阿尔茨海默病伴发癫癎的研究

目的:探讨阿尔茨海默病伴发癫（疒间）的临床特征. 方法:对45例老年型阿尔茨海默病(AD)与47例健康老人进行对照分析. 结果:AD有7例伴发癫（疒间）.AD伴发癫（疒间）的患者年龄明显较不伴发癫（疒间）者为轻.AD伴发癫（疒间）的类型均为全身强直-阵挛性发作,药物较易控制. 结论:AD可能伴有癫（疒间）发作,对有发作者应予抗癫（疒间）治疗.     

抗癫药物致癇作用的临床观察

目的　研究抗癫疒间 药物 (AEDs)的致疒间作用及对其的预防和治疗。方法　总结 32例AEDs致癫疒间 发作加重患者的临床资料。结果　 32例中以服用卡马西平最多 (19.12 % )、次之为巴比妥类药物 (15 % )、苯妥英钠 (14 .5 % )、合并用药 (14 .2 9% )、苯二氮 艹卓 类 (8.6 9% )、丙戊酸钠 (2 .78% )。 32例经停药并合理选择其他抗癫疒间 药物及减少药物剂量治疗 ,2 7例有效 ,5例无效。结论　AEDs能致癫疒间 发作加重 ,应合理选择抗癫疒间 药物     

脑卒中后继发癫(疒间)的临床观察

目的 探讨脑卒中与继发性癫(疒间)的临床关系.方法 对1313例脑卒中患者的临床资料进行回顾性分析. 结果脑卒中后继发癫(疒间)90例,占6.85%,癫(疒间)发作类型以全身强直阵挛发作常见,脑卒中以缺血性脑血管病继发癫(疒间)常见,脑卒中以脑叶常见. 结论脑卒中继发癫(疒间)与卒中时间、类型、发作部位有关.     

多种术式联合治疗难治性额叶癫疒间临床分析

目的观察额叶致疒间灶切除术和选择性胼胝体切开术联合癫疒间灶周围多处软膜下横纤维切断手术治疗难治性额叶癫疒间的临床疗效。方法分析在我院接受多种术式联合治疗的19例难治性额叶癫疒间的临床资料,应用Engel标准进行术后效果分级,并对其中15例随访6个月~4a,总结手术体会,评价其远期疗效。结果按Engel分级:Ⅰ~Ⅱ级15例(78.9%),Ⅲ级4例(21.1%)。术后出现短暂肢体偏瘫6例,缄默2例,小便失禁3例,无死亡。癫疒间灶病理检查多为神经元变性及胶质增生。结论难治性额叶癫疒间手术治疗效果较满意,需要多种术式联合治疗才能提高手术效果。     

皮质电刺激在功能区致疒间灶手术中的应用

目的：分析皮质电刺激在功能区致疒间灶手术中的应用。方法回顾性分析8例功能区难治性癫疒间病人的临床资料。根据视频脑电图及电刺激结果，确定致疒间灶与功能区之间位置关系，术中保护功能区，最大程度切除致疒间灶。结果手术顺利，术后2例左额叶软化灶病人术前肌力4级，术后无肌力下降；1例右枕叶皮质发育不良病人，术后视野部分缺损。随访6个月~3年，6例病人无癫疒间发作，2例病人发作减少90%。Engel分级Ⅰ~Ⅱ级。结论皮质电刺激是定位大脑功能区的金标准，应用皮质电刺激治疗功能区癫疒间可以在保留功能区的基础上最大程度切除致疒间区，提高癫疒间病人生活质量，更好地控制癫疒间。     

氯氮平致脑电图异常及癫癎发作的文献回顾

目的:了解我国应用氯氮平引起脑电图(EEG)异常及癫疒间发作的情况。方法:通过数据库及手工检索收集1979年至2004年国内有关氯氮平所致EEG异常及癫疒间发作的文献143篇,其中癫疒间发作的个案报道30例。结果:异常发生率为67.20%(5 543/8 249),癫疒间发作的发生率为0.95%(297/31 185)。资料显示,氯氮平的口服剂量越大,发生EEG异常及癫疒间的风险就越大、EEG异常程度就越重。结论:服用氯氮平治疗的患者定期进行EEG及血药浓度监测十分必要。     

