Lennox-Gastaut综合征及其外科治疗

Lennox-Gastant综合征是儿童常见的重症癫痫之一。其临床表现复杂多样。虽然对该病的非手术治疗方法众多,但效果不佳。因此,许多学者正在探索采用外科手段来治疗本病。本文将对该病的临床特点和非手术治疗方法作一简述,并就目前所采用的各种外科治疗方法的原理和疗效进行综述。     

Lennox-Gastaut综合征治疗的初步经验

目的总结Lennox-Gastaut综合征的临床特征及治疗经验。方法根据术前对病人的临床分析和痫灶定位检查,术中采用皮质脑电监测,选择致痫灶切除术、前颞叶切除术、前颞叶及海马切除术、胼胝体切开术和多处软膜下横纤维切断术等几种适宜术式联合使用治疗9例Lennox-Gastaut综合征病人。结果术后随访2～3年,根据癫痫外科手术疗效分类标准,Ⅰ级2例(22.22%),Ⅱ级5例(55.56%),Ⅲ级1例(11.11%),Ⅳ级1例(11.11%),手术总有效率达77.78%(7/9)。结论选择适当的手术方式联合应用治疗Lennox-Gastaut综合征,可以取得最佳的治疗效果。     

切除性手术治疗学龄儿童病灶性Lennox-Gastaut 综合征

目的：研究MRI阳性的学龄期儿童Lennox-Gastaut 综合征（ LGS）患者切除性手术的疗效,并分析手术适应证。方法回顾性分析解放军总医院第一附属医院神经外科收治的学龄期LGS儿童患者14例,其中左侧大脑半球切除术1例,单脑叶切除术9例,多脑叶切除术4例。对患者的人口学特征和临床特征,手术方法,手术结果进行分析。结果本组患者MRI显示局限性异常6例,广泛性异常8例。病理灶全切除9例。术后1年和3年的癫痫无发作率为64．3％和57．1％。其中病理灶全切除和MRI显示局限病理者术后3年无发作率分别为75％和83．3％。术后较术前QOL改善者11例,术后IQ改善者9例。术后3年总体患者满意度评分为86．07％±12．66％。满意度与术后无癫痫发作有明显相关性（ P＝0．001）。术后使用AEDs较术前显著下降。结论切除性手术治疗学龄期儿童合并病理灶相关的LGS癫痫控制效果良好,QOL和IQ显著改善,安全性高。 MRI显示局限性病理灶的患者应当优先考虑切除性手术,完整切除病理灶疗效可能更好。     

妥泰治疗Lennox-Gastaut综合征48例疗效观察

目的对加用TPM治疗Lennox-Gastaut综合征患儿48例,观察临床疗效和不良反应。方法对符合起病年龄的婴幼儿至青少年,临床有不典型失神,强直-痉挛发作,失张力及大发作等多种发作,绝大多数有病因,且伴有精神运动发育落后,脑电图显示1.5～2.5Hz慢棘慢复合波确诊为Lennox-Gastaut综合征,均应用妥泰单一治疗并进行疗效分析,妥泰剂量由1mg/(kg.d)开始,每5～7d增加1mg/(kg.d),直至出现疗效或不能再耐受的不良反应。结果2006-01～2007-08来我院神经科住院房48例Lennox-Gastaut综合征,经妥泰治疗3～15个月,平均疗程6个月,总有效率62.5%,发作消失19例占39.6%,其中强直痉挛发作减少≥50%为60.2%,不典型失神发作减少≥50%为59.3%,发作消失为40.7%,失张力发作7例,无效3例,4例发作消失。15例单一妥泰治疗发作消失8例,显效3例,无效4例,总有效率73.3%。丙戊酸钠与卡马西平在妥泰添加前后其血药浓度无明显变化。而治疗有效30例妥泰有效剂量范围2～15mg/(kg.d),平均(6.4±2.8)mg(kg·d),其不良反应有体质量下降5例,嗜睡11例,纳差8例,言语不清1例,注意力不集中4例,类暑热症4例,失平衡1例。结论通过妥泰治疗Lennox-Gastaut综合征疗效分析,认为妥泰是一种广谱,有效而安全的新型抗癫药物,其不良反应轻,多为可耐受的躯体感觉症状,值得推广应用。     

拉莫三嗪治疗Lennox-Gastaut综合征的临床观察

应用拉莫三嗪添加、开放性自身对照法观察Lennox-Gastaut综合征患者8例,观察时间为3～31个月。结果:应用LTG后,2例患者(25%)癫痫发作停止,4例患者(50%)癫痫发作减少大于75%,总有效率75%。LTG对Lennox-Gastaut综合征的各型癫痫发作均有较好效果,脑电图有改善的倾向。部分患者应用LTG治疗后生活质量有一定的改善。副作用少。治疗前后各项实验室检查未见有临床意义的异常改变。     

