全面性强直-阵挛发作间期脑血流量改变的磁共振动脉自旋标记研究

目的应用MRI脉冲式动脉自旋标记（PASL）技术,研究全面性强直．阵挛发作患者发作间期局部脑血流量改变,阐述全面性强直一阵挛发作可能的神经病理生理学机制。方法选择29例诊断明确、病程〉1年且发作〉3次的全面性强直一阵挛发作患者作为研究对象,以年龄、性别、利手性相匹配并例数相当的正常健康志愿者作为对照。通过siemens3．0TMRI扫描仪采集全脑PASL数据,通过两独立样本t检验比较全面性强直．阵挛发作患者发作间期相对正常人脑血流量改变的脑区。结果与对照组相比,全面性强直一阵孪发作患者发作间期脑血流量的改变以降低为主（P〈0．05）,主要包括双侧丘脑、脑干和小脑,以及右侧楔前叶部分皮质区域;而且发作间期双侧丘脑局部脑血流量改变与发作时间（r=-0．090,P=0．643）及发作频率（r=-0．115,P=0．551）无明显相关关系。结论全面性强直-阵挛发作患者在发作间期以丘脑、脑干和小脑等脑区血流灌注降低为主,支持特发性全面性癫癎的“中脑癫癎理论”。这些脑区可能与特发性全面性癫癎的病理生理学机制密切相关。     

特发性全面性癫■患者认知损害的研究进展

特发性全面性癫■是一组以全面性发作为主要临床表现的癫■综合征,大多在儿童期至成年早期发病,主要发作类型包括失神发作、肌阵挛发作及全面性强直-阵挛发作。众多近期研究表明,特发性全面性癫■患者可出现多个认知领域的认知损害。文中综述特发性全面性癫■患者认知损害的研究进展。     

发作间期癫(癎)患者脑电非线性分析

目的:通过比较癫(癎)发作间期不同发作类型患者与正常对照组之间脑电非线性特点,探讨非线性脑电图在发作间期癫(癎)患者脑功能评价中的意义及应用价值.方法:对发作间期36例癫(癎)发作患者(其中21例强直阵挛发作患者和15例复杂部分性发作)和32例健康对照组分别行安静闭眼、闭眼心算、安静睁眼3种脑功能活动状态下的脑电图检查...     

不同发作类型癫痫患儿血清神经元特异性烯醇化酶水平研究

目的探讨不同发作类型癫癎患儿血清神经元特异性烯醇化酶水平变化与脑损害之间的关系。方法按照1981年国际抗癫癎联盟制定的癫癎发作类型分类标准,共明确诊断190例癫癎患儿（强直-阵挛发作41例、强直性发作34例、阵挛性发作22例、肌阵挛发作12例、无张力性发作17例、失神发作22例、单纯部分性发作21例及复杂部分性发作21例）,于癫癎发作72 h内施行长程视频脑电图观察和血清神经元特异性烯醇化酶检测。结果不同发作类型癫癎患儿血清神经元特异性烯醇化酶水平均高于正常对照组（P=0.000）,其中以肌阵挛发作组[（32.42±6.62）ng/ml]水平最高,除与强直-阵挛发作组（P=0.062）外,与其他各发作类型之间差异均有统计学意义（P=0.000）;而其他各类型之间差异无统计学意义（均P>0.05）。秩相关分析显示,癫癎患儿血清神经元特异性烯醇化酶水平与长程视频脑电图异常程度呈正相关（r₁=0.613,P=0.000）。结论癫癎发作后血清神经元特异性烯醇化酶水平即升高,提示癫癎发作对患儿脑组织有一定损害;而且癫癎放电对神经元损害越严重、血清神经元特异性烯醇化酶水平升高越明显,不同发作类型中以肌阵挛发作、强直-阵挛发作患儿血清神经元特异性烯醇化酶水平最高,提示这两种发作类型对脑组织的损害高于其他类型。     

