脑沟皮层内小病灶致症状性癫痫患者的脑电图特点及外科治疗(附11例报告)

目的探讨脑沟皮层内小病灶致症状性癫痫患者的脑电图特点及外科治疗效果。方法回顾性分析11例脑沟皮层内小病灶致症状性癫痫患者的脑电图、手术方法及其疗效等临床资料。结果此类患者发作起始形式的头皮脑电图特点是病灶附近区域节律性3～4Hzδ波持续数秒后转为5～6Hz节律性θ波。所有患者均行手术切除病灶加皮层热灼术。术后随访1～3年,按Engel分级,Ⅰ级10例,Ⅱ级1例。随访期间所有患者没有癫痫再发作。结论手术切除病灶加皮层热灼术治疗脑沟皮层内小病灶致症状性癫痫可获得良好效果。     

大脑半球切除治疗难治性癫痫的术前评估及手术改良(附58例报告)

目的 研究大脑半球切除治疗由一侧大脑半球广泛病变引起的难治性癫痫的术前评估及手术改良.方法 回顾性分析清华大学玉泉医院癫痫中心2006年11月至2011年7月58例行大脑半球切除治疗癫痫病例.根据患者的临床发作、影像学及脑电图特点进行癫痫起源一侧半球的定侧;根据功能磁共振、磁共振弥散成像、PET -CT等进行术前神经功能分析及术后神经功能预测;选择制定合适的半球切除手术计划.结果 58例均行一侧大脑半球切除,32例左侧半球,26例右侧半球,其中5例行保留运动区的大脑半球切除.无手术死亡,4例少量术腔出血,5例切口缺血愈合不良并脑脊液漏.随访1年至5年8个月,48例术后癫痫无发作;10例术后有程度不等的发作.按照Engle评分Ⅰ级48例(83％);Ⅱ级7例(12％);Ⅲ级3例(5％).结论 改良大脑半球切除对于一侧半球病变引起的难治性癫痫控制效果及脑功能代偿良好,无严重并发症,术后停药复发率低.     

发笑性癫痫的研究进展

发笑性癫痫（Gelastic seizure,GS）是一种不合时宜的以爆发性发笑为主要临床特点,伴或不伴其他癫痫性发作事件的癫痫发作类型,按症状出现时间、有无情绪改变及意识障碍情况分为单纯性和非单纯性发笑发作。笑的产生的神经通路主要分为情感和意志两大通路,其神经网络涉及了额叶、岛叶、边缘叶、颞叶、脑干等相关神经结构。GS最常见的病因为下丘脑错构瘤,立体定向脑电图可以记录到病变本身的放电,手术切除下丘脑错构瘤可以使大多数患者GS发作立即消失,少数患者症状得到部分改善其头皮脑电图仍可监测到癫痫样放电,尽早手术治疗可能改善预后。非下丘脑源性GS,影像学检查无明显异常者,脑电图多为局灶性放电并且对抗癫痫发作药物治疗效果较好,而影像学提示结构异常的患者多为多灶性放电且对药物治疗效果较差,少数药物难治性非下丘脑源性的GS手术治疗可以有效控制症状。本文对GS这一个较为少见的癫痫发作类型的病因、涉及的神经网络、脑电特点、治疗选择进行综述,以期提高对该病的认识。     

大脑半球多脑叶离断术治疗难治性癫痫的疗效

目的 探讨大脑半球多脑叶离断术治疗难治性癫痫的手术经验及疗效。方法 回顾性 分析2013 年4 月—2017 年7 月北京功能神经外科研究所采用大脑半球多脑叶离断术治疗难治性癫痫患 者共22例,介绍手术方式,观察术后病理及疗效,记录并发症情况。结果 Engel Ⅰ级患者15例（占68%）, Engel Ⅱ级患者6例（占27%）,Engel Ⅲ级患者1例（占5%）。术后出现肢体功能障碍较术前加重的患者4 例, 1 例患者出现颅内感染,无一例出现含铁血黄素沉积症。结论 大脑半球多脑叶离断术治疗半球病变 导致的难治性癫痫有较好的疗效,尤其适合那些运动、感觉、语言功能仍然存留在患侧的难治性癫痫患者。     

