中国农村地区全身强直-阵挛发作癫癎患者的认知功能评估

目的：评价中国农村地区未经抗癫癎药物（AEDs）治疗的全身强直-阵挛发作（GTCS）癫癎患者的认知功能的状况.方法：采用数字广度测验、言语流畅性测验、听觉词语测验及数字划消测验对144例GTCS患者和144名健康对照者进行认知功能评定.结果：GTCS患者的注意力及计算能力较差,并有瞬间语言记忆、自发的语言运动能力、思维组织能力及干扰抑制能力的下降.结论：未经AEDs治疗的GTCS患者存在广泛的认知功能障碍.AEDs的非规范使用是认知功能损害的主要影响因素.     

A Trial of Discontinuation of Barbiturates in Patients with Secondary Generalized Epilepsy

Abstract: The vast majority of patients with secondary generalized epilepsy (SGE) were under polytherapy including barbiturates. We were able to completely withdraw barbiturates in 17 cases with a refractory course of SGE.
Valproic acid (VPA) is considered as one of the best drugs for SGE. Barbiturates decrease the serum level of VPA and this may lead to a reduced efficacy of VPA. The dosage of VPA was adjusted to attain the level of 100-150 ju.g/ml. Barbiturates were then gradually decreased and finally discontinued. After six months of follow-up, 13 patients (76%) showed a reduced seizure frequency. Also, the physical and psychological conditions improved in a good number of cases. Barbiturates are not necessarily required in pharmacotherapy of SGE and may be safely withdrawn.     

Seizure generation: The role of nodes and networks

The longstanding dichotomy between the concepts of "focal" and "primary generalized" epilepsy has become increasingly blurred, raising fundamental questions about the nature of ictal onset in localized brain regions versus large-scale brain networks. We hypothesize that whether an EEG discharge appears focal or generalized is driven by the pattern of connections in brain networks, irrespective of the presence of focal brain abnormality. Using a computational model of a simple "brain" consisting of four regions and the connections between them, we explored the effects of altering connectivity structure versus the effects of introducing an "abnormal" brain region, and the interactions between these factors. Computer simulations demonstrated that electroencephalography (EEG) discharges representing either generalized or focal seizures arose purely as a consequence of subtle changes in network structure, without the requirement for any localized pathologic brain region. Furthermore we found that introducing a pathologic region gave rise to focal, secondary generalized, or primary generalized seizures depending on the network structure. Counterintuitively, we found that decreasing connectivity between regions of the brain increased the frequency of seizure-like activity. Our findings may enlighten current controversies surrounding the concepts of focal and generalized epilepsy, and help to explain recent observations in genetic animal models and human epilepsies, where loss of white matter pathways was associated with the occurrence of seizures.     

Impact of Cardiac-Based Vagus Nerve Stimulation Closed-Loop Stimulation on the Seizure Outcome of Patients With Generalized Epilepsy: A Prospective,Individual-Control Study

ObjectivesWe designed a prospective, individual-controlled study to evaluate the effect of cardiac-based VNS (cbVNS) in a cohort of patients with generalized epilepsy (GE).Materials and MethodsTwenty patients were included. They were followed up for six months under regular VNS (rVNS) and subsequently for six months during cbVNS. Stimulation parameters were 500 μsec, 30 Hz, and up to 2.5 mA. Seizure frequency was documented after two, four, and six months during the rVNS and cbVNS phases. Patients with at least 50% seizure frequency reduction were considered responders. The total and relative amount of stimulation cycles generated by both rVNS and cbVNS activation were documented. Findings during rVNS were compared to baseline and cbVNS data were compared to those during rVNS.ResultsThere was a significant decrease in mean seizure frequency (61% [95% CI, 48-74]; p < 0.001) during the rVNS phase compared to baseline. There was no additional significant (16% [95% CI, 4-35]; p = 0.097) mean seizure frequency reduction during cbVNS compared to the rVNS phase. Fifteen patients (75%) were considered responders after rVNS. Four patients (20%) were considered responders after six months of cbVNS. During the cbVNS phase, the mean total number of cycles/day was 346, 354, and 333 for months two, four, and six, respectively; the cycles generated by rVNS were 142, 138, and 146 for months two, four, and six, respectively; and cycles generated by cbVNS were 204, 215, and 186 for months two, four, and six, respectively. There was no relationship between the mean total number of cycles (−6[95% CI, −85 to 72]; p = 0.431), the mean number of auto-stimulation cycles (27[95% CI,−112 to 166]; p = 0.139), the mean number of regular cycles (−33[95% CI,−123 to 57]; p = 0.122), or the mean percentage of auto-stimulation cycles (13[95% CI,19- 45]; p = 0.109) and outcome during the cbVNS phase. Eight patients showed some decrease in seizure frequency during cbVNS.ConclusionsrVNS was effective in reducing seizure frequency in patients with generalized epilepsy, but activation of the cbVNS feature did not add significantly to rVNS efficacy. On the other hand, although not statistically significant, 40% of the patients showed some reduction in seizure frequency, which might prove useful at an individual level.     

