脑磁图定位顽固性癫痫手术治疗51例临床分析

目的　探讨脑磁图在顽固性癫痫术前评估的价值。方法　术前对 5 1例顽固性癫痫患者行脑磁图、磁共振及视频脑电检查 ,结合术中皮层脑电图分别进行选择性海马杏仁核切除术、标准前颞叶切除术及致痫灶切除术等手术 ;并用脑磁图定位神经导航下手术切除致痫灶 2例 ,神经导航下海马杏仁核切除 2例。结果　术后发作完全停止的占 76 5 % ,发作减少 >75 %的占 11 8% ,发作减少>5 0 %的占 5 8% ,发作减少不足 5 0 %的占 5 8% ;脑磁图与视频脑电及皮层脑电图的定位符合率分别为 82 %和 96 % ;脑磁图对内侧颞叶癫痫的定位敏感性较差 ;脑磁图定位导航手术可在切除致痫灶的同时保护脑的重要结构。结论　脑磁图是一项灵敏的无创性癫痫灶定位方法 ,是癫痫外科术前评估中的一项技术突破     

Insular and insulo-opercular epilepsy in childhood: An SEEG study

PurposeIn recent years, there have been series analysing the electro-clinical correlations of insular epilepsy in adult populations. In contrast, the ictal semiology in children with insular epilepsy is poorly described. Considering that early and successful surgery may greatly improve the cognitive outcome and quality of life, it is worthwhile to deepen our knowledge of insular epilepsy in children.MethodsWe retrospectively evaluated ten children with drug-resistant focal insular epilepsy who had been consecutively explored with stereoelectroencephalography (SEEG), followed by individually tailored resective surgery that included part of the insula in all cases. A detailed anatomo-electro-clinical analysis of non-invasive EEG and SEEG data was performed. At least one of the electrodes explored the insular cortex. SEEG analysis confirmed that the insular cortex was included in the ictal onset zone.ResultsEpilepsy onset was mostly during the first year of life, characterized by subtle seizures as well as spasms and myoclonic seizures. Later on, neurovegetative signs and asymmetric tonic and hypermotor seizures (HMS) dominated the ictal semiology. The epileptogenic zone was frequently wider than insular with frontal and central predominance. In eight patients, the tailored resection included a lesion. In seven patients, an Engel class 1 outcome as well as neuropsychological and behavioural improvement was obtained.ConclusionsSEEG is feasible and useful in children with drug-resistant insular epilepsy which is often characterized by autonomic symptoms as the initial symptoms and should be suspected in cases with HMS, asymmetric tonic seizures and even asymmetric spasms. Early propagation is mostly frontal and central. Analysis of a larger population is required to refine these findings.     

Proposed criteria for referral and evaluation of children for epilepsy surgery: recommendations of the Subcommission for Pediatric Epilepsy Surgery

The Commission on Neurosurgery of the International League Against Epilepsy (ILAE) formed the Pediatric Epilepsy Surgery Subcommission in 1998 and charged it with formulating guidelines and recommendations for epilepsy surgery in childhood. Also endorsed by the Commission on Paediatrics, the following document is the consensus agreement after a meeting of 32 individuals from 12 countries in 2003. The panel agreed that insufficient class 1 evidence exists to recommend practice guidelines at this time. Instead, the panel generated criteria concerning the unique features of pediatric epilepsy patients to justify dedicated resources for specialty pediatric surgical centers, suggested guidelines for physicians for when to refer children with refractory epilepsy, and recommendations on presurgical evaluation and postoperative assessments. The panel also outlined areas of agreement and disagreement on which future research and consensus meetings should focus attention to generate practice guidelines and criteria for pediatric epilepsy surgery centers.     

Epileptic seizures and epilepsy: definitions proposed by the International League Against Epilepsy (ILAE) and the International Bureau for Epilepsy (IBE)

The International League Against Epilepsy (ILAE) and the International Bureau for Epilepsy (IBE) have come to consensus definitions for the terms epileptic seizure and epilepsy. An epileptic seizure is a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain. Epilepsy is a disorder of the brain characterized by an enduring predisposition to generate epileptic seizures and by the neurobiologic, cognitive, psychological, and social consequences of this condition. The definition of epilepsy requires the occurrence of at least one epileptic seizure.     

