边缘性癫癎和边缘性癫癎状态的临床特点

目的 探讨边缘性癫癎(LE)和边缘性癫癎状态(LSE)的临床特点.方法 回顾分析2例LE和1例LSE患者的临床资料.结果 2例LE癫癎患者均为青年期发病,表现为发作性意识障碍、紧张、恐惧及各种形式的自动症和自主神经症状.脑电图呈现一侧或双侧颞区疒间样放电.头颅MRI示1例左侧海马硬化,1例右颞叶内侧钙化灶.1例LSE患者系中年男性,表现发作性意识障碍、言行紊乱和精神症状,每次发作持续半小时以上;脑电图呈现双侧额叶、颞叶θ波或δ波;头颅MRI示:左颞叶前部蛛网膜囊肿.3例经用卡马西平或奥卡西平治疗发作得到控制.结论 LE和LSE的临床特点是发作性自动症、自主神经和精神症状,与颞叶内侧海马及杏仁核损害有关.     

双侧侧脑室旁结节样灰质异位症4例临床及影像学研究

对4例双侧侧脑室旁结节样灰质异位症(BPNH)患者进行回顾性研究,总结其临床及颅脑MRI特点.BPNH患者颅脑MRI共同特征为双侧侧脑室周围见不规则的小结节样灰质信号.4例患者中,2例灰质结节,呈对称、连续分布,合并小脑发育不良,枕大池扩大；l例结节分布不对称,左侧明显,亦合并小脑发育不良,枕大池扩大；l例结节分布不对称,右侧明显,未见小脑发育不良.2例发作间期动态脑电图正常；另外2例分别于右颞区及左额下区、左颞区见癫痫波.该组患者精神智能发育均正常.均未发现神经系统以外的异常.总体预后良好.BPNH治疗结果和预后因素中经典型双侧侧脑室旁结节样灰质异位症以及合并小脑蚓部发育不良是提示预后良好的因素.     

小儿颞叶癫癎的临床表现及脑电图分析

目的 探讨小儿颞叶癫癎的临床特征及脑电图特点.方法 收集16例颞叶癫癎患儿,对其发作的临床特点及脑电图资料进行分析,对比难治性癫癎的脑电图变化规律.结果 患儿的癫癎发作形式主要表现为单纯部分性发作、复杂部分性发作、继发全面性发作.单纯部分发作频率高而持续时间短,复杂部分发作持续时间长但发作频率低并常见发作后朦胧状态.颞叶癫癎的脑电图特点:背景正常者约占62.5%(10/16);背景异常约占37.5%(6/16);异常放电及部位:颞叶棘波或慢波放电,表现为单侧或双侧同步或不同步放电.结论 小儿颞叶癫癎是一组部分性症状性癫癎综合征,多表现为复杂部分发作,临床发作及同步脑电图特点可为临床诊治提供帮助.     

卡马西平加重癫癎鼠失神发作的机制探讨

目的探讨卡马西平加重癫癎失神发作的机制.方法13周龄雌性GEARS鼠14只分为2组,用微导管在双侧丘脑网状核(Rt)或丘脑腹侧基底核(VB)各注射0.2μL卡马西平(含卡马西平15 nMOL)或溶剂.注射药物后即刻记录90 min脑电图并分析.结果在VB注射卡马西平与注射溶剂相比,脑电图癫癎失神发作的累积时间和频率明显增加(P=0.01,n=7和P=0.03,n=7).而在Rt注射卡马西平组与溶剂组比较,两组间各项指标均无统计学差异.结论在VB注射卡马西平可加重癫癎鼠的失神发作,而注射于Rt对其无影响.提示卡马西平加重癫癎鼠失神发作的神经解剖部位在VB.     