24h视频脑电图监测在发作性疾病中的应用

目的探讨24h视频脑电图(Video-EEG)对癫疒间的诊断及鉴别诊断价值。方法 158例常规脑电图(EEG)无疒间样放电患者分为临床癫疒间组和可疑癫疒间组进行24hVideo-EEG监测并分析结果。结果临床癫疒间组疒间样放电总发生率60.0%,可疑癫疒间组总发生率15.1%,疒间样放电多出现于非快速眼动睡眠期。结论 Video-EEG可提高癫疒间样放电的阳性率,对癫疒间和非癫疒间发作的诊断与鉴别诊断有重要价值。     

托吡酯加用治疗难治性部分性癫癎发作的临床研究

目的:观察托吡酯加用治疗癫（疒间）难治性部分性发作的疗效.方法:32例难治性部分性发作或继发性全身性发作癫（疒间）患者,病程1年以上,一直服用一种或两种基础抗癫（疒间）药物每月仍有≥4次以上的发作.托吡酯采用加量法,加量期8周,目标剂量为200mg@d-1,观察12周后进入延长期.结果:32例病人经过治疗后,发作100%消失者5例(15.62%),发作≤75%13例(40.62%),发作≤50%11例(34.37%),无改善2例(6.25%),因不良反应而中断1例(3.13%).结论:托吡酯是一种非常有效的新型抗癫（疒间）药物.     

脑磁图对病灶性新皮质癫(疒间)术前评估的价值

目的 研究病灶性新皮质癫(疒间)发作间期MEG 和MRI 一致性与预后的关系.方法 23 例病灶性新皮质癫(疒间)接受了术前评估及手术治疗,所有患者术前均接受MEG 检测,数据分析处理采用等价电流偶极子(ECD)法和合成孔径法(SAMg2).7 例患者接受了颅内电极植入后长程视频皮层脑电图监测.术后随访一年以上并运用Engel 法评价预后.结果 MEG 与MRI 一致的病例手术有效率(Engel class I or II)及术后无发作率(Engel class IA)均优于不一致者.结论 病灶性新皮质癫(疒间),MEG 结果与MRI 结果一致时手术效果良好,对此类病例不需要再进行颅内电极植入行癫(疒间)灶定位,颅内电极植入仅适用于MEG 结果与MRI 结果不一致的病例或需要功能区定位者.     

深部电极引导立体定向手术治疗颞叶癫(疒间)

目的 评价深部电极引导立体定向手术治疗颞叶癫(疒间)的疗效.方法 对94例无明显病灶的颞叶癫(疒间)病人采用深部电极引导立体定向海马杏仁复合体热凝毁损术,术后随访癫(疒间)控制效果.对其中27例行手术前后神经心理学检查,评价手术对脑功能的影响.结果 80例获1～7年随访,其中发作完全消失47例(58.8%),减少75%以上18例(22.5%),减少50%～75%9例(11.2%),无变化6例(7.5%).神经心理学检查表明手术前后智力、记忆力差异无统计学意义(均P＞0.05).结论 选择合理手术人路及术中应用深部电极取代以往的解剖学定位,达到理想有效的毁损范围,有助于提高颞叶癫(疒间)的手术疗效.     