癫癎及癫癎综合征国际分类(1989年)的临床应用分析

目的探讨各类癫和癫综合征在就诊人群中的分布,以利于癫患者的诊治。方法按国际抗癫联盟(ILAE)1989年推荐的癫和癫综合征的分类方案对门诊患者进行调查。结果1191例患者中,部分性癫和综合征766例(64·3%),全面性癫和综合征240例(20·2%),不能确定为部分性或全面性的癫及综合征79例(6·7%),特殊综合征16例(1·3%),其他非癫疾患90例(7·6%)。在部分性癫和综合征中,症状性者占了绝大多数,而全面性癫和综合征中,特发性者占绝大多数。结论正确地进行癫分类对临床有重要的指导意义,各种癫综合征的分布是有规律可循的。     

Epilepsy Surgery in the First Three Years of Life

Summary: Purpose: Partial seizures in early postnatal life may be catastrophic and associated with poor long-term outcome. Epilepsy surgery can alleviate partial seizures in older children and adults, but there is little experience with surgical therapy in infancy apart from hemispheric epilepsy syndromes.
Methods: We analyzed the results of cortical resection to treat medically refractory partial epilepsy in 31 children (16 boys, 15 girls) aged <3 years (mean, 18.3 months). Subjects were included only if seizure relief was the primary indication for surgery.
Results: Follow-up of at least 1 year (mean, 4.6 years) in 26 patients revealed that 16 were seizure-free, 4 had >90% seizure reduction, and 6 had <90% reduction. There was no significant difference in seizure outcome between hemispherectomy/multilobar resections and lobar resections or temporal versus extratemporal resection. Seizure outcome was independent of the amount of cortex removed in nonlesional patients. Only the presence of a discrete lesion on preoperative neuroimaging correlated with a favorable outcome. Family perceptions of accelerated development in seizure-free patients were not confirmed on developmental assessment.
Conclusions: We conclude that cortical resection often benefits very young children with catastrophic partial seizures, but does not guarantee enhanced neurological development. The location and extent of the excised cortex may not be critical as long as the entire epileptogenic region and tesion are removed.     

Paediatric epilepsy surgery in the posterior cortex: a study of 62 cases

Past surgical series have emphasized the diagnostic complexity of posterior cortex epilepsy. Available data are sparse, especially in children, and most published series report a high number of surgical failures and post‐operative neurological deficits. In this article, we present a paediatric cohort of 62 children who underwent surgery for drug resistant posterior cortex epilepsy before the age of 16 years with a mean post‐operative follow‐up of 6.94 years (range: 2–16). Mean age at epilepsy onset was 3.2 years and 28 children (45%) had onset before 1 year of age. The mean age at surgery was 7.9 years (range: 1–16). Daily seizures were present in 63% of children. MRI was positive in 58 cases (93.5%) and invasive stereo‐EEG was judged mandatory in 24/62 (39%) of patients. Surgery was confined to the parietal lobe in 11 children, the occipital lobe in 8, the occipito‐parietal region in four, the occipito‐temporal region in 18, and involved both the temporal and parietal lobes in the remaining 21. Following surgery, 53 subjects (85.5%) remained seizure‐free and among those who underwent a SEEG procedure, 75% achieved seizure freedom. Focal cortical dysplasia was the most frequent histopathological diagnosis (50%), followed by tumoural (24%) and gliotic lesions (14.5%). An older age at epilepsy onset, the presence of a rather restricted epileptogenic area, and a complete resection of the epileptogenic zone were predictive of a favourable surgical outcome. These results demonstrate that a good surgical outcome is possible in children with drug resistant posterior cortex epilepsy. Accurate analysis of the chronology of ictal semiology and electrophysiological features, viewed in the context of the complete electroclinical pattern, provides a topographical orientation for posterior cortex epilepsy and, together with the presence of a lesion detectable on imaging, may improve the rate of surgical success of posterior cortex epilepsy at paediatric age.     

Surgery for pharmacoresistant epilepsy in the developing world: A pilot study

Epilepsy prevalence in the developing world is many fold that found in developed countries. For individuals whose conditions failed to respond to pharmacotherapy, surgery is the only opportunity for cure. In Uganda, we developed a center for treatment of intractable temporal lobe epilepsy (iTLE) that functions within the technologic and expertise constraints of a severely low resource area. Our model relies on partnership with epilepsy professionals and training of local staff. Patients were prescreened at regional clinics for iTLE. Individuals meeting inclusion criteria were referred to the treating Ugandan hospital (CURE Children's' Hospital of Uganda, CCHU) for video-EEG (electroencephalography), computed tomography (CT) imaging, and neuropsychological evaluation. Data were transferred to epilepsy experts for analysis and treatment recommendations. Ten patients were diagnosed with iTLE and surgically treated at CCHU. Six (60%) were seizure free, and there was no neurologic morbidity or mortality. Our model for surgical treatment of pharmacoresistant TLE has functioned successfully in a true developing world low resource setting.     