新发病的特发性全面强直阵挛发作患者局部脑血流观察

目的 :探讨特发性全面强直阵挛发作 (generalizedtonicclonicseizure ,GTCS)癫患者发作间期局部脑血流 (rCBF)特征。方法 :用单光子发射计算机断层显像 ( 99Tcm ECD SPECT)技术对 2 0例新发病的特发性GTCS患者发作间期rCBF特征进行观察 ,以兴趣区(ROI)法作半定量分析。结果 :2 0例新发病的特发性GTCS患者发作间期rCBF异常率为 3 0 % ( 6例 ) ,主要表现为一侧丘脑区或基底节区的放射性稀疏。结论 :新发病的特发性GTCS患者的rCBF存在两种特征 :①正常的CBF ;②一侧丘脑区CBF降低 ,CBF降低的丘脑区可能为亚临床病灶     

Considerations on the Significance Attributed to Unusual Cerebral Histological Findings Recently Described in Eight Patients with Primary Generalized Epilepsy

The authors, although they express appreciation of the importance of publishing careful anatomical reports in cases of epilepsy, express some doubts concerning the pathological nature of the histological features reported by Meencke and Janz in the brains of eight patients suffering from primary generalized epilepsy. As illustrated and described, most of the histological findings considered abnormal are commonly seen in neurologically normal controls. Also, true "minor dysgenetic changes" have been observed in individuals free of cerebral disorders. Furthermore, the hypothesis drawn from the neuropathological observations to explain the etiopathogenesis of this category of epilepsy and the physiopathogenesis of the seizures does not seem acceptable to the authors.     

EEG of a Nocturnal Seizure in a Patient with "Benign Epilepsy of Childhood with Rolandic Spikes"

The first EEG recording is reported of a child with "benign epilepsy of childhood with Rolandic spikes" during a partial motor (facial) seizure, which occurred during Phase II of natural sleep.     

149例顽固性癫痫皮质脑电图监测下痫灶切除手术的临床分析

目的 总结皮质脑电图监测下痫灶切除手术治疗顽固性癫痫的临床疗效。方法 回顾性分析149例经皮质脑电图监测下痫灶切除手术治疗的149例顽固性癫痫患的临床资料，并结合随访资料讨论痫灶定位、手术要领、适应症及疗效判定标准。结果 随访11个月～21年，按Engle标准对术后疗效进行判断，I级31．5％，Ⅱ级25．5％，Ⅲ级28．2％，Ⅳ级14．8％。手术总有效率为85．2％，无手术死亡及严重并发症发生。结论 皮质脑电图监测下痫灶切除手术安全、有效，可为治疗顽固性癫痫的首选方案。     

Failure of Naloxone to Modify Electroencephalogram Interictal Epileptiform Discharges in Patients with Primary Generalized Epilepsy After Sleep Deprivation

Summary: Sleep deprivation (SD) is a method widely used to activate EEG epileptiform activity, but the basis of this effect remains unknown. One possibility is that SD shares a common mechanism with physical and psychological stresses that also precipitate seizures. Because endogenous opioids are released during stress, opioids may play a role in enhancing epileptiform EEG patterns after SD. We report the effects of SD on EEG epileptiform activity in a small but highly homogeneous population of 13 epileptic patients with idiopathic (primary) generalized epilepsy (IGE). SD increased EEG interictal epileptiform discharges (IEDs); this activation was not modified by naloxone (NAL). Our results, in contrast to those of previous investigations of localization-related epilepsy, which showed an increase in IEDs after NAL administration, suggest a possible difference in the mechanism whereby SD enhances IEDs in IGE and localization-related epilepsy.     

癫痫复杂部分发作患者血清髓鞘碱性蛋白含量研究

目的　探讨癫痫复杂部分发作 (ECPS)患者血清髓鞘碱性蛋白 (MBP)水平及其与癫痫发作的相关性。方法　用酶联免疫吸附测定法对 34例 ECPS患者及 2 8例精神分裂症 (SP)患者及 32名正常人血清 MBP进行检测。结果　 ECPS组血清 MBP含量明显高于 SP组及正常组 (P<0 .0 1) ,且血清 MBP含量与癫痫发作频繁、近周内发作、脑电图及脑 CT/ MRI异常有关 (P<0 .0 5)。结论　提示 ECPS可能与髓鞘损害有关。     

