% of the patients' onset ages are younger than 3 years old, 59.8% preoperative electroencephalogram recording a diffusion epileptiform discharge, 32.5% orientation of magnetic resonance imaging (MRI) and electroencephalography (EEG) is inconsistent, 49.4% postoperative electroencephalogram (EEG) reveal an epileptiform discharge, 45.2% of the patients had intellectual disability, 36.1% had an absence of a lesion on MRI, in seizure control group. However,in seizure group they respectively 36.8%, 72.2%, 89.5%, 68.4%,94.1%, 89.5%. Patients in seizure control group got an average scores of (89.4±18.53) in performance intelligence quotient (PIQ)test, while, seizure group 65.80±15.71.There has a statistical significance between two groups. ConclusionPostoperative seizure outcome was favorable in patients with FCD, onset ages younger 3 years old, intellectual disability,getting a lower scores in PIQ test, preoperative electroencephalogram recording a diffusion epileptic discharge, inconsistent orientation of MRI and EEG, and postoperative EEG reveal an epileptiform discharge may be predictive for the postoperative outcome.
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.