Establishing criteria for pediatric epilepsy surgery center levels of care: Report from the ILAE Pediatric Epilepsy Surgery Task Force

Presurgical evaluation and surgery in the pediatric age group are unique in challenges related to caring for the very young, range of etiologies, choice of appropriate investigations, and surgical procedures. Accepted standards that define the criteria for levels of presurgical evaluation and epilepsy surgery care do not exist. Through a modified Delphi process involving 61 centers with experience in pediatric epilepsy surgery across 20 countries, including low–middle- to high-income countries, we established consensus for two levels of care. Levels were based on age, etiology, complexity of presurgical evaluation, and surgical procedure. Competencies were assigned to the levels of care relating to personnel, technology, and facilities. Criteria were established when consensus was reached (≥75% agreement). Level 1 care consists of children age 9 years and older, with discrete lesions including hippocampal sclerosis, undergoing lobectomy or lesionectomy, preferably on the cerebral convexity and not close to eloquent cortex, by a team including a pediatric epileptologist, pediatric neurosurgeon, and pediatric neuroradiologist with access to video-electroencephalography and 1.5-T magnetic resonance imaging (MRI). Level 2 care, also encompassing Level 1 care, occurs across the age span and range of etiologies (including tuberous sclerosis complex, Sturge-Weber syndrome, hypothalamic hamartoma) associated with MRI lesions that may be ill-defined, multilobar, hemispheric, or multifocal, and includes children with normal MRI or foci in/abutting eloquent cortex. Available Level 2 technologies includes 3-T MRI, other advanced magnetic resonance technology including functional MRI and diffusion tensor imaging (tractography), positron emission tomography and/or single photon emission computed tomography, source localization with electroencephalography or magnetoencephalography, and the ability to perform intra- or extraoperative invasive monitoring and functional mapping, by a large multidisciplinary team with pediatric expertise in epilepsy, neurophysiology, neuroradiology, epilepsy neurosurgery, neuropsychology, anesthesia, neurocritical care, psychiatry, and nursing. Levels of care will improve safety and outcomes for pediatric epilepsy surgery and provide standards for personnel and technology to achieve these levels.     

国际抗癫(癎)联盟国际癫(癎)综合征分类可分类率比较及架构分析

目的 比较国际抗癫(癎)联盟(International League Against Epilepsy,ILAE)1989、2001、2006年癫(癎)综合征分类的可分类率并分析架构变化.方法 先后依据3个分类对2007年8月1日至2008年3月31日连续就诊于北京协和医院癫(癎)中心的1356例癫(癎)患者进行癫(癎)综合征诊断,并进行统计学分析.结果 3个分类的可分类率分别为75.5 % 、89.0 % 和88.1 % ,2001及2006年分类明显优于1989年分类(x2=116.3,P<0.01);对特异综合征的可分类率分别为11.6 % (157例)、12.0 % (162例)及11.9 % (160例),差异无统计学意义(x2=0.09,P>0.05).结论 ILAE 2006年癫(癎)综合征分类引入了较科学的分类模式和评价体系,提高了癫(癎)的可分类率,可尝试应用于临床及基础研究.     

Ganglioglioma and Intractable Epilepsy: Clinical and Neurophysiologic Features and Predictors of Outcome After Surgery

Summary: Purpose: To review the clinical, neurophysiologic, and radiological data of patients with ganglioglioma who had undergone evaluation and surgery in our Epilepsy Program.
Methods: The medical and neurophysiologic records of 38 patients with intractable epilepsy and ganglioglioma were re- viewed. Data underwent statistical analysis.
Results: There were 28 temporal and 10 extratemporal resections, with a mean age at seizure onset of 10.5 years and mean age at surgery of 22 years. Five tumor resections performed earlier were recorded. Twenty-nine patients had auras and 20 had secondarily generalized seizures. All 28 patients with temporal tumor had complex partial seizures. Preoperative MRI demonstrated the tumor in 36 of 36 patients: 17 of 29 demonstrated gadolinium enhancement, and 17 of 36 had mass effect. Scalp interictal sharp waves were present in 32 patients, and in 15 they were multiregional. In two patients, scalp EEG seizure onset was from the hemisphere contralateral to the tumor. Postoperatively, 79% of patients (30 of 38) were seizure-free (Engel's class I) at 6 months, 72% at 1 year (26 of 36), and 63% at 2 years (20 of 32). Excellent outcome was associated with a lower age at operation (p = 0.008), shorter duration of epilepsy (p = <0.01), absence of generalized seizures (p = <0.01), and no epileptiform discharges on a postoperative EEG (p = 0.01).
Conclusions: Good surgical outcome is expected in patients with ganglioglioma despite years of medically resistant seizures. Good outcome may be achieved despite EEG findings that may conflict with tumor location, and is more likely when surgery is performed relatively soon after epilepsy onset.     