长期应用抗癫癎药控制的颞叶癫癎患者发作间期SPECT异常灌注灶修复与长程视频脑电图变化的临床研究

目的：探讨抗癫癎药（AEDs）长期控制的颞叶癫癎患者发作期间应用单光子发射电子计算机断层扫描（SPECT）检查异常灌注灶的修复。方法：颞叶癫癎患者经AEDs、步长脑心通胶囊与尼莫地平治疗,并在两次发作间期行SPECT、长程视频脑电图（V-EEG）和CT/MRI检查。结果：44例癫癎患者平均年龄为25岁;平均病程为3.5年;44例患者中病因明确者为19例（家族史2例,围产期损伤1例,热性惊厥3例,颅脑损伤出血1例,颅脑外伤5例,脑膜脑炎3例,脑部手术2例,多病因2例）。癫癎发作控制平均为25个月。治疗前发作间期SPECT异常（颞叶和非颞叶）感兴趣区（ROI）为77.3%,长程V-EEG异常77.3%（其中癎样放电88.2%）,治疗前CT/MRI异常47.7%。治疗后发作间期SPECT正常增加4.5%（P〉0.05）,异常灶减少8.3%（P〉0.05）;长程V-EEG正常增加27.3%（P〈0.05）,癎样放电减少53.3%（P〈0.05）;结论：长期控制的颞叶癫癎患者的异常灌注灶修复明显低于其他类型癫癎,防治重点应放在颞叶癫癎症状出现之前的继发性全身强直阵挛发作。     

卡马西平对诱发电位的影响及意义

目的 研究长期口服卡马西平治疗癫癎对诱发电位的影响,并讨论其意义.方法选择尚未治疗的癫癎病人31例作为试验组;以性别、年龄与癫癎组相匹配的健康正常人26例作为对照组.两组先分别做脑干听觉诱发电位（BAEP）、事件相关电位P300、视觉诱发电位（VEP）和体感诱发电位（SEP）,之后癫癎组开始卡马西平治疗,服药一年后再作上述各项检查.结果 癫癎组病人治疗前各项电生理学指标与正常对照组相比无显著性差异;癫癎组卡马西平治疗后各项电生理指标与治疗前相比BAEP各波、P300以及VEP的P100波潜伏期均显著延长;SEP的潜伏期无显著变化.结论 神经电生理学检查可以早期发现长期服用卡马西平导致的亚临床毒性.     

奥卡西平与卡马西平单药治疗儿童部分性癫(癎)的对照研究

目的 比较奥卡西平与卡马西平单药治疗儿童部分性癫(癎)的疗效.方法 71例新诊断的儿童部分性癫(癎)患者按单双号顺序分为奥卡西平组(35例)和卡马西平组(36例),并给予相应的药物治疗;6个月后进行疗效评价,观察不良反应.结果 奥卡西平组和卡马西平组分别有25例及29例完成6个月治疗;脑电图改善率分别为44.0%及44.8%;显效率分别为92.0%及86.2%;不良反应发生率分别为22.2%及41.2%,两组间比较差异无统计学意义(均P>0.05);卡马西平组2例出现严重不良反应.结论 奥卡西平与卡马西平单药治疗新诊断的儿童部分性癫(癎)均有很好的疗效,不良反应及耐受性相似,但奥卡西平组未见严重的不良反应.     

卡马西平加重癫鼠失神发作的机制探讨

目的探讨卡马西平加重癫癎失神发作的机制。方法13周龄雌性CEARS鼠14只分为2组,用微导管在双侧丘脑网状核(Rt)或丘脑腹侧基底核(VB)各注射0．2μL卡马西平(含卡马西平15nMOL)或溶剂。注射药物后即刻记录90min脑电图并分析。结果在VB注射卡马西平与注射溶剂相比,脑电图癫癎失神发作的累积时间和频率明显增加(P=0．01,n=7和P=0．03,n=7)。而在Rt注射卡马西平组与溶剂组比较,两组间各项指标均无统计学差异。结论在VB注射卡马西平可加重癫癎鼠的失神发作,而注射于Rt对其无影响。提示卡马西平加重癫癎鼠失神发作的神经解剖部位在VB。     

良性伴海马硬化的颞叶癫临床特点分析

目的观察伴海马硬化的颞叶癫(TLE-HS)对药物治疗的反应性,分析药物反应良好的良性伴海马硬化的颞叶癫的临床特点。方法 46例颞叶癫患者经MRI证实伴海马硬化,抗癫药物治疗至少随访2年,超过发作周期无癫发作,与51例对抗癫药物耐药的患者比较人口学资料、早期突发损伤因素、癫家族史、临床症状、发作间期脑电图样放电、海马硬化侧别、药物治疗方案等特征,并采用多因素前进法Logistic回归分析筛选药物治疗反应良好的影响因素。结果良性TLE-HS组与对照组患者发病年龄(P=0.041)、病程(P=0.001)、热性惊厥史(P=0.019)、癫发作频率(P=0.001)和药物治疗方案(P=0.000)差异有统计学意义,而性别、年龄、出生史异常、脑炎史、颅脑创伤史、癫家族史、癫持续状态、认知功能障碍、精神障碍,以及发作类型、先兆、是否存在发作间期和发作间期脑电图样放电、海马硬化侧别差异均无统计学意义(P0.05);其中,热性惊厥史是药物治疗反应良好的危险因素(OR=3.405,95%CI:1.080~10.737;P=0.037),而低癫发作频率(OR=0.275,95%CI:0.100~0.758;P=0.013)和单药治疗(OR=0.135,95%CI:0.049~0.373;P=0.000)是药物治疗反应良好的保护因素。结论良性伴海马硬化的颞叶癫多于青少年后期发病,发病初期癫发作频率低,较少伴热性惊厥史,单药治疗特别是卡马西平或奥卡西平疗效较好。     