Outcomes of epilepsy surgery in childhood-onset epileptic encephalopathy

Purpose: to evaluate the outcomes and role of epilepsy surgery in children with intractable epileptic encephalopathy (EE). Methods: ninety-five children (64 boys, 31 girls) with intractable EE were treated by epilepsy surgery at Severance Children’s Hospital from 2003 to 2008. Surgical treatments included lobar resection, hemispherotomy and corpus callosotomy (CC). Seventy-six children were Lennox–Gastaut syndrome (LGS), and 19 had West syndrome. Results: of the 76 patients with LGS, CC was performed in 37 patients (48.7%), lobar resection in 29 (38.2%) and hemispherotomy in 10 (13.2%). Of the 19 patients with West syndrome, respective surgery was performed in 15 patients (78.9%) and CC in 4 (21.1%). Of the patients receiving respective surgery, Engel’s class I outcomes were achieved for 24 of 39 (61.5%) of LGS patients, and for 9 of 15 (60.0%) of West syndrome. Malformations of cortical development were commonly observed, appearing in 73.5% (36/49). In neuropsychiatric tests, 19 of 27 with LGS demonstrated improvement in postoperative cognitive function. More significant intellectual improvement correlated well with shorter epilepsy duration, good seizure outcomes, and decreased number of antiepileptic drugs. Conclusions: epilepsy surgery should be considered in treating childhood intractable EE with expectation of improvement of both seizure and cognitive outcomes, even in cases of LGS.     

Prenatal Etiologies of West Syndrome

Summary: We investigated the etiology of West syndrome (WS) with special reference to prenatal factors in 180 cases. Prenatal cause was the most frequent diagnosis (77 cases, 42.8%), followed by perinatal (25 cases, 13.9%) and postnatal factors (12 cases, 6.7%); 48 cases (26.7%) were of uncertain etiology; eighteen cases (10.0%) were idiopathic. Of the three forms of age-dependent epileptic encephalopathy, prenatal cause was present in 12 of 15 cases (80.0%) of early-infantile epileptic encephalopathy with suppression-burst, 77 of 180 cases (42.8%) of WS, and 31 of 123 cases (25.2%) of Lennox-Gastaut syndrome (LGS). Prenatal factors of WS included tuberous sclerosis (23), chromosome abnormalities (10), cerebral dysgen- esis (10), porencephaly (7), hydrocephalus (5), Aicardi syndrome (3), Aicardi syndrome associated with chromosome abnormality (1), and other causes (18). Chromosome abnormalities with WS consisted of 6 cases with 21 trisomy and one case each with 18q duplication, t(l;y) translocation, 7q duplication, and partial 2p trisomy. One patient with Aicardi syndrome also had a t(12;21) translocation. No significant difference was observed in the age of onset of WS among the five etiologic groups. The evolution from WS to LGS was not influenced by etiology, except for the idiopathic group. In patients followed for over 3 years, seizure remission occurred in 46.8% (22 of 47 cases) of the prenatal group. This was lower than the other four groups. Intellectual prognosis was also relatively poor in those with prenatal onset. Pyridoxal phosphate (PAL-P) treatment was effective in 9 of 70 (12.9%) prenatal cases and 5 of 18 (27.8%) idiopathic cases. Notably, efficacy rates of 14.3, 14.8%, and 20.0% were observed in those with tuberous sclerosis, brain malformation, and chromosome abnormality, respectively. No significant difference in the effect of synthetic ACTH (Cortrosyn-Z) was observed among the five groups. These findings suggested that WS due to prenatal etiologies and those due to other etiologies are essentially homogeneous in age of onset and clinicoelectrical features, although each has some individual characteristics. We conjecture that WS is one form of an age-specific epileptic reaction to diverse nonspecific exogenous insults.     

Neuropathology of the limbic system and brainstem in West syndrome.