Sleep profile and Polysomnography in patients with drug-resistant temporal lobe epilepsy (TLE) due to hippocampal sclerosis (HS) and the effect of epilepsy surgery on sleep-a prospective cohort study

ObjectivesWe analyzed changes in sleep profile and architecture of patients with drug-resistant TLE-HS using three validated sleep questionnaires- Epworth Sleepiness Scale (ESS), Pittsburgh Sleep Quality Index (PSQI), NIMHANS Comprehensive Sleep Disorders, and polysomnography (PSG). We studied the effect of epilepsy surgery in a subset of patients.MethodsIn this prospective observational cohort study, sleep profile of 40 patients with drug-resistant TLE-HS was compared to 40 healthy matched controls. Sleep architecture of 22 patients was studied by overnight PSG and compared to 22 matched controls. Sleep profile was reassessed in 20 patients after a minimum period of three months after epilepsy surgery.ResultsThe mean PSQI was higher among patients compared to controls(P=0.0004) while mean ESS showed no difference. NCSDQ showed fewer patients feeling refreshed after a night's sleep compared to controls (p=0.006). PSG revealed a higher time in bed (p=0.0001), longer total sleep time (p=0.006) and more time spent in NREM stage 1 (p=0.001) and stage 2 (p=0.005) while spending less time in stage 3 (p=0.039) among TLE patients. Sleep efficiency was worse in patients on ≥3 ASMs compared to those on 2 ASMs (p-0.044). There was no change in mean ESS (p=0.48) or PSQI (p=0.105) after surgery.ConclusionsPatients with drug-resistant TLE-HS have an altered sleep profile and architecture. Patients on ≥3 ASMs have a lower sleep efficiency. Reassessment at short intervals after epilepsy surgery did not reveal significant changes in sleep profile.     

Nonsymptomatic generalized epilepsy in children younger than six years: excellent prognosis,but classification should be reconsidered after follow-up: the Dutch Study of Epilepsy in Childhood

PURPOSE: To assess the prognosis and the accuracy of the epilepsy classification in young children with nonsymptomatic generalized epilepsy. METHODS: Of the cohort of the Dutch Study of Epilepsy in Childhood (n = 466), all children younger than 6 years with a diagnosis of idiopathic (IGE) or cryptogenic (CGE) generalized epilepsy either at intake (n = 108) and/or after 2 years of follow-up (n = 102) were included. The number of reclassifications after 2 years was determined, and the reasons for reclassification were analyzed. All children receiving a diagnosis of IGE or CGE at 2 years were followed up for 5 years to study their outcome in terms of terminal remission (TR). Data on their level of intellectual functioning were collected at the start of this analysis. RESULTS: The epilepsy syndrome was reclassified in 17 children. In 14 of them, the seizure type also was reclassified, and in three, the course of the epilepsy determined the new epilepsy type. Two other children had a reclassification of their seizure types without a change of the epilepsy type. Many children were categorized as having IGE not otherwise specified. In all probability, this is a heterogeneous group, containing patients with various epilepsy syndromes, with generalized tonic-clonic seizures as a common hallmark. Of the 102 children with IGE or CGE at 2 years of follow-up, 75% had a TR of >6 months after 2 years, and 85% a TR of >or=1 year after 5 years. CONCLUSIONS: In a fair proportion of children with nonsymptomatic generalized epilepsy in this age group, it is not possible to classify firmly the epilepsy and/or the seizures immediately after the intake. Instead, they are reclassified during the course of the disease. This and the apparent heterogeneity of the category IGE not otherwise specified point to inherent drawbacks of the current International League Against Epilepsy (ILAE) classification of epilepsy and epileptic syndromes. The prognosis of IGE at this young age is generally excellent.     