枕叶癫痫外科治疗的特点与疗效(附13例报道)

目的 探讨枕叶癫痫外科治疗的特点与手术疗效.方法 回顾分析13例枕叶癫痫患者完整的临床资料,总结其发作症状学、影像学、EEG、神经病理学及手术治疗等方面的特征与手术疗效.结果 局灶性皮质发育不良为最常见病理表现(6例).术后2周时6例患者原有视觉障碍加重或新出现视觉障碍.术后随访2～5年,Engel's I级7例(53.8%),II级1例,III级2例,IV级3例.结论 定位明确的枕叶癫痫可以通过适当的手术治疗获得较满意疗效,但常常加重视觉功能障碍.     

伴有丛集发作的儿童癫痫患者的手术治疗

目的 探讨伴有丛集发作的儿童癫痫患者的手术评估及疗效。方法 回顾性分析33例伴有丛集发作的儿童癫痫患者的临床资料,其中缓解性手术治疗8例,切除性手术治疗25例。结果 术后随访8~42个月,疗效按Engel分级:缓解性手术治疗患者Ⅱ级2例,Ⅲ级4例,Ⅳ级2例;切除性手术治疗患者Ⅰ级16例,Ⅱ级4例,Ⅲ级3例,Ⅳ级2例。无手术相关永久性并发症发生。结论 对于伴有丛集发作的儿童癫痫患者,应尽早进行综合的术前评估并采取相应的手术治疗以获得更好的控制疗效,从而改善患者预后。     

起源于颞叶后外侧皮质癫痫的临床特征和手术疗效

目的分析起源于颞叶后外侧皮质癫痫的临床特征和手术疗效。方法回顾性纳入首都医科大学附属北京天坛医院神经外科2015年3月至2017年12月收治的10例起源于颞叶后外侧皮质癫痫患者。所有患者均行致痫灶切除术,分析起源于该部位癫痫的临床特征,观察手术疗效。结果10例患者平均起病年龄为(14.0±7.6)岁,平均手术年龄为(18.8±6.0)岁,癫痫平均病程为(5.1±3.3)年。所有患者MRI检查病灶均为阳性。发作症状学中,4例患者有自动症表现,包括口咽部自动症和双上肢摸索症状,其中3例出现在发作早期;6例患者表现为偏转发作,均出现于发作早期,其中4例偏转方向与病灶侧别一致。致痫灶切除术后,2例患者术后1个月偶尔出现癫痫发作,3个月后完全消失;所有患者术后平均随访(18.6±5.3)个月,癫痫发作均完全消失(国际抗癫痫联盟分级1级)。结论偏转发作是起源于颞叶后外侧皮质癫痫患者的常见发作症状,部分患者也可出现颞叶内侧癫痫发作表现。临床诊断需要结合症状学、电生理学及影像学综合评估,单纯病灶切除可获得良好的预后。     

大脑半球萎缩继发癫痫患者术后生活状况分析

目的研究大脑半球萎缩继发癫痫患者手术后生活状况改善情况。方法对2010年3月至2013年12月手术治疗的12例大脑半球萎缩继发癫痫患者手术前后的生活质量、精神症状、日常生活能力比较并进行追踪随访,应用患者生存质量专用量表(QOLIE-31)、精神症状自评量表(SCL-90)、和日常生活能力量表(ADL)测评,并进行统计学分析。结果 12例患者术后随访13~44个月,平均随访时间中位值18.6±5.3月,7例患者无癫痫发作,达到Engle's I级,5例患者Engle'sⅡ级。生活质量、精神症状、日常生活能力方面均有明显改善,与术前各项指标行统计学对照比较(P<0.05)。结论大脑半球萎缩继发癫痫患者应及早手术治疗,术后不但能够良好的控制癫痫发作,还能全面提升患者生活质量、精神症状、日常生活能力。     

