Landau-Kleffner综合征的临床特征(附3例报告)

目的探讨Landau-Kleffner综合征(LKS)的临床特征。方法回顾性分析3例LKS患者的临床资料。结果3例临床表现均有获得性失语、癫发作及精神行为异常;脑电图呈局灶性棘波放电,头颅CT及MRI未见异常。经治疗癫发作控制较好,但失语及精神行为异常恢复较慢。结论LKS是以获得性失语和癫发作为临床特征;脑电图可见异常放电。治疗后可部分恢复。     

Landau-Kleffner综合征六例

目的研究Landau-Kleffner综合征(LKS)的临床、脑电图(EEG)特征、治疗反应及预后。方法对6例LKS患儿的临床及EEG资料进行分析,并对其治疗效果进行随访。结果该组患儿起病年龄3~8岁,男女比例2:1。起病前智力及语言发育正常。无癫痫家族史。均有获得性失语,表现为听觉性失认。均出现癫痫发作,表现为部分性发作、全面强直阵挛发作和不典型失神发作。听力检查正常。EEG均异常,清醒期EEG显示单侧或双侧颞区为著高波幅棘慢波;睡眠期EEG显示癫痫样放电均较清醒期明显增多,并泛化至全导,其中2例表现为慢波睡眠期持续性棘慢复合波。头颅磁共振(MRI)检查均正常。6例患儿经抗癫痫药物治疗其癫痫发作均完全控制。经皮质激素治疗,3例患儿失语完全恢复,2例部分恢复,1例未恢复。结论 Landau-Kleffner综合征是以获得性失语和癫痫发作为主要临床表现的儿童期癫痫综合征。EEG表现为以颞区为著的癫痫样放电,睡眠期全导泛化。癫痫发作经抗癫痫药物治疗可以控制且转归良好。早期合理应用皮质激素可以改善失语状况,但仍有患儿遗留语言障碍。     

Landau-kleffner综合征或伴有抽搐的获得性失语

Landau-Kleffner综合征(LKS)或伴有抽搐的获得性失语(acquired aphasia with convulsive disorder)是一种罕见的语言障碍,多数伴有不同形式的癫痫发作和普遍双侧同步的棘慢活动(SW)脑电图异常,也可见于无任何临床抽搐的患儿.1957年由Landau和Kleffner最先报告,并认为其临床表现甚为重要.尽管推测LKS十分少见,但自1957年至1990年间已有160例以上的病例报告,认为儿童期LKS比其他任何     

获得性癫性失语症的临床特点和免疫球蛋白治疗观察

获得性癫性失语症又称为Landau Kleffner综合征 (LKS) ,195 7年由Landau和Kleffner首先报道 ,主要表现为获得性失语及脑电图异常 ,约 80 %的病例伴有癫发作 ,70 %有明显的心理行为障碍〔1〕。近年来共诊断LKS 4例 ,应用静脉注射免疫球蛋白 (IVIG)治疗取得较好疗效 ,并进行了为期 0 5～ 1a的随访 ,报道如下 :1　临床资料例 1　男性 ,5岁。 3岁 6个月时出现失语 ,不理解别人的语言 ,并随后出现癫发作 ,为强直 阵挛发作 ,性格行为改变 ,易发怒 ,哭闹。脑电图示左侧颞部爆发性的 2 5Hz棘慢波活动 ,睡眠脑电图描记呈持续棘慢波状…     

剥夺睡眠脑电图在癫痫治疗停药过程中的临床应用体会

癫痫患者经过正规的抗癫痫药物治疗,临床发作消失3年左右,停药就会成为医患双方共同关注的问题.在多数癫痫病例中,规范化的抗癫痫药物对控制癫痫临床发作有效的同时,EEG亦有相应的改善,而证实发作性疾患存在的棘波或尖波放电常常(约80%的机会)只有在睡眠描记时才能发现,现将37例临床发作消失三年以上的癫痫患者的常规清醒脑电图和剥夺睡眠脑电图进行对照分析,可以看出常规清醒EEG作为停药的指标有其片面性,而剥夺睡眠脑电图作为参考指标,可为临床停药减量时机的选择提供更有价值的指导.     

