获得性癫痫性失语综合征的临床特点分析

目的 研究中国人获得性癫痫性失语(Landau-Kleffner Syndrome,LKS)的临床、脑电图及治疗特点.方法 对中国大陆地区报道的文献中71例LKS患儿的临床资料进行回顾总结,并结合文献进行探讨.结果 获得性癫痫失语均发作于儿童期,临床均有失语及脑电图癫痫样放电,其中以混合性失语及腩电图棘-慢复合波为多见.多数临床有癫痫发作,听力均无异常,多数伴有精神行为异常.癫痫发作用抗癫痫药物控制良好,经皮质激素及免疫球蛋白的治疗,失语大多能改善.结论 中国儿童获得性癫痫性失语主要临床表现为获得性失语和癫痫发作,多伴有其它精神行为症状.EEG常见颞区的限局性放电,失语表现为听觉失认.早期诊断并采取包括抗癫痫药物和皮质激素应用的综合治疗,患者的预后总体良好.     

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…     

小儿颞叶癫的临床表现及脑电图分析

目的探讨小儿颞叶癫的临床特征及脑电图特点。方法收集16例颞叶癫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的脑电图变化规律。结果患儿的癫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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是一组部分性症状性癫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。     

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:     

VEEG监测对鉴别癫及非癫发作的意义

目的探讨发作期视频脑电图（VEEG）在诊断癫性发作及发作类型的临床价值。方法对435例发作性疾病患者的临床资料和发作期VEEG进行回顾性分析。结果 VEEG共监测到临床发作733次,发作期脑电图记录到样放电557次。诊断为癫381例,其中癫合并非癫性发作（NES）24例,占6.3%;单纯NES 54例。癫发作类型以部分性发作为主,癫发作及发作间期样放电多见于NREMⅠ、Ⅱ期。结论 发作期VEEG监测对鉴别癫及非癫发作有重要的鉴别意义。     

视频脑电图在小儿癫痫诊断中的应用

目的评价视频脑电图(video-EEG)在小儿癫诊断中的应用价值。方法对126例具有发作性症状的患儿进行连续8h的包括清醒、睡眠、诱发试验及必要的认知测验的视频脑电图监测。结果经发作期视频脑电图证实,39例初诊为癫性发作的患儿中14例(35%)为非癫性发作;15例其他症状发作中13例(86%)为非癫性发作。64例样放电患儿中51例(80%)确定发作类型,22例(34%)确定癫类型。视频脑电图可发现短暂轻微的癫发作及样放电引起的一过性认知损伤。结论视频脑电图在排除非癫性发作、确定癫性发作的类型、评价脑电-临床关系方面可提供准确可靠的证据,进一步提高癫的临床诊断水平。     

长程视频脑电监测对癫的诊断价值

目的评价长程视频脑电图对癫等发作性疾病的诊断价值,提高癫及癫综合征的诊断。方法对在本院神经内科癫中心门诊及住院患者中首次以发作性疾病就诊的患者进行不同时程的视频脑电图监测,对其临床资料进行回顾性分析。结果 24 h组视频脑电监测对癫异常脑波放电的阳性检出率较高(76.9%),8 h组最低(30.1%),15 h组介于两者之间(55.4%),在24 h组1920例患者中有1476例有异常放电,287例记录到临床同步发作,其中153例明确癫发作类型,153例中96例进一步明确为癫综合征,使对癫的分型及癫综合征的分类更加明了细化。结论 24 h视频脑电监测能够显著提高癫患者的诊断率及异常脑波的检出率,有效地降低了假阴性率并且在癫的鉴别诊断、分型及癫综合症的诊断方面有重要的临床意义,同时对其他发作性疾病的鉴别提供了可靠的的临床依据。     

