【摘要】 目的 探讨用视频脑电图和MRI诊断药物难治性癫痫的临床价值。 方法 收集2006年12月-2010年5月间经手术和病理证实的药物难治性癫痫患者38例。其中，海马硬化25例，颞叶萎缩伴脑发育不良2例，脑灰质移位及巨脑回4例，血管畸形3例，胶质瘤2例，脑内囊肿1例，外伤性癫痫1例。用视频脑电图监测癫痫发作期及发作间期痫样放电的来源部位及脑电活动特点，用MRI扫描显示痫灶区的表现特征，并与手术、病理改变对照，进行回顾性分析。 结果 视频脑电图对癫痫发作期的致痫灶来源定位准确率为100%（38/38），发作间期定位准确率为53%（20/38）。MRI对发作间期的致痫灶及相关病变定位诊断准确率为89%（34/38），病变定性准确率为79%（30/38）。 结论 视频脑电图和MRI检查有机结合，对药物难治性癫痫，能更有效检出致痫灶的部位及性质，为药物难治性癫痫患者的手术治疗，提供重要信息。【Abstract】 Objective To study the clinical diagnosis value of video-electroencephalography (EEG) and MRI on pharmacal intractable epilepsy. Methods From December 2006 to May 2010, 38 cases of pharmacal intractable epilepsy were confirmed through operation and pathologic examination. Among them, there were 25 cases of hippocampal sclerosis, 2 cases of temporal lobe atrophy combined with brain dysplasia, 4 cases of heterotopic gray matter and macrogyria, 3 cases of vascular malformation, 2 cases of glioma, 1 case of cyst in brain, and 1 case of traumatic epilepsy. Video-EEG was applied to monitor the source of epileptoid discharge and the features of brain electrical activity during and between the occurrences of epilepsy. MRI was used to detect the manifestation characteristics of the epilepsy focus, and retrospective analysis was done to compare these findings with operational and pathological results. Results The accuracy rate of Video-EEG in locating the epilepsy focus was 100% (38/38) during the occurrence of epilepsy, and 53% (20/38) between the occurrences of epilepsy. The accuracy rate of MRI in diagnosing the epilepsy focus and relevant abnormalities during the occurrence of epilepsy was 89% (34/38), and 79% (30/38) in characterizing the abnormalities. Conclusion Video-EEG combined with MRI examination is effective in locating and characterizing the epilepsy focus, which can provide more useful information for the surgery in treating pharmacal intractable epilepsy.
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.
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 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.
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 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.
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.