抗癫痫药物对癫痫患者脑电活动的影响

癫痫是一组由大脑神经元异常放电所引起的以短暂中枢神经系统功能异常为特征的慢性脑部疾病,具有突然发生、反复发作的特点,大脑皮层神经元过度放电是各种癫痫发作的病理基础.癫痫所见的脑电图（electroencephalogram,EEG）称为癫痫脑电图,其主要内容为癫痫波,即头皮电极记录到的反映大脑神经细胞功能状态的电变化.有些抗癫痫药物在抑制痫样放电治疗癫痫的同时,对EEG背景活动也有一定的影响,并因此而引起一系列的临床副作用.本就抗癫痫药物抑制痫样放电的机理及其对EEG背景活动的影响做一综述,以期为广大临床工作提供些许帮助。     

长程脑电图监测对癫痫无发作患者停药后复发风险的预估

目的对癫痫无发作患者停用抗癫痫药物之前脑电图异常放电的独立风险因素进行分析,探讨通过长程脑电图监测预估癫痫无发作患者停药后复发风险的临床价值。方法对2009-01—2011-12在盐城市第四人民医院神经内科门诊经过抗癫痫药物治疗,癫痫无发作3a以上的62例原发性癫痫患者,减药、停药前行长程脑电图监测,停药后进行2~3a的随访。结果停药前有癫痫样放电患者21例,无癫痫样放电41例;有癫痫样放电患者减、停药1a内复发6例,复发率28.6%,停药后2~3a复发3例,复发率14.3%,总体复发率42.9%;无癫痫样放电患者停药后1a内复发4例,复发率9.7%,停药后2~3a复发2例,复发率4.9%,总体复发率14.6%。两者复发率差异有统计学意义(P0.05)。结论抗癫痫药物治疗后3a以上癫痫无发作的患者不论减、停药前长程脑电图监测有无异常都有一定的癫痫复发率,而脑电图有异常放电的患者减、停药后复发率高,停药后复发风险大,长程脑电图监测对癫痫无发作患者停药后复发风险的预估有重要的临床价值。     

儿童睡眠中癫痫电持续状态患儿的临床及脑电图特征

目的探讨儿童睡眠中癫痫性电持续状态(ESES)的临床和脑电图变化特征。方法通过长程视频脑电图(VEEG)监测,对35例合并ESES的癫痫患儿进行临床资料及脑电图资料的回顾性分析。结果35例患儿的VEEG,NREM期均见ESES现象,放电以Rolandic区为主。发病后精神行为异常25例(71.4%),智力障碍19例(54.2%),语言障碍22例(62.8%)。经抗癫痫药治疗后,26例有效,临床发作完全控制或明显减少;7例无效者采用甲泼尼龙冲击治疗后,在控制临床发作和痫样放电方面有良好的效果;2例无效。结论 ESES是涉及多种癫痫综合征的特殊EEG,NREM睡眠期持续放电是引起神经心理损伤的主要原因。而长程视频脑电图是诊断ESES的有效工具,便于早期采取干预措施,是改善神经心理损伤的关键。     

获得性癫癎性失语症的临床特点和免疫球蛋白治疗观察

获得性癫癎性失语症又称为Landau-Kleffner综合征(LKS),1957年由Landau和Kleffner首先报道,主要表现为获得性失语及脑电图异常,约80%的病例伴有癫癎发作,70%有明显的心理行为障碍[1].近年来共诊断LKS 4例,应用静脉注射免疫球蛋白(IVIG)治疗取得较好疗效,并进行了为期0.5～1a的随访,报道如下:     

良性家族性婴儿癫痫的临床特点

目的探讨良性家族性婴儿癫痫(BFIE)的临床特点。方法回顾性分析1例PRRT2基因突变和双侧额区放电的BFIE患者的临床资料,并进行文献复习。结果 BFIE主要临床表现为局灶性癫痫发作,PRRT2为主要致病基因。发作间歇期EEG多无异常,局灶性放电部位多为额区。本病预后良好,多数抗癫痫药物对本病有效。结论婴儿期以局灶性癫痫起病,如丛集性发作,且智力、运动发育正常,需结合基因和EEG检查,考虑BFIE可能。     

Landau–Kleffner syndrome: A study of 29 patients

PurposeThe aim of the study was to retrospectively analyze the electroclinical features, etiology, treatment, and prognosis of 29 patients with Landau–Kleffner syndrome (LKS) with a long-term follow-up.MethodsInclusion criteria were a diagnosis of LKS with: (1) acquired aphasia or verbal auditory aphasia; (2) with or without focal seizures, secondarily generalized tonic-clonic seizures, absences, or atonic seizures.ResultsMean follow-up was 12 years. All cases except six had seizures. Before the onset of aphasia, developmental language and behavioral disturbances were present in 19 and 14 patients, respectively. All patients had verbal auditory agnosia. Aphasia was severe in 24 patients and moderate in five. Nonlinguistic cognitive dysfunctions were moderate in 14 patients. Behavioral disturbances were found in 16 patients. During the continuous spike-and-wave discharges during slow sleep phase, the spike-wave index was >85% in 15, 50–85% in eight, and 30–50% in four. In two patients, the EEG recording showed occasional bilateral spikes, without continuous spike-and-wave discharges during slow sleep. In this phase, the awake EEG recording showed more frequent interictal abnormalities, predominantly in the temporal regions. Eight patients recovered language completely, but the remaining patients continue to have language deficits of different degrees.ConclusionLandau–Kleffner syndrome is an epileptic encephalopathy characterized by acquired verbal auditory aphasia and seizures in most of the patients associated with continuous or almost continuous spike-and-wave discharges during slow wave sleep. The most commonly used treatments were clobazam, ethosuximide, sulthiame. High-dose steroids were also administered. Adequate and early management may avoid language and cognitive deterioration.     