Severe Myoclonic Epilepsy of Infants (Dravet Syndrome): Natural History and Neuropsychological Findings

Summary:  Severe Myoclonic Epilepsy in infancy (SMEI, or Dravet syndrome) is a drug-resistant epilepsy that occurs in the first year of life of previously healthy children. The main clinical features are prolonged and repeated febrile and afebrile generalized or unilateral convulsive seizures. In the course of the epilepsy, cognitive deterioration becomes evident, and interictal myoclonus, clumsiness and ataxia appear. One third of the children with SMEI show de novo mutations of the SCN1A gene, and additional familial genes probably contribute to the phenotype. While the clinical picture of SMEI has been well studied, neuropsychological data remain scarce. Global mental retardation, attention deficit and psychotic behavior have been reported but the long-term outcome has not been evaluated. We conducted a longitudinal neuropsychological study of children with SMEI. Twenty children, aged 11 months to 16 years, were prospectively examined using standardized neuropsychological tests. Correlation analysis with other clinical features was performed in 12 cases. Marked slowing or stagnation of psychomotor development, accompanied by psychotic or autistic traits and hyperactivity, was observed between the ages of one and four years. In the later stages (at ages 5 to 16 years), cognitive function stabilized but remained below normal. In children with a more favorable course, language capacities were better preserved than visuospatial functions, and behavior improved. The cognitive and behavioral impairment tended to correlate with the frequency of convulsive seizures (>5 per month). The data suggest that SMEI can be considered as a prototype of an epileptic encephalopathy.     

Epilepsy in Colombia: epidemiologic profile and classification of epileptic seizures and syndromes

PURPOSE: A national study was performed in Colombia to determine the general and regional prevalence of epilepsy, clinical profiles, seizure types, and clinical syndromes. METHODS: Based on the National Epidemiological Study of Neurological Diseases (EPINEURO), we evaluated and followed up for 1 year all the subjects with epilepsy from the National Sample. Clinical profiles were further assessed. Seizure types and epilepsy syndromes were established according to the international classifications. RESULTS: General prevalence was found to be 11.3 per 1,000, with little variation among regions, except the eastern region, where prevalence was 23 per 1,000; prevalence for active epilepsy was 10.1 per 1,000. Women have a slightly greater (not statistically significant) risk. Most seizures are focal (partial), frequently with secondary generalization. The most frequent epilepsy syndrome encountered was partial symptomatic/cryptogenic (80%). Epilepsy onset in Colombia occurs most frequently in childhood. CONCLUSIONS: Prevalence rates of epilepsy in Colombia are similar to those reported in nations with comparable developmental status and have diminished over time. The study presents the distribution of seizures and syndromes. The most frequent types are focal syndromes.     

Surgical management of cortical dysplasia in infancy and early childhood

Purpose: To describe operative procedures, seizure control and complications of surgery for cortical dysplasia (CD) causing intractable epilepsy in infancy and early childhood. Methods: Fifty-six consecutive children (less than 6 years old) underwent resective epilepsy surgery for CD from December 2000 to August 2011. Age at surgery ranged from 2 to 69 months (mean 23 months) and the follow-up was from 1 to 11 years (mean 4 years 4 months). Results: Half of the children underwent surgery during infancy at an age less than 10 months, and the majority (80%) of these infants needed extensive surgical procedures, such as hemispherotomy and multi-lobar disconnection. Seizure free (ILAE class 1) outcome was obtained in 66% of the cases (class 1a; 55%): 85% with focal resection (n = 13), 50% with lobar resection (n = 18), 71% with multilobar disconnection (n = 7) and 67% with hemispherotomy (n = 18). Peri-ventricular and insular structures were resected in 23% of focal and 61% of lobar resections. Repeated surgery was performed in 9 children and 5 (56%) became seizure free. Histological subtypes included hemimegalencephaly (16 patients), polymicrogyria (5 patients), and FCD type I (6 patients), type IIA (19 patients), type IIB (10 patients). Polymicrogyria had the worst seizure outcome compared to other pathologies. Surgical complications included 1 post-operative hydrocephalus, 1 chronic subdural hematoma, 2 intracranial cysts, and 1 case of meningitis. No mortality or severe morbidities occurred. Conclusions: Early surgical intervention in children with CD and intractable seizures in infancy and early childhood can yield favorable seizure outcome without mortality or severe morbidities although younger children often need extensive surgical procedures.