左乙拉西坦添加治疗Lennox-Gastaut综合征临床疗效观察和脑电图改变

目的研究左乙拉西坦(LEV)添加治疗儿童癫痫性脑病Lennox-Gastaut综合征(LGS)的临床疗效和脑电图(EEG)改变。方法选取2014年1月至2018年5月在河北省儿童医院神经内科就诊47例LGS患儿,在原有药物治疗的基础上添加LEV口服,对比治疗前和治疗后4w、8w的癫痫发作控制有效率、EEG改善情况和安全性评价。结果 47例患者,在不同的癫痫发作类型中,强直发作、不典型失神发作、阵挛发作、强直-阵挛发作、强直-失张力发作、失张力发作、肌阵挛发作、癫痫样痉挛发作总有效率分别是63.3%、64.3%、50%、60%、50%、60%、61.5%、65%,(P> 0.05)。47例癫痫发作的患者中34例患者视频脑电图(VEEG)有效率72.3%;11例患者(23.4%)无缓解;2例患者(4.3%)EE G恶化。醒睡各期治疗后较治疗前癫痫放电指数均显著减少(P <0.001)。17例(36.2%)患者报道了不良事件,多动症5例(10.6%),疲劳3例(6.4%),嗜睡3例(6.4%),指压性水肿1例(2.1%),食欲不振、呕吐、睡眠欠佳、皮疹等其他不良事件5例(10.6%),所有不良事件均未危及生命。结论 LEV添加治疗儿童癫痫性脑病LGS是一种安全有效的治疗方法。     

学龄儿童病灶性Lennox-Gastaut综合征的临床特征及脑电图

目的分析学龄儿童病灶性Lennox-Gastaut综合征(LGS)的临床特征及脑电图特征,为LGS进一步的临床研究和诊断提供参考。方法选择2011-08—2015-05在我院诊治的LGS学龄儿童32例为LGS组,选择同期在我院进行检查的32例非癫痫正常儿童32例为对照组,记录与调查所有儿童的临床资料、临床表现特征、脑电图特征、智力发育与随访情况等。结果观察组患儿的起病年龄(5.21±2.91)岁;其中8例出生时有异常,8例有惊厥史,9例有癫痫史,6例有智能发育迟滞家族史;强直发作24例,不典型失神11例,肌阵挛发作10例,痉挛发作7例,全面强直-阵挛性癫痫发作21例,失张力发作9例。对照组脑电图均正常,LGS组脑电图背景节律Ⅰ级12例,Ⅱ级16例,Ⅲ级4例;30例清醒或睡眠期可见到12次/s左右的快活动,20例存在一侧性放电波。LGS组智力评分为(61.14±11.84)分,智力障碍发生率为34.4%;对照组智力评分为(34.22±9.93)分,智力障碍发生率为3.1%,组间对比差异有统计学意义(P0.05)。治疗后3个月LGS组的癫痫无发作率为81.3%,6个月无发作率68.8%,1a无发作率为50.0%。结论学龄儿童病灶性LGS的癫痫发作类型多样,脑电图多显示背景ɑ节律异常和棘慢复合波,智能障碍的发生率较高,药物联合治疗的效果较好。     

Lennox-Gastaut综合征

本文综述了近年来Ｌｅｎｎｏｘ－Ｇａｓｔａｕｔ综合征的病因、发作类型、神经精神异常、脑电图表现以及治疗和预后等方面的研究进展。     

Lennox-Gastaut综合征外科治疗进展

<正>Lennox-Gastaut综合征(LGS)是最难治疗的儿童常见癫痫综合征之一,约占儿童癫痫的5%~10%,尽管应用多种新型抗癫痫药物的联合和个体化治疗,多数患儿的癫痫发作症状仍然控制不良,并常伴有认知功能的衰退和行为异常,严重影响了患儿的生存质量。该疾病发病高峰为3~5岁之间,约80%的患儿癫痫发作迁延至成年。Lennox和Gastaut两位学者先后总结和补充了该综合征患者癫痫发作的临床     