Brain Distortions in Patients with Primarily Generalized Tonic-Clonic Seizures

Summary: Purpose: A precondition for the diagnosis of primarily generalized epilepsy with tonic-clonic seizures (GTCS) is absence of brain pathology. This definition, based on normal findings on computed tomography (CT) and magnetic resonance imaging (MRI) is challenged however, by observation of microscopic migrational disturbances in patients with GTCS. In the present study, we examined whether hitherto undiscovered gross manifestations of the reported migrational disturbances may be detected by analysis of CT and MRI scans with a computerized anatomic brain atlas.
Methods : The atlas program permitted group comparisons of size, intrinsic proportion, and shape of the brain. Healthy men (n = 20), patients with partial seizures (n = 8), secondarily generalized partial seizures (n = 8), and patients with GTCS (n = 10) were studied. The contours of the brain of the computerized atlas were first transformed and adjusted to the contours, central structures, and ventricles of each subject's MRI scans. During this process, the specific parameters for the shape, size, and proportion of the brain were determined, resulting in a set of values for each subject. These values were then applied for comparisons between the four investigated groups.
Results : In relation to the controls, patients with GTCS had brains significantly flattened in the craniocaudal direction (p = 0.002), with a disproportionally small caudal part. The anterior portion of their brain was also, relatively elongated as compared with the posterior portion (p = 0.04). Similar systematic abnormalities were not observed in patients with partial epilepsy.
Conclusions : The observed deformations are compatible with previously reported findings of Purkinje cell degeneration and frontal lobe microdysgenesis in GTCS. The study suggests a new approach to identify effects of morphologic abnormalities in the brain when results of conventional structural neuroimaging are normal.     

Status of Follow-Up among Patients with Epilepsy in Epilepsy Clinic

Abstract: Out of 238 :consecutive patients entering our epilepsy clinic from October 1988 till March 1990, the status of follow-up was evaluated in October 1990. One hundred patients (42%) could adhere to the follow-up appointment and 33 :patients (14%) visited the clinic irregularly. A total of 105 :patients (44%) failed to return for the follow-up at least for 2 :months dated backward from October 1990. Seventy-five percent of the patients lost the follow-up within 6 :months. The main reasons of the dropout were poor seizure control, seizure free, denial of epilepsy, receiving operation in other hospitals. The appropriate strategies to aid in adherence to medical care among patients with epilepsy should be formulated through the analysis of factors contributing to the dropout, which might be strongly influenced by different medical and sociocultural backgrounds all over the world.     

Frequency of Automatic Stimulations in Responsive Vagal Nerve Stimulation in Patients With Refractory Epilepsy

BackgroundIn vagal nerve stimulation (VNS) therapy, the release of VNS model 106 (AspireSR) allowed for responsive VNS (rVNS). rVNS utilizes a cardiac-based seizure detection algorithm to detect seizure-induced tachycardia to trigger additional stimulation. There are some studies suggesting clinical benefits of rVNS over traditional VNS, but the performance and significance of autostimulation mode in clinical practice are poorly understood.ObjectivesTo assess the effect of initiation of rVNS therapy and altered stimulation settings on the number of daily stimulations and energy consumption in VNS therapy and to compare autostimulation performance in different epilepsy types.Materials and MethodsRetrospective follow-up of 30 patients with drug-resistant epilepsy treated with rVNS including 17 new implantations and 13 battery replaces at a single center in Finland. Our data consist of 208 different stimulation periods, that is, episodes with defined stimulation settings and both autostimulation and total stimulation performance-related data along with clinical follow-up.ResultsThe variation in autostimulation frequency was highly dependent on the duration of the OFF-time and autostimulation threshold (p < 0.05). There was a large additional effect of autostimulation mode on therapy time and energy consumption with longer OFF-times, but a minor effect with shorter OFF-times. Significantly more autostimulations were triggered in the temporal lobe and multifocal epilepsies than in extratemporal lobe epilepsies.ConclusionsThe initiation of autostimulation mode in VNS therapy increased the total number of stimulations. Shortening the OFF-time leads to a decreased number and share of automatic activations. Epilepsy type may affect autostimulation activity.     

Therapy-resistant epilepsy in children: with the view of the long-term outcome

Of 362 children with therapy-resistant epilepsy, 119 patients had generalized tonic seizures, which were the most common seizure type in patients with generalized epilepsy. The long-term observation of the patients with tonic seizures revealed that their seizures were completely controlled in 27 patients for more than 5 years. The effective antiepileptic drugs were VPA (10 patients), PHT (5), CLB (4), ZNS (2), VPA + PHT (4), ZNS + CLB (1) and VPA + AZA (1). For obtaining improvement of tonic seizures, another strong factor is reducing the number of antiepileptic drugs, such as the average number of drugs, which was 4.7 to 2.4 in these 27 patients.