有效控制与控制不良的特发性全面性强直阵挛发作癫癎患者局部脑血流的对照研究

目的　探讨有效控制与控制不良的特发性全面性强直阵挛发作癫 (GTCS)患者局部脑血流的差异 ,为评价癫预后和合理治疗提供依据。方法　对 2 9例有效控制的特发性GTCS患者和 12例控制不良的特发性GTCS患者进行发作间期单光子发射计算机断层 (99mTc ECD SPECT)脑血灌流显像 ,用感兴趣区 (ROI)方法对两组的局部脑血流 (rCBF)分布进行半定量分析 ,并将两组患者低局部脑血流的比率 (异常率 )与临床癫发作次数、脑电图 (EEG)进行比较。结果　ROI分析显示 12例控制不良的特发性GTCS患者大脑基底节区和丘脑的rCBF明显低于 2 9例有效控制的特发性GTCS患者大脑的相应脑区血流量 (P <0 0 5 )。有效控制组SPECT脑显像出现低血灌注区的异常率 (1/ 2 9)明显低于控制不良组 (9/ 12 ) ,两组具有显著性差异 (P <0 0 5 ) ;而两组患者的EEG异常率分别为 13/ 2 9、7/ 12 ,差异不具有显著性意义 (P >0 0 5 )。结论　控制不良的GTCS基底节及丘脑局部脑血流降低者明显多于有效控制的GTCS ;特发性GTCS患者发作间期大脑低血灌流区可能是亚临床病灶或是长期癫发作的脑损伤区。     

玩麻将诱发癫癎发作13例患者脑电图分析与临床提示

目的 分析玩麻将诱发癫癎患者发作间期脑电图(EEG)变化的特点及临床意义.方法 回顾性分析2006年7月～2011年3月在本院癫痫 间中心行长程(8～24h)视频EEG监测的13例因玩麻将诱发癫癎患者发作间期的EEG改变.结果 本组中有2例表现为全身强直-阵挛发作的患者发作间期EEG可见双侧大脑半球或双侧中央、额区多量单、连发棘波、尖、棘慢复合波、多棘波慢波发放,给予抗癫痫 间药物治疗,并嘱避免打麻将,未再发作.9例全身强直-阵挛发作和2例部分性发作继发全身性发作的患者发作间期EEG背景活动均出现不同程度的一种或多种异常改变,表现为α节律慢化、反应性及调节性消失、慢波性异常、快波性异常增多、清醒及浅睡眠中6或14Hz阳性棘波、中线θ节律.11例均未给予抗癫痫 间药物治疗,只嘱戒掉麻将并远离麻将环境.其中有1例发作间期EEG呈广泛间断性中-高幅5-7Hzθ节律,1例呈双侧额、中央、颞区阵发性高波幅3～5Hz慢波节律的患者在看家人打麻将、或通宵玩麻将中再次发作.结论 玩麻将诱发的癫癎属于反射性癫癎,虽然戒掉麻将是避免发作的最佳方法,但对于EEG检查有明显癫痫 间样放电,或遵医行为不良,不能彻底摆脱打麻将或麻将环境的患者,则应按癫癎发作类型正规服用抗癫癎药物.同时由于玩麻将诱发癫痫 间发作的刺激因素非常复杂,声光等视觉刺激、思考、躯体感觉、本体感觉、焦虑、惊吓、激动、劳累等刺激因素影响常使大脑处于过度兴奋、乏氧状态,导致EEG背景活动不同程度的改变,在患者情况允许的前提下应定时行长程EEG监测.     