同步录像脑电图对发作性疾病的诊断价值

目的 探讨同步录像脑电图(VEEG)监测对发作性疾病的诊断和鉴别诊断的临床价值.方法 对常规脑电图(EEG)无(癎)样放电的84例发作性疾病(其中49例临床诊断为癫(癎),35例可疑癫(癎))患者的VEEG资料进行回顾性分析.结果 癫(癎)组VEEG(癎)样放电检出率为80%,可疑癫(癎)组(癎)样放电检出率为31%,80%的(癎)样放电出现在睡眠期.结论 VEEG检测可明显提高(癎)样放电的检出率,为发作性疾病的诊断及鉴别诊断提供重要依据,有助于癫(癎)发作的分型.     

Clinical features and long term outcome of epilepsy in periventricular nodular heterotopia. Simple compared with plus forms

OBJECTIVES: Little is known about the long term outcome of patients with periventricular nodular heterotopia (PNH) and epilepsy, particularly the course of seizures. This study investigated the electroclinical and prognostic features of 16 patients with PNH. METHODS: Of 120 patients with epilepsy and malformations of cortical development, 16 had PNH. Of these, eight patients had periventricular nodules only (simple PNH) and eight also presented with other cortical or cerebral malformations (subcortical heterotopia; polymicrogyria; focal dysplasia; schizencephaly; cortical infolding; agenesis of the corpus callosum; mega cisterna magna and cerebellar atrophy) (PNH plus). All patients underwent clinical, neurophysiological, and MRI investigation. The mean follow up was 17.3 years (2-40 years). RESULTS: Two electroclinical patterns emerged: (1) The first pattern, associated with simple PNH, was characterised by normal intelligence and seizures, usually partial, which began during the second decade of life. The seizures never became frequent and tended to disappear or become very rare. The EEG showed focal abnormalities. (2) The second pattern, associated with PNH plus, was characterised by mental retardation and seizures that began during the first decade of life. The seizures were very frequent in most cases and sudden drops were observed in six patients. Seizures were medically refractory in four patients. The EEG showed focal and bisynchronous abnormalities. CONCLUSIONS: Two groups of PNH patients with different electroclinical and neuroradiological features can be identified after a long term follow up. The presence of other types of cortical or cerebral malformations, in addition to periventricular nodules, determines a poor prognosis.     

Pilomotor seizures: symptomatic vs. idiopathic report of two cases and literature review.

We report pilomotor seizures in two patients who presented with piloerection or gooseflesh spreading in a pattern similar to the 'Jacksonian march'. Gooseflesh was confined to the ipsilateral side in most of the episodes. Occasionally it spread to the contralateral side. It was also associated with other autonomic symptoms and complex partial features of temporal lobe origin. Simple partial status that progressed to complex partial status occurred in the second patient. Very rarely secondary generalization occurred. The cause was left sphenoid meningioma and temporal tip contusion in the first case. It was idiopathic in the second case, although a positive family history of complex partial seizures was obtained in this patient.Interictal electroencephalogram (EEG) showed left temporal focus in the first and bitemporal foci with right fronto-temporal dominance in the second. Parenteral phenytoin controlled the partial complex status in the second and carbamazepine controlled the episodes in both cases. To our knowledge all reported cases were symptomatic and our case of idiopathic aetiology is the first to be recorded. We endorse that pilomotor seizures are autonomic in nature and constitute a subtype of simple partial seizures. These autonomic simple partial seizures may progress to, or be a component of, complex partial seizures of temporal lobe origin. Based on their dominance in such a symptom complex and careful interpretation of the ictal history, it can be logically concluded that pilomotor seizures may be underestimated by both patients and physicians.     