Landau-Kleffner and autistic regression: the importance of differential diagnosis

Some neurological disorders may present psychiatric signs and symptoms, therefore the search for an etiological diagnosis is crucial. The aim of this study is to report the case of a patient with a neurological disorder, diagnosed during a psychiatric admission. A boy with normal neuropsychomotor development until the age of 3 years, started presenting epileptic seizures, followed by behavioral disorder and language deterioration. During neurologic follow-up, the patient was referred to the Psychiatry Department with a diagnosis of autism, in this case an autistic regression (AR). During his admission, diagnosis of Landau-Kleffner syndrome (LKS) was established on clinical and EEG grounds. LKS is characterized by acquired aphasia, epilepsy, EEG abnormalities and behavioral changes, including autistic traits. Language regression is observed LKS and AR. We stress the main differences between these two entities because misdiagnosis may postpone early intervention and consequent benefits, as observed in our case.     

A review of the relationships between Landau-Kleffner syndrome, electrical status epilepticus during sleep, and continuous spike-waves during sleep

The goal of this report is to review the relationships between Landau-Kleffner syndrome (LKS), electrical status epilepticus during sleep (ESES), and continuous spike-waves during sleep (CSWS). LKS is a clinical syndrome involving mainly acquired aphasia and sometimes seizures. Other clinical findings include cognitive impairments and global regression of behavior. The EEG may evolve from more benign conditions into ESES (or CSWS), seen in 50% of patients with LKS, or may also show focal findings. Seizures include atypical absence, generalized tonic-clonic, atonic, and partial motor attacks. Effective medications are discussed. The EEG patterns CSWS and ESES are likely equivalent terms. CSWS is used by some authors, and ESES by others. Patients with these patterns usually show mental retardation, seizures, and global regression. More benign EEG patterns, like focal discharges, may develop into these more severe generalized patterns, which are associated with atypical absences, negative myoclonus, and cognitive disturbances. Memory disorders are common, because the nearly continuous generalized discharges in sleep do not allow for the memory consolidation that also occurs during sleep. Medications and possible etiologies are discussed.     

SPECT abnormalities in Landau-Kleffner syndrome

Five right-handed children with acquired aphasia elipepsy syndrome (Landau-Kleffner, LKS), were investigated with 99^m TcHMPAO single photon emission computed tomography (SPECT) and the results were correlated with their EEGs and clinical history. The childrens' ages ranged from 2 to 5 years and the aphasia had been present for 6 to over 12 months. No clinical seizure had ever been onserved in the younger two children and their waking EEGs showed infrequent central spikes. Both children had areas of low intensity on SPECT, involving the left temporal lobe in one and the right temporal lobe in the other, which has also been reported in children with congenital dysphasia who have normal EEGs. The three older children presented with frequent generalized seizures, with the aphasia occurring 3–6 months later. The SPECT scans in these children were performed either in the ictal state, or when electrographic seizure activity was very frequent on EEG. All three children had hyperintense foci on SPECT involving the left posterior temporal region corresponding to Wernickes area. We conclude that LKS may be initially a unilateral seizure disorder of Wernickes area, with EEG discharges in the contralateral hemisphere representing propagation from the unilateral focus.     

Long-term outcome of epilepsy in Kabuki syndrome

Purposes and methods

Kabuki syndrome (KS) is a rare dysmorphic disorder characterized by multiple congenital anomalies and mental retardation. Although epilepsy is one of the most common clinical complications associated with KS, few studies have evaluated its electroclinical aspects and long-term outcome. Therefore, we describe here a clinical series of 10 Caucasian KS patients who developed epilepsy in childhood. We followed all children for at least 5 years.

Results

All patients presented partial seizures and interictal EEGs revealed focal epileptic paroxysms with prevalent involvement of temporo-occipital areas. Seven children had no central nervous system abnormalities, but enlargement of lateral ventricles, corpus callosum hypoplasia, and adenohypophysis hypoplasia were revealed in three. Although antiepileptic drug (AED) treatment was effective in controlling seizures and normalizing EEG abnormalities in 8 patients, the other 2 cases were resistant to multiple AEDs. In one of these two patients, withdrawal of AED resulted in status epilepticus and death.