Both West syndrome (WS) and Lennox-Gastaut syndrome (LGS) are associated with various developmental disorders and it has been discussed whether the cerebral cortex or subcortical structures are important in the pathogenesis of both epileptic syndromes. Here we briefly review the literature on the neuropathological findings in WS and LGS, and present our data on immunohistochemical analysis of the brainstem and limbic lesions in autopsy cases of lissencephaly and sequels of hypoxic ischemic encephalopathy (HIE) caused by perinatal asphyxia manifested as both WS and LGS (WS/LGS). Nowadays, the neuroradiological examinations and surgical pathology in WS cases demonstrate dysplastic cerebral lesions more frequently than previously expected. On the other hand, we have delineated the common brainstem lesions such as small size of the tegmentum and spongy state and/or gliosis in the central tegmental tract in a number of WS autopsy cases of various etiologies. Recently, we reported the reduced expression of tyrosine hydroxylase, methionine enkephalin and parvalbumin in the brainstem in autopsy cases of lissencephaly and sequels of HIE manifested as WS/LGS, regardless of the cerebral changes. In the same subjects, we examined the expression of glutamate transporters and calcium-binding proteins in the limbic system by immunohistochemistry. These represent markers of glutamate neurotoxicity and the GABAergic inhibitory neuron system, respectively. The altered expressions of glial glutamate transporters and calcium-binding proteins in the limbic system seemed to reflect temporal lobe sclerosis, irrespective of the past history of WS, and there were no differences in the limbic involvement between the cases manifested as WS/LGS and disease controls of sequels of HIE not manifested as WS/LGS. It is more likely that the brainstem lesions contribute to the pathogenesis of WS and/or LGS more than the heterogeneous limbic lesions in these cases.     

Long-Term Prognosis of Lennox-Gastaut Syndrome

Summary: We investigated the long-term prognosis of Lennox-Gastaut syndrome (LGS) in 72 patients followed up for >10 years. Long-term seizure and intellectual outcomes were poor, as previously reported. The diagnosis of LGS was first made in the age range from 2 to 15 years with peak occurrence at 5 years. Progressive IQ score deterioration with age was apparent. At the last examination, 33% of patients with cryptogenic and 55% with symptomatic LGS had lost the characteristics of LGS, and their seizure disorders were classifiable as symptomatic generalized epilepsies, severe epilepsy with multiple independent spike foci, or localization-related epilepsies. Disabling drop attacks appeared in 46% of patients and tended to occur at older than 10 years. Gait deterioration was recognized in 12 patients and seemed to be due largely to, progression of the epileptic encephalopathy. The gait disturbances, as well as increased frequency of violent drop attacks, were disabling in daily life and resulted in some patients being wheelchair bound.     

Vagus nerve stimulation vs. corpus callosotomy in the treatment of Lennox–Gastaut syndrome: A meta-analysis

PurposeLennox–Gastaut syndrome (LGS) is an epileptogenic disorder that arises in childhood and is typically characterized by multiple seizure types, slow spike-and-wave complexes on EEG and cognitive impairment. If medical treatment fails, patients can proceed to one of two palliative surgeries, vagus nerve stimulation (VNS) or corpus callosotomy (CC). Their relative seizure control rates in LGS have not been well studied. The purpose of this paper is to compare seizure reduction rates between VNS and CC in LGS using meta-analyses of published data.MethodsA systematic search of Pubmed, Ovidsp, and Cochrane was performed to find articles that met the following criteria: (1) prospective or retrospective study, (2) at least one patient diagnosed with Lennox–Gastaut syndrome, and (3) well-defined measure of seizure frequency reduction. Seizure reduction rates were divided into seizure subtypes, as well as total seizures, and categorized as 100%, >75%, and >50%. Patient groups were compared using chi-square tests for categorical variables and t-test for continuous measures. Pooled proportions with 95% confidence interval (95% CI) of seizure outcomes were estimated for total seizures and seizure subtypes using random effects methods.Results17 VNS and 9 CC studies met the criteria for inclusion. CC had a significantly better outcome than VNS for >50% atonic seizure reduction (80.0% [67.0–90.0%] vs. 54.1% [32.1–75.4%], p < 0.05) and for >75% atonic seizure reduction (70.0% [48.05–87.0%] vs. 26.3% [5.8–54.7%], p < 0.05). All other seizure types, as well as total number of seizures, showed no statistically significant difference between VNS and CC.ConclusionsCC may be more beneficial for LGS patients whose predominant disabling seizure type is atonic. For all other seizure types, VNS offers comparable rates to CC.     