Electrocorticography in temporal lobe epilepsy surgery

The results of intra-operative cortical EEG recordings (ECoG) were assessed in 30 patients with medically refractory complex partial seizures submitted to temporal lobectomy. Interictal spiking was most frequently recorded from mesial temporal lobe structures (amygdaloid-hippocampal region) and from the temporal neocortex synchronously, as observed in 21/30 patients. In 7 patients, the interictal spiking involved the temporal neocortex exclusively. Electrical stimulation of mesial temporal lobe structures reproduced the patient's habitual warning in 10 patients. The results of this study confirms that ECoG provides reliable intra-operative mapping of the epileptogenic brain tissue to be excised.     

Functional interactions in brain networks underlying epileptic seizures in bilateral diffuse periventricular heterotopia.

OBJECTIVE: Our aim was to investigate relationships between heterotopic and remote cortical structures at seizure initiation, in a patient with bilateral periventricular nodular heterotopias (BPNH) explored by intracerebral electrodes. METHODS: Stereoelectroencephalography (SEEG) was performed in a man with BPNH and refractory epilepsy to investigate the hypothesis of right temporal lobe epilepsy and the possible involvement of heterotopic structures during seizures. SEEG signals were analyzed with quantification of functional coupling between different brain structures during seizures, using nonlinear regression. We have used Z-score transformation of correlation values to reflect the change from the preictal period. Relationships between BPNH and cortical structures were investigated using analysis of stimulation-induced potentials. RESULTS: Three spontaneous seizures were recorded and analyzed. Signal analysis of interdependencies in two seizures demonstrated a large initial network involving both heterotopia and cortical structures. Stimulations of heterotopia induced responses in remote cortical structures. CONCLUSIONS: Distinct epileptogenic networks were identified, in which leader structures were either the heterotopic or the mesial temporal structures, with functional connections between heterotopic and cortical areas. SIGNIFICANCE: These results confirm that a vast epileptogenic network, including heterotopic and cortical neurons, may be responsible for seizure generation in BPNH. This may explain certain surgical failures in this group.     

Intracranial EEG findings in patients with lesional lateral temporal lobe epilepsy

PURPOSE: Intracranial EEG in patients with lesional lateral temporal lobe epilepsy is rarely reported. Therefore, the number of patients with seizures arising independently from ipsilateral mesial structures or contralateral hemisphere has not been clarified. We analyzed the intracranial EEG of cases with localized lesion in the lateral temporal cortex. METHODS: We studied 15 patients who satisfied the following criteria: (1) MRI depicted a lesion less than 4cm in diameter located lateral to the collateral sulcus and at least 3cm posterior to the temporal pole; (2) intracranial EEG with electrodes placed on bilateral temporal lobes captured at least one complex partial seizure; and (3) postoperative follow-up period of 2 years or longer. The mean age of seizure onset was 16.6 years (range, 11-25) and that at surgery was 26.7 years (range, 16-36). RESULTS: A total of 147 complex partial seizures, 51 simple partial seizures, 16 secondarily generalized seizures, and over 80 subclinical seizures were recorded. On the lesional side, many clinical seizures were recorded from the lateral cortex. Independent of the lateral temporal onset seizures, ictal discharges originating from the mesial temporal structures were recorded in 7 of 15 patients (47%). Moreover, onset of ictal discharges from the contralateral temporal lobe was recorded in 7 of 15 patients (47%). Interictal spikes from ipsilateral mesial structures were recorded in all patients. The presence of ipsilateral mesial onset seizures was not associated with hippocampal neuron losses. CONCLUSION: Intracranial EEG analysis revealed that approximately one-half of the patients with structural lesions in the lateral cortex showed independent epileptogenic areas in ipsilateral mesial structures. Although ictal discharges originating from the contralateral temporal lobe were recorded in a half of these patients, this finding does not constitute a contraindication of resective surgery. Interictal spike is not an indicator of whether mesial structures should be resected.     

Exacerbation of Partial Seizures and Onset of Nonepileptic Myoclonus with Carbamazepine

A child had two to three generalized tonic-clonic (GTC) seizures per week unresponsive to phenobarbital (PB) and valproate (VPA). Interictal EEG demonstrated left occipital spikes. When carbamazepine (CBZ) therapy was started, he developed very frequent (4-6/day) complex partial seizures (CPS) characterized on ictal EEG by focal right temporal lobe discharges. The seizure exacerbation, which was associated with development of nonepileptic, multifocal myoclonus, resolved 24 h after CBZ was discontinued. The exacerbation occurred with therapeutic CBZ serum levels, but may have been related to the toxic levels of carbamazepine-10, 11-epoxide (CBZE).     