胚胎发育不良性神经上皮肿瘤致癫痫的手术治疗

目的 探讨胚胎发育不良性神经上皮肿瘤(DNT)致癫痫患者的手术策略.方法 回顾性分析自2008年1月至2013年8月首都医科大学宣武医院神经外科手术治疗14例DNT患者的经验,术前常规通过MRI及视频脑电图(VEEG)等检查对肿瘤及致痫灶进行评估,术中行皮层脑电监测(EcoG)对致痫灶定位,手术方式:肿瘤病灶加致痫灶切除.结果 14例患者肿瘤均得到全切,手术后病理证实为DNT,未行放疗和化疗,无肿瘤复发及恶性转化.10例患者术后癫痫发作得到完全控制(Engel Ⅰ级),4例患者术后稀少发作(EngelⅡ级).结论 DNT是低级别神经元～胶质细胞肿瘤,常见于青少年,多伴药物难治性癫痫,除切除肿瘤外,积极处理肿瘤外致痫灶可以很好地控制癫痫.     

功能性大脑半球切除治疗婴儿偏瘫伴顽固性癫痫(附2例报道并文献复习)

目的探讨功能性大脑半球切除治疗婴儿偏瘫伴顽固性癫痫的手术方法及术后疗效。方法报道功能性大脑半球切除治疗2例婴儿偏瘫伴顽固性癫痫,结合文献复习,介绍其临床表现、手术方法及术后疗效。结果术后随访发现2例患者癫痫发作均消失,其中1例已停用抗癫痫药物治疗。结论功能性大脑半球切除是治疗婴儿偏瘫伴顽固性癫痫的一种有效方法,术后患者癫痫发作一般均控制满意,且患者的行为得到改善,神经功能缺失症状也未加重。     

A case of frontal lobe epilepsy presenting with gelastic seizures]

We described a 9-year-old boy with frontal lobe epilepsy presenting with gelastic seizures. CT-scan showed mild widening of the left sylvian fissure. Abnormal findings in the left frontal operculum were detected by both MRI and SPECT. Attacks mainly consisted of gelastic seizures with comfortable feeling followed by screaming with fear. Administration of anticonvulsants resulted in reducing the frequency and severity of seizures. Finally the patient had brief laughter attacks only. In the present case, the clinical course suggests that the gelastic seizures does not occur by way of the spreading of epileptic discharges to the temporal or hypothalamic region; rather it might occur as a focal symptom of the frontal region.     

Gelastic seizures involving the left parietal lobe

Gelastic seizures have been described in various epilepsies arising from the temporal or frontal lobes, although the most commonly encountered form is related to the presence of a hypothalamic hamartoma. We describe a patient with gelastic seizures involving the left parietal lobe. Our patient, an 8-year-old girl, underwent interictal video/EEG monitoring and MRI. The seizures consisted of brief staring followed by smiling and laughing. Electroencephalography during the gelastic seizures showed rhythmic spikes and waves in the left parietal lobe. MRI revealed the characteristic features of focal cortical dysplasia. Our findings suggest that the left parietal lobe may actively participate in the particular epileptogenic network generating gelastic seizures.     

Gelastic seizures involving the right parietal lobe.

Gelastic seizures have been described in various epilepsies arising from the temporal or frontal lobes, although the most commonly encountered form is related to the presence of an hypothalamic hamartoma. We report a patient with gelastic seizures involving the right parietal lobe. Our patient, a 32-year-old man, underwent video-EEG monitoring, interictal and ictal brain SPECTs during gelastic seizures. Subtraction ictal SPECT co-registered to MRI (SISCOM), was performed to localize any ictal hyperperfusion during these gelastic seizures. The seizures consisted of brief staring followed by smiling and laughing. Electroencephalography during the gelastic seizures showed rhythmic sharp waves in the right parietal lobe. SISCOM showed ictal hyperperfusion in the right parietal lobe and medial portions of right cerebellum. Our findings suggest that the right parietal lobe may actively participate in the particular epileptogenic network generating gelastic seizures.     