Tourette综合征的外科治疗

本文介绍外科治疗Ｔｏｕｒｅｔｔｅ综合征的方法，ＴＳ的病理学，ＴＳ和强迫症之间的联系及其外科治疗的神经解剖机理，并对进一步研究提出一些建议。     

Alertness and incidence of seizures in patients with Lennox-Gastaut syndrome

Sixteen subjects affected by Lennox-Gastaut syndrome (11 males and five females, ranging from 5 to 15 years of age [mean age, 9 years 11 months]) were followed for periods of 9 months to 5 years 9 months, and were studied during three to five prolonged hospitalizations for total periods of 2-9 months with a veiw to examining the distribution of epileptic seizures during four states of vigilance, evaluated from a behavioral point of view: sleep, drowsiness, inactive wakefulness, and active wakefulness. it was ascertained that the overall average of 406 seizures daily, directly observed, was distributed as follows: 26 (6.40%) during sleep; 128 (31.52%) during drowsiness; 219 (53.94%) during inactive wakefulness; and 33 (8.12%) during active wakefulness. The comparison between the incidence of seizures observed during active wakefulness and those observed during both drowsiness and inactive wakefulness was significant (p less than 0.001). The latter two states represent, in our study, the shortest period of the day (8 h as compared with the 16 h of sleep and active wakefulness), thus making the results of the comparison even more significant. The results of this study suggest the importance of a stimulating environment for children affected by Lennox-Gastaut syndrome, and they point out that an overdose of antiepileptic drugs, not uncommon in the treatment of this syndrome, may make seizures more frequent.     

Cutis verticis gyrata, mental retardation and Lennox-Gastaut syndrome: a case report

Cutis verticis gyrata (CVG) is an abnormality of the scalp characterized by the formation of furrows and folds which cannot be flattened by traction or pressure. Primary and secondary forms of CVG have been described. We report on a patient affected by cutis verticis gyrata, mental regression and Lennox-Gastaut syndrome (LGS). Serum hormonal levels, karyotype and X fragile studies were normal. Magnetic resonance imaging of the brain showed only atrophic changes. The etiology of primary CVG remains unknown as does its relation with LGS. Received: 29 March 2001 / Accepted in revised form: 2 June 2001     

Uncovered primary seizure foci in Lennox-Gastaut syndrome after corpus callosotomy

Purpose

Corpus callosotomy (CC) is a palliative surgical procedure to control atonic, tonic, or generalized tonic-clonic seizure in Lennox-Gastaut syndrome (LGS). Here, we report patients with LGS who underwent resective surgery, following CC better delineating the presumed seizure foci localized in one hemisphere.

Methods

We retrospectively reviewed seven patients with LGS who underwent CC and subsequent cortical resection. The median follow-up duration after lobectomy was 20 months (range, 15-54 months) and three patients had follow-up periods over 24 months. The findings of video electroencephalography (EEG) monitoring, structural and functional neuroimagings were compared between pre- and post-CC.

Results

Four patients had Engel class I and one patient had Engel class II outcomes following cortical resection; post-CC, compared to pre-CC, showed better localized ictal/interictal epileptiform discharges in the unilateral frontal area in two patients, in the unilateral parieto-temporo-occipital areas in one patient and in the unilateral fronto-temporal areas in the remaining two patients. Two patients had Engel Class III outcome following cortical resection; post-CC EEG continued to show multifocal epileptiform discharges but predominantly arising from a unilateral frontal area. Following CC, positron emission tomography showed localized glucose hypometabolism of which location was concordant with post-CC EEG abnormalities in all patient. Similarly, ictal/interictal single photon emission computed tomography also showed localized abnormalities concordant with post-CC EEG abnormalities in five of the six patients. Pathological assessment revealed cortical dysplasia in six patients, whereas no pathological abnormality was found in the remaining patient, who obtained Engel Class I outcome following cortical resection.

Conclusion

CC could change EEG findings, glucose metabolisms and cerebral blood flows, and it is sometimes helpful in delineating the primary seizure focus in patients with LGS.     

Occurrence, outcome, and prognostic factors of infantile spasms and Lennox-Gastaut syndrome

PURPOSE: To analyze the occurrence, outcome, and prognostic factors of infantile spasms (IS) and the Lennox-Gastaut syndrome (LGS) in a defined population. METHODS: All children treated because of IS and LGS in the Department of Pediatrics, University of Oulu, from January 1, 1976, until December 31, 1993, who came from the primary catchment area of the hospital were included. Detailed information concerning their individual pre-, peri-, and postnatal medical histories and medical and laboratory examinations were compiled. RESULTS: Thirty-seven children (18 boys) had IS, and 25 (14 boys) had LGS. The occurrence of IS of 0.41/1,000 live births 195% confidence interval (CI), 0.29-0.57/1,000] did not differ significantly from that of LGS, which was 0.28/1,000 live births (95% CI, 0.18-0.41/1,000). Ten (27%) of the 37 patients with IS evolved to LGS, which was 40% of the LGS cases. All the 10 children with both IS and LGS had symptomatic epilepsy, were mentally retarded, and had active epilepsy at the end of approximately 10 years' follow-up. Twenty-six (87%) of the 30 symptomatic IS cases and all the 17 symptomatic LGS cases were due to either congenital or genetic etiologies. The outcome in cryptogenic IS cases was favorable; the risk for a poor neurologic and mental outcome was extremely low; odds ratio, 0.015 (95% Cl, 0.001-0.196), as it was for therapy-resistant epilepsy; odds ratio, 0.013 (95% CI, 0.001-0.166). In LGS patients, cryptogenic etiology did not decrease the risk for a poor outcome. CONCLUSIONS: Cryptogenic etiology is associated with a very low risk for a poor outcome in IS patients, but not in LGS patients. The outcome of IS children and the relation of IS to LGS are determined by the underlying brain disease, not by the epilepsy itself.     