Episodische Epilepsie

We report two patients with episodic seizure clustering with a delayed diagnosis of epilepsy. Patient 1: a 68-year-old woman had a four year history of clusters of complex partial seizures. Repetitive seizures manifested almost continuously for 24 hours every 30 or 31 days. Interictal electro-encephalograms (EEG) showed a normal α-rhythm. An ictal EEG demonstrated rhythmic theta-activitiy over the whole left temporal lobe. Patient 2: a 30-year old man had a four year history of clusters of complex partial seizures once a year. Seizures manifested repetitively over a period of up to four days once a year. Interictal EEGs were normal. MRI showed right hippocampal sclerosis. Ictal EEG demonstrated rhythmic right temporal theta activity.¶???In conclusion, normal interictal investigations in patients with infrequent epileptic seizures or clusters of seizures do not exclude a diagnosis of epilepsy.     

Autonomic seizures in an infant: unusual cutaneous and cardiac manifestations

An eight-month-old infant developed autonomic seizures, manifested by skin reaction (harlequin-like syndrome) and paroxysmal bradycardia. Interictal EEG showed multifocal spikes, 24-hour EEG/ECG monitoring disclosed episodes of cerebral decremental response associated with cardiac nodal rhythm. Head CT and other laboratory tests were normal. Despite excellent seizure control with carbamazepine, the child has moderate psychomotor retardation.     

Effects of seizures and carbamazepine on interictal spiking in amygdala kindled cats

We examined the influence of seizures and carbamazepine (CBZ) on spiking rates in kindled cats. In the first experiment, spiking rates were measured before and after seizures, with and without CBZ. CBZ was administered immediately after seizures in order not to affect them. Spiking rates were measured over 9 h during the different sleep stages. In a second experiment, CBZ was administered before and after seizures so as to affect seizure strength and thus measure its effect on spiking. Results confirmed earlier findings of a large increase in spiking following a stage 6 seizure in fully kindled animals. We also established that: (1) repeated daily seizures caused a further increase in spiking until a ceiling was reached; (2) increased spiking was not a direct effect of postictal alterations in sleep stages; (3) CBZ, despite its effectiveness as an anticonvulsant, did not reduce spiking but rather increased it; (4) postictal increases in spiking were related to seizure 'strength'. These findings support the hypothesis that spiking rates are primarily influenced by seizure occurrence, as was found in patients with temporal lobe seizures, and that anticonvulsants act differently on seizures and spikes. This emphasizes the possibility of distinct pathophysiological mechanisms for interictal spikes and seizures.     

Pharmacologic evidence for abnormal thalamocortical functioning in GABA receptor beta3 subunit-deficient mice, a model of Angelman syndrome

PURPOSE: gamma-Aminobutyric acid receptor (GABA(A)r) subunit beta3-deficient mice model Angelman syndrome by displaying impaired learning, abnormal EEG with interictal spikes and slowing, myoclonus, and convulsions. The beta3-subunit deficiency causes a failure of intrathalamic reticular nucleus inhibition, leading to abnormally synchronized thalamocortical oscillations. We postulated that this pathophysiology underlies the abnormal cortical EEG and triggers interictal spikes and seizures, but extrathalamic regions also contribute to interictal spikes and seizures, so that the EEG slowing should reveal an absence-like response profile, whereas spikes and seizures have dual responsiveness to absence and partial-seizure drugs. METHODS: Recording electrodes were implanted over the parietal cortices of wild-type, heterozygotes, and homozygous null mice. In each experiment, EEG was recorded for 45 min, either drug or vehicle administered, and EEG recorded for another 3 h. Each EEG was scored for slow-wave activity, interictal spikes, and seizures by a reader blinded to treatments. RESULTS: Interictal spiking and percentage of time in EEG slowing in heterozygotes were increased by the proabsence drug baclofen (GABA(B)-receptor agonist), whereas CGP 35348 (GABA(B)-receptor antagonist) had the opposite effect. The antiabsence drug ethosuximide markedly suppressed EEG slowing and interictal spiking in heterozygote and null mice. Broad-spectrum clonazepam and valproate were more effective on interictal spiking than on EEG slowing, and fosphenytoin suppressed only interictal spiking. CONCLUSIONS: The results suggest that this model of Angelman syndrome, although not expressing typical absence seizures, is characterized by hypersynchronous thalamocortical oscillations that possess absence-like pharmacologic responsiveness and promote EEG slowing, interictal spikes, and convulsive seizures.