戊四氮点燃猫癫痫形成过程中行为和脑电的动态研究

目的:观察戊四氮点燃猫癫癎形成过程中行为和脑电特征。方法:用低于急性致惊厥剂量的戊四氮(PIZ,25 mg·kg~1)给成年雄性猫每日肌肉注射,观察脑电活动和行为特征。结果:1周后,猫出现发作性面部抽搐,脑电为不对称近额部的散发性棘波,基本节律存在;2周后出现节律性点头,脑电为双侧对称性广泛的散发性棘慢波;16 d后出现发作性四肢强直,脑电活动背景节律变慢,棘慢波呈现阵发性;20 d后出现持续半小时以上的全面性强直阵挛发作,脑电活动为较多不规则的θ和δ波,棘慢波持续时间明显延长。结论:PTZ点燃猫癫癎形成过程中,其行为和脑电阶段性变化明显,与人类癫癎的局灶性继发全面性发作癫癎表现相似。     

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     

癫痫状态的治疗

癫癎状态是指频繁反复或长时间的癫癎发作,造成固定而持续的状态.即使不伴意识障碍,这种连续发作也至少持续30分钟才能归于癫癎状态的概念内.目前把癫状态癎分为:(1)惊厥癫癎状态,即反复强直阵挛发作间期病人不能恢复正常觉醒状态;(2)非惊厥癫癎状态,如失神状态和复杂部分发作状态,临床上表现为延长的"朦胧状态";(3)持续部分发作或称"持续部分性癫癎",此类病人无意识障碍.癫痫状态的危险性强直阵挛癫癎状态是神经科急症,应尽快地制止其发作.如能在60分钟内控制发作则可防止发生严重持久的脑损伤或死亡.以前,病死率为5——     

儿童肌阵挛癫(癎)临床和录像脑电图分析

目的:探讨儿童肌阵挛癫(癎)患儿的临床、脑电图(EEG)和治疗特点.方法:对35例肌阵挛癫(癎)患儿的临床表现、录像脑电图(V-EEG)及抗癫(癎)药物的治疗效果进行回顾性分析.结果:35例均有肌阵挛发作,以肌阵挛为唯一的发作形式9例,其它26例合并强直阵挛发作、强直发作、部分性发作等发作类型.30例患儿EEG可见全导...     

Serum serotonin levels in patients with epileptic seizures

Profound cardiovascular and/or respiratory dysfunction is part of the terminal cascade in sudden unexpected death in epilepsy (SUDEP). Central control of ventilation is mediated by brainstem rhythm generators, which are influenced by a variety of inputs, many of which use the modulatory neurotransmitter serotonin to mediate important inputs for breathing. The aim of this study was to investigate epileptic seizure–induced changes in serum serotonin levels and whether there are potential implications for SUDEP. Forty‐one epileptic patients were pooled into 2 groups based on seizure type as (1) generalized tonic–clonic seizures (GTCS) of genetic generalized epilepsy and focal to bilateral tonic–clonic seizures (FBTCS; n = 19) and (2) focal seizures (n = 26) based on clinical signs using surface video‐electroencephalography. Postictal serotonin levels were statistically significantly higher after GTCS and FBTCS compared to interictal levels (P = .002) but not focal seizures (P = .941). The change in serotonin (postictal‐interictal) was inversely associated with a shorter duration of tonic phase of generalized seizures. The interictal serotonin level was inversely associated with a shorter period of postictal generalized electroencephalographic suppression. These data suggest that peripheral serum serotonin levels may play a role in seizure features and earlier postseizure recovery; these findings merit further study.     

小脑共济失调为主要症状的多系统萎缩患者脑99mTc-SPECT影像学特征分析

目的探讨小脑共济失调症状为主的多系统萎缩(MSA-C)患者的脑局部血流量水平变化规律。方法以在我院神经内科就诊的12例MSA-C患者与25名正常对照者为研究对象,进行锝[~(99m)Tc]双半胱乙酯为显像剂的脑单光子发射计算机断层成像(SPECT)检查以评价脑局部血流量,其SPECT影像以SPM8、xjview9软件进行基于体素的形态学分析(VBM),将与正常对照在脑局部血流量有上统计学差异的区域投射至标准脑模版上,并分析其主要累及脑区、峰值位置、最大t值等特征。结果与正常对照者相比较,MSA-C患者主要出现小脑双侧小脑后叶、小脑蚓部、脑桥的脑局部血流量显著下降(P 0. 01FDR校正,簇水平);未发现有明显统计学意义上的大脑皮质及壳核的脑局部血流量降低。结论 MSA-C患者主要可出现小脑及脑桥的脑局部血流量降低。     