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。     

获得性癫[间] 性失语症

获得性癫性失语症(LKS)在1957年由Landau和Kleffner首先报道,目前已被国际抗癫联盟(ILAE)和世界卫生组织的国际疾病分类(ICD,WHO)所接受.在癫和癫综合征的国际分类中,本综合征列在"兼有全身性和部分性发作的癫 "之下.本病为一种比较罕见的癫综合征,临床以获得性失语、癫发作、脑电图(EEG)异常为特征.我国自1992年[1]开始见少量报道.现结合国内外文献介绍如下. 1 临床特点 目前认为LKS为一种发生于儿童期的功能性疾病,具有如下特点:①发病年龄3～8岁;②获得性失语,即在已获得与年龄相当的语言能力的前提下,丧失已经获得的语言功能;③爆发性脑电活动(PA),通常在双颞侧,左侧多见,睡眠中明显增多甚至呈睡眠脑电连续状态(ESES);④癫发作容易控制并具有自限性;⑤多伴有性格和行为异常;⑥没有可以解释症状学的脑部疾病;⑦随着脑部异常电活动的消退,可以获得一定程度的改善.     

脑血管病继发癫86例临床分析

目的探讨脑血管病继发癫发作的临床特征、脑电图特点,可能的病理机制、药物治疗及其预后转归。方法对我院神经内科近3a脑血管病继发癫发作86例患者临床资料进行回顾性分析。结果 86例患者中脑出血41例(47.7%),脑梗死27例(31.4%),蛛网膜下腔出血18例(20.9%),全面强直—阵挛发作46例(53.49%),部分运动性发作继发全面强直—阵挛发作20例(23.26%),部分运动性发作17例(19.77%),癫持续状态3例(3.49%)。脑电图异常74例。结论出血性脑血管病癫发生率较高,以全面性强直阵挛发作为主,动态脑电图对治疗和判断预后有很大帮助。经抗癫药物治疗,随访1a,75例无再发作,2例死亡。有9例需长期服抗癫药。患者一般预后良好。     

Landau-Kleffner syndrome beginning with stuttering: case report

Landau-Kleffner syndrome is marked by an acquired aphasia in children who have had normal language and motor development. A 3.5-year-old girl was referred to our clinic with stuttering. She was diagnosed as having benign myoclonic epilepsy of infancy at 3.5 months of age and treated with valproic acid. Her electroencephalogram (EEG) returned to normal at the end of the first year. The therapy was stopped after a 2-year seizure-free period. She started to stutter prominently 3 months after the discontinuation of antiepilepsy drugs. She had no verbal agnosia. Her EEG revealed multiple spike and wave discharges. She was diagnosed as having Landau-Kleffner syndrome. Her previous epilepsy history had contributed to her having obtained an EEG in the early period. We suggest that if a child with normal language function starts to stutter, Landau-Kleffner syndrome must be considered in the differential diagnosis.     

Landau-Kleffner syndrome. EEG topographic studies

Spectral and historical topographic mapping of EEG was done on 2 siblings with Landau-Kleffner syndrome. The clinical features of the elder sister were acquired aphasia developed at the age of 5, followed by convulsions a year later, and those of the younger brother were progressive ataxia, hemiparesis, urinary incontinence and convulsions at the age of 4 years and 10 months, followed by acquired aphasia a year later. The most prominent spectral mapping features were high spectral powers of delta, theta and alpha waves over the fronto-centro-parietal area. The power of alpha and beta wave bands, reflecting sharp or spike waves, varied spatio-temporally over the central, parietal, temporal and frontal areas. The historical mapping revealed variabilities of paroxysmal discharges in modes of propagation. These results suggested that electrophysiological dysfunction of the fronto-centro-parietal areas associated with markedly unstable paroxysmal discharges is the main feature of Landau-Kleffner syndrome.     

Congenital hydrocephalus and continuous spike wave in slow-wave sleep--a common association?

Early-onset hydrocephalus was identified as the common denominator in 5 of 12 consecutive patients with Landau-Kleffner syndrome or continuous spike wave in slow-wave sleep who presented during the last 6 years. This association seems to be more common than expected, and the course of epileptic disease and outcome differs from that of previous reports. We present the clinical cases and discuss the possible connection between early hydrocephalus and epilepsy, especially continuous spike wave in slow-wave sleep. A retrospective analysis was undertaken of the clinical course and electroencephalographic (EEG) data before and after the development of continuous spike wave in slow-wave sleep in a group of five children with early-onset hydrocephalus identified in three epilepsy clinics in Israel. Landau-Kleffner syndrome or continuous spike wave in slow-wave sleep was identified in 12 patients during 6 years (1995-2000). Five patients had a history of early-onset hydrocephalus. Three patients had ventriculoperitoneal shunts; two of them were never shunted. Patients' seizures and continuous spike wave in slow-wave sleep epileptiform activity was resistant to appropriate anticonvulsant treatment. Pediatric neurologists and neurosurgeons should be aware of the association between continuous spike wave in slow-wave sleep and early-onset hydrocephalus. When a child with hydrocephalus presents with behavioral, cognitive, or motor deterioration, he or she should undergo sleep EEG in addition to investigation of shunt function. Our series demonstrated the presence of continuous spike wave in slow-wave sleep and localization-related epilepsy that did not originate from the shunt site. This group of patients is heterogeneous, and the exact trigger for continuous spike wave in slow-wave sleep development is still unclear.     