Aicardi Syndrome: A Longitudinal Clinical and Electroencephalographic Study

Summary: We report clinical and EEC follow-up of 6 children with Aicardi syndrome. Age at seizure onset was <3 months in 5 patients and 4 months in 1 patient. All patients had spasms, and these continued at time of follow-up in 5 patients. Five patients had seizures other than spasms which disappeared during early infancy. Bilateral independent bursts (BIBs) characteristic of Aicardi syndrome were noted in 4 patients. In 1, BIBs showed suppression-burst patterns. BIBs converted to hypsarrhythmia or multifocal spikes with a tendency to BIBs during sleep. BIBs disappeared completely during both waking and sleeping states between the ages of 3 years 1 month and 4 years 9 months. After BIB disappearance, the EEG of 1 patient showed diffuse slow spike-and-wave complexes most of which appeared asynchronously. At onset, 1 patient had early-infantile epileptic encephalopathy with suppression-burst which evolved initially into West syndrome (WS) and then Lennox-Gastaut syndrome (LGS). The other 3 children also had WS.     

Callosotomy vs Vagus Nerve Stimulation in the Treatment of Lennox-Gastaut Syndrome: A Systematic Review With Meta-Analysis

BackgroundLennox-Gastaut syndrome (LGS) is a severe drug-resistant epileptic syndrome. Palliative treatments such as corpus callosotomy (CC) and vagus nerve stimulation (VNS) have emerged as treatments to reduce the number of seizures in patients. The aim of this study is to compare the effectiveness of CC and VNS in patients with LGS studied in the last 30 years.Materials and MethodsWe conducted a systematic review with meta-analysis and collected papers from PubMed (MEDLINE), Ovidsp, Web of Science, and Cochrane Library data bases. The articles analyzed were published between January 1990 and December 2020. Keywords were chosen based on internal and external validation in the PubMed data base (the analysis is available in the Supplementary Data Supplementary Appendix). Prospective or retrospective case reports (n ≥ 2), case series, cohort studies, or case-control studies involving patients with LGS were included in the analysis. We selected studies that had no age or sex restriction and that provided data on seizures before and after treatments. Studies not written in English, published without peer review, or not indexed in the data bases were excluded. Other exclusion criteria were the absence of seizure data and the impossibility of extracting this information from the studies. To analyze the results, we used the random-effects model based on the assessment of heterogeneity (I² statistics) in two scenarios. In scenario 1, we assessed the incidence of patients with a seizure reduction ≥ 50%; in scenario 2, we assessed the incidence of patients with a seizure reduction > 0%.ResultsOf the 7418 articles found using the keywords, 32 were considered eligible. Of these, 18 articles were on VNS (175 patients) and 14 on CC (107 patients). For scenario 1 (seizure reduction ≥ 50%), CC had an incidence of 65% (95% CI, 37%–94%), with an I² value of 82.7%; VNS had an incidence of 34% (95% CI, 11%–57%), with an I² value of 80.7%. For scenario 2 (seizure reduction > 0%), CC had an incidence of 80% (95% CI, 58%–100%), with an I² value of 84.7%; VNS had an incidence of 64% (95% CI, 38%–89%), with an I² value of 90.8%. There was an overlap of confidence intervals, with no statistical difference between the treatments in both scenarios.DiscussionOur analysis of LGS showed that the CC and VNS treatments are significantly beneficial to reducing seizures, without superiority between them.     