Regional cerebral blood flow in partial complex epilepsy with and without the presence of lesions

Regional cerebral blood flow (r CBF) was measured by the I.V. 133 Xenon method and use of 27 detectors in 91 patients with complex partial epilepsy in interictal periods (at least 48 h over a complex partial seizure). Some were also examined less than 48 h before or after seizures. All were studied with ictal and interictal electroencephalography (EEG), polysomnography, computed tomography (CT), some had nuclear magnetic resonance scans (MR). The blood flow values were compared with a group of a 20 normal subjects matching for age. A significant decrease of r CBF ranged from 15% to 25% was found in the temporal region in three groups of epileptic patients: with repeated normal CT scans and lateralized EEG abnormalities (N = 46); with cortical atrophy in CT scan (N = 12); with neurosurgical focal lesions on CT and or MR scans glioma, arteriovenous malformation) (N = 10). r CBF was normal or decreased by less than 15% in the other regions of the brain. Patients with repeated normal CT scans and bilateral EEG abnormalities either asynchronous or alternatively observed in the right side or left side on waking EEG or during NREM sleep and REM sleep, did not show reduction in r CBF. In a previous study, r CBF distribution was also found normal during interictal phase in patients with primary generalized epilepsy.(ABSTRACT TRUNCATED AT 250 WORDS)     

Patients with West syndrome whose ictal SPECT showed focal cortical hyperperfusion

To clarify the mechanism of clustered spasms in West syndrome (WS), we examined ictal SPECT and EEG, as well as those during the interictal period, in three patients with symptomatic WS who had apparent focal cerebral lesions. For ictal SPECT and EEG, we monitored the patients with EEG, and as soon as we noticed the occurrence of clustered spasms clinically and electroencephalographically, [(99m)Tc]ECD was injected intravenously and flushed thoroughly with saline. In these three patients, regional cerebral blood flow (rCBF) increased during ictus and decreased during the interictal period in the area that coincided with the focal cerebral lesion recognized by CT/MRI. The ictal hyperperfusion of bilateral basal ganglia was also detected in two of the three patients. The ictal EEG showed a diffuse slow wave complex corresponding to a clinical spasm. The sharp waves that preceded the diffuse slow wave complex and the spasm appeared in the same area in which rCBF increased during ictus. None of the patients showed partial seizure before or after clustered spasms clinically or electroencephalographically during the ictal SPECT study. Secondary generalization from a cerebral focus may explain the mechanism of spasms in these patients with WS: focal cortical discharge may primarily generate clustered spasms and trigger the brainstem and basal ganglia structures to produce spasms.     

Cerebral blood flow abnormality in patients with idiopathic generalized epilepsy

Purpose

To investigate the regional cerebral blood flow (rCBF) changes in patients with idiopathic generalized epilepsy (IGE), we compared brain single photon emission computed tomography (SPECT) images of drug naïve IGE patients with those of age/sex matched healthy volunteers.

Methods

Brain interictal ^99mTc-ethylcysteinate dimer SPECT was performed in 21 patients with IGE (M/F = 10/11, 21.3 ± 2.7 years) and 21 normal controls. The seizure types were generalized tonic-clonic seizure in 14 patients and myoclonic seizures with rare generalized tonic-clonic seizures in seven. Differences of rCBF between an IGE group and a normal control group were examined by the statistical parametric mapping (SPM) of brain SPECT images using independent t test.

Results

The SPM analysis showed that the rCBF of the IGE patients was significantly reduced in the anterior and posterior cingulate gyri, bilateral anterior nuclei and right dorsolateral nucleus of the thalamus, right superior colliculus of the midbrain, and the cerebellum at the level of uncorrected p < 0.005. In the small volume correction analysis for the thalamus and brainstem, the rCBF was also significantly decreased in the same brain regions at the level of FDR corrected p < 0.05. No brain regions of the IGE patients had increased rCBF.