A note on gelastic epilepsy

Laughter epilepsy or gelastic seizures have been described in various epilepsies arising from the temporal or frontal lobes, but most commonly from hypothalamic hamartomata. Gelastic seizures also arise from temporal and frontal lobe tumours and atrophic lesions. The essential clinical features are: stereotyped recurrence; absence of external precipitants; concomitance of other manifestations generally accepted as epileptic; presence of interictal or interictal EEG epileptiform discharges, and absence of conditions in which pathological laughter might occur. The history and clinical significance are discussed.     

Shift-back of right into left hemisphere language dominance after control of epileptic seizures: evidence for epilepsy driven functional cerebral organization

Atypical, i.e. right hemisphere language dominance is frequently observed in early onset left hemisphere epilepsies. In left mesial temporal lobe epilepsy, where eloquent cortex is not directly involved, it is a matter of debate, to which degree atypical language dominance is driven not only by morphological lesions but also by epileptic dysfunction, and whether atypical dominance is hardwired or not. Taking this as the background this study evaluated the hypothesis that epilepsy driven atypical dominancy might be reversible when seizures are successfully controlled. This was evaluated in patients with left mesial temporal lobe epilepsy, who were atypically language dominant by means of language fMRI before surgery, and became seizure free after left selective amygdalo-hippocampectomy. Three out of 53 consecutive atypically dominant patients with chronic epilepsy fulfilled these criteria. Postoperative follow-up language fMRI indicated reversal of right into left dominance in one patient going along with unexpected losses in verbal memory performance. The two other patients experienced unchanged or even enhancement of the pre-existing dominance pattern, going along with consistent postoperative performance changes in cognition. The data thus provide supporting evidence that atypical language dominance can indeed be functionally driven and moreover that in at least some patients, right hemispheric language can shift-back to the left hemisphere when the driving factor, i.e. seizures, becomes successfully controlled. The results have clinical implications for outcome prediction after brain surgery in atypically dominant patients with epilepsy. However, further research in larger groups of atypically dominant patients is required to identify the conditions under which atypical dominance becomes hardwired and when not.     

Gelastic seizures: Incidence,clinical and EEG features in adult patients undergoing video‐EEG telemetry

This study aimed to determine clinical features of adult patients with gelastic seizures recorded on video –electroencephalography (EEG) over a 5‐year period. We screened video‐EEG telemetry reports for the occurrence of the term “gelastic” seizures, and assessed the semiology, EEG features, and duration of those seizures. Gelastic seizures were identified in 19 (0.8%) of 2,446 admissions. The presumed epileptogenic zone was in the hypothalamus in one third of the cases, temporal lobe epilepsy was diagnosed in another third, and the remainder of the cases presenting with gelastic seizures were classified as frontal, parietal lobe epilepsy or remained undetermined or were multifocal. Gelastic seizures were embedded in a semiology, with part of the seizure showing features of automotor seizures. A small proportion of patients underwent epilepsy surgery. Outcome of epilepsy surgery was related to the underlying pathology; two patients with hippocampal sclerosis had good outcomes following temporal lobe resection and one of four patients with hypothalamic hamartomas undergoing gamma knife surgery had a good outcome.     

Surgical treatment of extra‐hypothalamic epilepsies presenting with gelastic seizures

We provide an overview of the surgical outcome of extra‐hypothalamic epilepsies with gelastic seizures based on an original case report and a summary of the literature. Twenty‐two articles providing information on the outcome of resective surgery in 39 patients with extra‐hypothalamic gelastic seizures from the temporal (19 patients) or frontal lobe (20 patients) were selected. We add another case of temporal lobe gelastic seizures to the literature with a video demonstrating the mirthful component of this patient's laughing seizures. Drug‐refractory cases of gelastic seizures from the temporal or frontal lobes are amenable to surgical treatment following thorough investigation with imaging, as well as scalp and intracranial EEG.     