Lennox-Gastaut syndrome: a comprehensive review

Lennox-Gastaut syndrome (LGS) is considered an epileptic encephalopathy and is defined by a triad of multiple drug-resistant seizure types, a specific EEG pattern showing bursts of slow spike-wave complexes or generalized paroxysmal fast activity, and intellectual disability. The prevalence of LGS is estimated between 1 and 2% of all patients with epilepsy. The etiology of LGS is often divided into two groups: identifiable (genetic-structural-metabolic) in 65 to 75% of the patients and LGS of unknown cause in others. Lennox-Gastaut syndrome may be considered as secondary network epilepsy. The seizures in LGS are usually drug-resistant, and complete seizure control with resolution of intellectual and psychosocial dysfunction is often not achievable. Reduction in frequency of the most incapacitating seizures (e.g., drop attacks and tonic-clonic seizures) should be the major objective. Valproate, lamotrigine, and topiramate are considered to be the first-line drugs by many experts. Other effective antiepileptic drugs include levetiracetam, clobazam, rufinamide, and zonisamide. The ketogenic diet is an effective and well-tolerated treatment option. For patients with drug resistance, a further therapeutic option is surgical intervention. Corpus callosotomy is a palliative surgical procedure that aims at controlling the most injurious seizures. Finally, vagus nerve stimulation offers reasonable seizure improvement. The long-term outcome for patients with LGS is generally poor. This syndrome is often associated with long-term adverse effects on intellectual development, social functioning, and independent living.     

Long-term follow-up study of Lennox-Gastaut syndrome in patients with severe motor and intellectual disabilities: with special reference to the problem of dysphagia.

A long-term follow-up study of Lennox-Gastaut syndrome (LGS) ( > 10 years) was conducted with 38 patients with severe motor and intellectual disabilities (SMID) to clarify the relationship between the rapid development of dysphagia and epileptic seizures, and to elucidate the long-term evolution of LGS in patients with SMID. Those who showed a relatively favourable seizure outcome were compared to those with a poor seizure outcome. Poor seizure outcome correlated strongly with: (a) an early appearance of dysphagia and additional deterioration of the already retarded mental function; (b) a predominance of atypical absence seizures; and (c) persistent frequent epileptiform discharges during electroencephalographic evaluations. Neither age at seizure onset nor intelligence level at the time of the last examination was correlated with seizure prognosis. Further, seizure prognosis was not related to the aetiology of LGS. Repeated seizures apparently caused development of progressive epileptic encephalopathy, in addition to the underlying severe brain damage. Since development of dysphagia burdens an already severely handicapped patient with intensive medical care, efforts to reduce the seizures and design a long-term care plan are of great importance.     

Early-infantile epileptic encephalopathy with suppression-bursts, Ohtahara syndrome; its overview referring to our 16 cases.

Ohtahara syndrome (OS) is characterized by frequent tonic spasms, with or without clustering, of early onset within a few months of life, and a suppression-burst (S-B) pattern in electroencephalography (EEG). Tonic spasms occur in not only waking but also sleeping state in most cases. Partial seizures are observed in about one-third of cases. Brain imagings reveal structural abnormalities including malformations, notably asymmetric lesions in most cases.S-B pattern is persistently observed regardless of circadian cycle. Bursts of 1-3s duration alternate with nearly flat suppression phase of 2-5s at an approximately regular rate; 5-10s of burst-burst interval. Some asymmetry in S-B is noted in about two-thirds of cases. Ictal EEG of tonic spasms shows principally desynchronization with or without initial rapid activity. Tonic spasms appear concomitant with bursts.Characteristic age-dependent evolution from OS to West syndrome (WS) in many cases, and further from WS to Lennox-Gastaut syndrome (LGS) in some, proceed concomitantly with EEG transition from S-B to hypsarrhythmia at around age 3-6 months, and further from hypsarrhythmia to diffuse slow spike-waves at around age 1.Under the inclusive concept of the age-dependent epileptic encephalopathy, OS, WS, and LGS have common characteristics such as age preference, frequent minor generalized seizures, and continuous massive epileptic EEG abnormality. Mutual transition suggests the same pathophysiology among three syndromes and the age factor should be considered as the common denominator responsible for the manifestation of each of their own specific clinico-electrical features. Namely, these syndromes may be the age-specific epileptic reaction to various non-specific exogenous brain insults, acting at the specific developmental stages.