Conclusion

Our study demonstrates that the interictal rCBF in drug naive IGE patients is reduced in the cingulate gyrus, thalamus, brainstem and cerebellum. This result suggests that dysfunctions in these brain regions are associated with IGE.

     

Postictal psychosis: A case control series of 20 patients and 150 controls

We compared clinical data, EEG, and video-EEG studies in a consecutive series of 20 patients with postictal psychosis (PP) to 150 consecutive epilepsy patients with complex partial (CPS) or generalized tonic-clonic (GTCS) seizures but without PP. There was a lucid interval between last seizure and onset of psychosis ranging from 2.3 to 72 h (mean, 25 h). Duration of PP ranged from 16 to 432 h (mean, 83 h). Age, sex, epilepsy type (partial vs. generalized), and history of febrile seizures were similar in the PP and control groups. Patients with PP had more frequent GTCS during monitoring than controls (2.8 vs. 1.3; P < 0.001). Patients with PP were more likely to have a history of encephalitis (P < 0.0001) and psychiatric hospitalization (P < 0.002). More patients with PP had bilateral interictal epileptiform discharges during monitoring than controls (P < 0.0002). Postictal psychosis most often develops in patients with bilateral dysfunction following a cluster of GTCS.     

CBF changes in drug naive juvenile myoclonic epilepsy patients

Abstract Purpose The role of thalamus and brainstem in generalized epilepsy has been suggested in previous studies. The aim of the present study was to assess regional cerebral blood flow (rCBF) abnormality in juvenile myoclonic epilepsy (JME) patients. Methods ^99mTc-ethylcysteinate dimer brain single photon emission computed tomography (SPECT) was performed in 19 drug naive JME patients and 25 normal controls with the similar age and gender distribution. Differences of rCBF between a JME group and a normal control group were examined by the statistical parametric mapping of brain SPECT images using independent t test. The regression analyses in SPM were also performed between rCBF and the age of seizure onset or the disease duration in JME group. Results Compared to normal controls, the JME group showed a significant rCBF reduction in bilateral thalami, red nucleus, midbrain, pons, left hippocampus, and in the cerebelli (FDR corrected p < 0.01) whereas rCBF increase in the left superior frontal gyrus (uncorrected p < 0.001 but FDR corrected p > 0.05). Disease duration was negatively correlated with rCBF in bilateral frontal cortices, caudate nuclei, brainstem and cerebellar tonsils. Conclusions Our results suggest that abnormal neural networks in the thalamus, hippocampus, brainstem and cerebellum are associated with JME.     

Regional cerebral blood flow and distribution of [99mTc]ethyl cysteinate dimer in nonhuman primates

Increases in regional cerebral blood flow have been described in a variety of cerebral pathologic states, including stroke and seizure disorders. The usefulness of technetium-99m-labeled cysteinate dimer as a marker in the measurement of regional cerebral blood flow was tested in five cynomolgus monkeys. To expand the range of blood flow to beyond the normal limits, 40 mg/kg i.v. of the carbonic anhydrase inhibitor acetazolamide was administered. Regional cerebral blood flow in all five monkeys was measured using radiolabeled microspheres (before and after acetazolamide) and the marker (after acetazolamide) in 60-70 samples from 12 brain regions. Acetazolamide significantly increased the mean +/- SEM regional cerebral blood flow measured using microspheres from 0.56 +/- 0.21 to 1.71 +/- 0.9 ml/min/g (p less than 0.01 for each region). A significant positive correlation was found between regional cerebral blood flow values calculated using microspheres and the marker after normalizing the values to those in the cerebellum (r = 0.773, p less than 0.0001). The mean +/- SEM regional cerebral blood flow determined using the marker in a single monkey (1.21 +/- 0.04 ml/min/g) did not differ significantly from that determined in the same monkey using microspheres (1.13 +/- 0.04 ml/min/g). These data support the potential use of this new brain perfusion imaging agent to assess regional cerebral blood flow over a clinically relevant range of blood flows.