An unusual case of epilepsy exhibiting gelastic seizure, simple visual hallucination, and transient swelling of the left parieto-occipital region]

We report a 74-year-old man with gelastic seizure, simple visual hallucination, and adversive seizure. The patient described his visual hallucinations as "rotating light like a firefly" and "mimicking a stream". Brain CT scan showed a transient swelling as well as low density of a left parieto-occipital region. Electroencephalographic study revealed spikes and fast waves beginning at left occipital region. Although temporal lobe and hypothalamic lesions (especially hypothalamic hamartomas) are well known as origins of gelastic seizures, we could not find any report that described a series of occurrence of gelastic seizure and simple visual hallucination. Usually, simple visual hallucination is thought to occur in occipital lesion. In our case, it is possible that gelastic seizure and simple visual hallucination are related to the epileptic discharge from occipital lesion directly or indirectly. The reversible brain swelling with low density seen in the present case might be caused by cytotoxic edema due to status epilepticus.     

Clinical Profile of Partial Seizures Beginning at Less Than Four Years of Age

Clinical features of all our 46 patients whose recurrent partial seizures began at age less than or equal to 47 months were studied. Thirty-seven (80%) had motor seizures which were exclusively unilateral in 14 patients, bilaterally synchronous in 8, and both unilateral and bilaterally synchronous in 15. Thirty-three patients (72%) had complex partial seizures (CPS), and 11 patients (24%) had simple partial somatosensory seizures. Clinical seizures were recorded by EEG in 18 patients (39%), and a subclinical (larval) seizure was recorded in 1; those in 10 patients arose from a temporal lobe, 4 were from a frontal lobe, and in 5 they involved two adjacent lobes from onset. Several extra-ictal features reliably indicated lobe or hemisphere of seizure onset. The lobe of principal interictal EEG spikes corresponded to seizure origin in 33 of 34 patients (97%), and regional delta activity corresponded to lobe of seizure origin in 22 of 24 patients (92%). The seizures of all 13 patients with unilateral neurologic signs corresponded to the hemisphere implicated by the deficit. Computed tomography (CT) scan showed lesions in 30 patients (65%) and corresponded to the lobe of seizure onset in 27 of the 30 (90%). Seizures arose primarily from a frontal lobe in 22 of 46 patients (48%), from a temporal lobe in 14 patients (30%), and from an occipital-parietal-posterotemporal region in 6 patients (13%); only the hemisphere of origin could be determined in 4 patients. Cortical developmental abnormalities were the most common known cause (12 patients, 26%); febrile seizures and primary brain tumors occurred in 10 and 8 patients, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)     

Gelastic seizures due to right temporal cortical dysplasia.

Gelastic seizures are an uncommon seizure type. They are most frequently observed in patients with hypothalamic hamartoma. Their association with other types of cerebral lesions is rare. Depending on the location of the lesion, gelastic seizures may or may not be accompanied by a subjective feeling of mirth. The pathophysiological mechanisms of this type of seizure are still undefined, and little is known about which pathways promote laughter and its emotional content, mirth. We present a young man with drug-resistant, gelastic seizures due to focal cortical dysplasia of the right inferior temporal gyrus. The lesion was evident on cranial MRI. Interictal EEG displayed a right temporal focus, whereas ictal EEG was not informative. Ictal loss of consciousness precluded reporting of any possible emotional experience. The patient underwent surgical resection of the lesion and has been seizure-free with anti-epileptic medication for two years. Although various anatomical regions may elicit laughter, in view of the current literature it seems that the anterior cingulate region is involved in the motor aspects of laughter, while the basal temporal cortex is involved in the processing of mirth. The fact that the present case exhibited gelastic seizures stresses once more the importance of the baso-lateral temporal cortex in the genesis of this type of seizures.[Published with video sequences].