Community-Based Study of Lennox-Gastaut Syndrome

Summary: Purpose : Before 1986, the spectrum of childhood epilepsies, including Lennox-Gastaut syndrome (LGS) and Doose syndrome (DS), known collectively as "epilepsia myoclonica astatica", was believed to represent a single disease. More recently, some investigators have considered these syndromes to be parts of a continuum. To clarify these theories, neurobiologic factors of the syndromes were studied to determine which qualities were shared and which were unique.
Methods : A retrospective (1975–985), community-based (Helsinki metropolitan area and the province of Uusimaa) study was designed to seek children with features of LGS and DS. It was assumed that recall bias and the selection of documented history would be similar throughout the group. Ranks of increasing pathology were assigned to different seizure types, EEG results, and drug treatments. A similar procedure was applied to epidemiologic data. Spearman rank-order correlations were calculated to determine which features correlated with LGS and which correlated with less severe epilepsy.
Results : The survey comprised 75 patients with broadly defined LGS. The annual incidence was 2 in 100,000 children aged 0 to 14 years. Prenatal or perinatal abnormalities did not correlate with severity of epilepsy. As compared with the relatively favorable ranks, the severe epilepsy ranks were more often associated with an early onset of epilepsy, an infectious disease at the onset, delayed development before epilepsy, abnormalities in neurologic or neuroradiologic examinations, and a deteriorating course of the condition.
Conclusions : Patients with LGS are more likely than patients with less severe epilepsy to have a younger age at onset of epilepsy, an infection or both, and a deteriorating course of the condition.     

Subacute necrotizing encephalomyelopathy (Leigh disease): report of a case with Lennox-Gastaut syndrome

More than 100 cases of Leigh disease have been reported. None have shown Lennox-Gastaut syndrome. We report here the first known case of Leigh disease with Lennox-Gastaut syndrome, and discuss the clinical course in detail. A 3 1/2-year-old-boy was admitted with multiple symptoms and despite various therapies he died at age of ten years. Serial CT showed marked cerebral atrophy, ventricular dilatation, and an arachnoid cyst in the posterior fossa. Histopathological findings on autopsy of the brain and spinal cord were consistent with those characteristic of Leigh disease. In the chronic course of Leigh disease, the cerebral cortex was also involved and the Lennox-Gastaut syndrome might be complicated.     

Lennox-Gastaut syndrome with late-onset and prominent reflex seizures in trisomy 21 patients

Purpose:   Lennox-Gastaut syndrome (LGS) is a severe epileptic condition characterized by multiple seizure types including tonic seizures, slow spike-and-wave discharges on electroencephalography (EEG), and cognitive impairment. LGS can occur in apparently healthy subjects or in patients with preexisting brain damage. The onset peaks between 3 and 5 years of age and the prognosis is usually poor. Herein we report 13 subjects with trisomy 21 who developed LGS.
Methods:   We retrospectively reviewed the clinical and EEG data of consecutive patients with LGS and trisomy 21 referred to five epilepsy centers over the last 30 years.
Results:   Data for 13 patients (8 male, 5 female) were collected. The mean age at onset was 9.1 years (range 5–16). The mean age at last follow-up was 23.5 years (range 11–43 years). Seizure onset was after age 8 years in eight (62%) patients and between age 5 and 8 in the other five. In none of the cases did a West syndrome precede the onset of LGS. Nine of 13 patients (69%) had unambiguous reflex seizures, mostly precipitated by sudden unexpected sensory stimulations, usually preceding or accompanying the onset of a full-blown LGS picture. Interictal and ictal EEG findings were typical for LGS. All patients were drug-resistant.
Discussion:   Patients with trisomy 21 may present a peculiar LGS, characterized by late onset and high occurrence of reflex seizures. Mechanisms underlying this particular presentation of LGS may include dendritic rarefaction and decreased interneurons, as well as functional abnormalities leading to overall decreased brain inhibition in these patients.