Landau-Kleffner syndrome with mitochondrial respiratory chain-complex I deficiency

Landau-Kleffner syndrome is characterized by epileptic aphasia associated with electrical status epilepticus of slow wave sleep. A 5-year-old female, who had manifested normal developmental progress, was referred with principal complaints of fluctuating sensory aphasia and bizarre behavior during the preceding 4 months. Landau-Kleffner syndrome was confirmed by clinical and electroencephalographic features; in addition, the patient's mitochondrial respiratory chain-complex I deficiency was confirmed by fibroblast culture with the evidence of energy metabolism disorder. This patient's seizures were intractable to many antiepileptic drugs, adrenocorticotrophic hormone, and intravenous immunoglobulin, with catastrophic cognitive and behavioral decline, but the seizures were successfully controlled by ketogenic diet with supplementary mitochondrial cocktail including coenzyme Q10, riboflavin, L-carnitine, and high-dose multivitamins. The patient finally regained fully normal cognitive functioning. Landau-Kleffner syndrome with mitochondrial respiratory chain-complex I deficiency was controlled in this case by ketogenic diet and supplementary mitochondrial cocktail therapy.     

The Landau-Kleffner syndrome--case report and theoretical considerations

The Landau-Kleffner syndrome is an acquired condition in children consisting of aphasia and epileptic discharges in the EEG with or without clinical manifestation of epilepsy. We describe a case of a very young child whose neurologic and language development before onset of the illness was uniquely well documented. Apart from her aphasia she showed several other disturbances of higher cortical functions in the acute phase of the illness. No anatomical basis has been discovered for this disease till the present day. We discuss the possibility of an electrical dysregulation in several parts of the cortex as a pathophysiological basis for this syndrome.     

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.     

Landau-Kleffner syndrome: a case study with a fourteen-year follow-up

In a 14-year follow-up the improvement of language functions and the decrease of EEG abnormalities are described in a girl with the Landau-Kleffner syndrome. In this case, the results of subsequent EEG recordings do not permit conclusions about the course of the aphasia. Disappearance of focal spike and waves, bilateral paroxysms and slow background activity lack synchronicity with improvement of language. Finally, subtle language deficits were still present 14 years after the onset of the aphasia; the lastly recorded EEG showed mild abnormalities.     

Acquired Aphasia, Dementia, and Behavior Disorder with Epilepsy and Continuous Spike and Waves During Sleep in a Child

Severe persistent neuropsychological disorders sometimes develop in the course of a focal epilepsy of unknown origin in previously normal children. Very frequent bilateral focal or generalized discharges are often noted on the sleep EEG records of these patients with no evidence of clinical seizures. The relation between these paroxysms and the observed deterioration remains unclear. We report a child with a partial complex epilepsy and severe disturbances of language, cognition, and behavior acquired in the early years of development who was followed for 15 years. A correlation between the evolution of the striking EEG abnormalities during sleep and the neuropsychological disorders could be established retrospectively. The observed sequence of onset and recovery of the aphasia, the dementia, and the "psychotic" behavior makes a direct causal relation between the deficits quite unlikely. Rather it suggests an association of independent symptoms with a specific language disorder becoming manifest in the course of the evolution. This child shows many of the main characteristics of the syndromes of "acquired aphasia with convulsive disorder" (Landau-Kleffner syndrome) and "epilepsy with continuous spike waves during sleep." Both syndromes describe probably different facets of a similar underlying, still unexplained cerebral dysfunction.     

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.