Conclusions

Partial seizures and temporo-occipital abnormalities on interictal EEG are common features of KS patients who suffer from epilepsy. Prognosis of this epilepsy is favourable in the majority of cases with complete disappearance of seizures and EEG abnormalities.     

Epileptiform Activity in Aphasia of Childhood:An Epiphenomenon?

Isolated aphasia with associated EEG epileptiform activity is a recognized syndrome in children. The relationship of the EEG abnormality and the type and severity of the speech impairment has not been well described. This relationship was studied in two children with severe expressive and receptive aphasia with generalized spike-wave discharges on EEG using prolonged EEG FM radiotelemetry and video recording (TEEG-VR). Speech was compared with 10 children with absence seizures with similar EEG abnormalities also evaluated using TEEG-VR. In addition, 43 cases of aphasia with epileptiform activity on the EEG reported in the English literature were reviewed. Speech abnormalities in absence seizures consisted of speech arrest, decreased speed of speech, and brief periods of partial or complete receptive and expressive aphasia, always directly associated with a spike-wave ictus. Speech abnormalities in acquired or congenital aphasia were not related to epileptiform activity and were characterized by severe articulation difficulties, syntactic transformation, paraphasia, and receptive and expressive aphasia. Anticonvulsants did not alter speech. Based on these two cases and the 43 others reviewed in the literature, it is proposed that epileptiform activity in this syndrome is an epiphenomenon reflecting underlying abnormalities of speech areas rather than the cause of the aphasia.     

获得性癫痫失语症(附7例报告)

目的:本文通过对7例获得性癫痫失语症(Landau-Kleffner综合征)患者的临床表现、脑电图特点和临床疗效的观察,结合文献进行探讨。结果:7例患者均有不同类型的癫痫发作,智力明显低下,伴有进行性失语。7例患者均接受免疫球蛋白和口服强的松治疗,激素治疗后平均2个月起效,8～12月4名患者发音明显改善,能较流利的对话,癫痫发作减少。3例治疗不理想,原因为激素应用时间短和病程太长。结论:Landau-Kleffner综合征只要能够早期诊断,尽早进行有效积极治疗,丧失的言语理解力可有不同程度的恢复,总体预后良好。     

Bilateral volume reduction of the superior temporal areas in Landau-Kleffner syndrome

No specific anatomic abnormalities have been detected in typical Landau-Kleffner syndrome (LKS), an acquired epileptic aphasia with language regression in children. In four children with typical LKS without obvious anatomic abnormalities, the authors performed MRI volumetric analysis of various neocortical regions and subcortical substructures. Volume reduction was detected in bilateral superior temporal areas (26 to 51%), specifically in planum temporale (25 to 63%) and superior temporal gyrus (25 to 57%), where receptive language is localized.     

Seizures and epilepsy among children with language regression and autistic spectrum disorders

Clinical and subclinical seizures occur frequently among children with autistic spectrum disorders. Electrographic status epilepticus in sleep, or continuous spike-wave in slow-wave sleep, is a typical feature of acquired epileptic aphasia and Landau-Kleffner syndrome. Seizures and epilepsy are more common among children with autistic spectrum disorder who experience language regression, especially those who experience language regression after the age of 2 years. Although the seizures associated with Landau-Kleffner syndrome and with acquired epileptic aphasia can be easily treated, improvement in language function often does not follow successful treatment of seizures. There are no published randomized clinical trials of treatments for Landau-Kleffner syndrome or for autistic language regression. Broad-spectrum antiepileptic drugs not associated with cognitive slowing are probably the treatment of choice for epilepsy among children with autistic spectrum disorder. Large multisite trials are needed to determine treatment efficacy among children with Landau-Kleffner syndrome and acquired epileptic aphasia and to establish whether there is a cause-effect relationship between electrographic status epilepticus in sleep or continuous spike-wave in slow-wave sleep and autistic language regression.     

Efficacy of very high dose steroid treatment in a case of Landau-Kleffner syndrome

Landau-Kleffner syndrome (LKS) is an acquired childhood aphasia associated with paroxysmal bitemporal electroencephalogram (EEG) abnormalities and, sometimes, clinical seizures. We report the case of a female aged 5 years 6 months who presented clinically with apparent hearing loss, deterioration in speech, and seizure activity over 12 days. The female had previous detailed speech/language assessments at 3 to 4 years of age due to articulation delay. LKS was diagnosed on EEG with bitemporal spike and wave activity during sleep. The patient was treated with high dose prednisolone 3mg/kg/day, intensive speech/language therapy, and followed a modified educational program. We recorded a marked regression in receptive and expressive language skills, as well as her speech, language, and cognitive profiles before and during treatment with prednisolone, during an 18-month follow-up period. The patient demonstrated an excellent clinical response highlighting the importance of a multidisciplinary approach to management of LKS.