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

Landau Kleffner综合征并不罕见 ,但由于医务人员对其认识不够 ,易导致漏诊和误诊 ,现对该综合征的病因、发病机制、临床表现、诊断、治疗和预后等近期文献作一综合论述     

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。     

Landau-Kleffner综合征二例报告

Ｌａｎｄａｕ－Ｋｌｅｆｆｎｅｒ综合征二例报告彭宏伟Ｌａｎｄａｕ－Ｋｌｅｆｆｎｅｒ综合征特征为反复癫痫发作，发作后长期获得性失语和ＥＥＧ异常，现报道２例如下。例１男，１０岁。右利手。第２胎，足月顺产。１岁学语，１．５岁可简单对话。９岁不明原因出现双上肢...     

Landau—kleffner综合征

Landau—kleffner综合征(L—KS)又称伴有惊厥性疾病的获得性失语(Acquired aphasia with convulsive disorder)。1957年由Landau和kleffner首次报道。临床上较为罕见,到1990年文献报道仅有160例,国内有2例报道。1985年癫痫综合征国际分类将其定为一独立的类型,1989年的修正分类亦然。随着累积病例的增加,有关L—KS是一独立的临床综合征,还是儿童良性功能性部分性癫痫的一个特殊类型尚有争议,Landau本人也对其原命名提出异议。目前该病以临床表现和脑电图(EEG)改变做出诊断,尚无统一的诊断标准。现较为完善的定义是:获得性的高级皮层功能改变伴有睡眠期EEG异常活动,癫痫预后良好。     

原发性进行性失语综合征新认识

原发性进行性失语综合征是一种以进行性语言障碍为惟一或突出临床特征的痴呆综合征,言语障碍最显 著,进展也最迅速。无有效治疗。     

非主侧半球损伤致运动性失语、Gerstmann综合征1例报告

患者 ,男 ,46岁。晨起床后准备上班时突发左侧肢体不能活动 ,言语不能 ,小便失禁 3小时于 2 0 0 0年 3月 10日抬送入院。患者为右利手。有长期吸烟史 ,无心脏病史。查体 :完全性运动性失语 ,听力正常 ,能通过点头或摇头方式回答医师提问 ,书写、计算、左右侧识别不能 ,双眼右斜视 ,伸舌左偏 ,左上下肢肌力 0～ 1级 ,偏身痛温觉减退 ,病理征阳性 ,右侧未见异常。心肺 (- )。头部 CT未见异常 ,血浆纤维蛋白原5 18mg/ dl(正常值 2 0 0～ 40 0 m g/ dl)。诊断脑梗死。入院后立即予降纤酶 2 0 U静滴 ,甘露醇脱水 ,尼莫地平解痉 ,丹参改善脑血液…     

获得性免疫缺陷综合征神经系统损害15例分析

目的 了解获得性免疫缺陷综合征(艾滋病)患者神经系统受累的临床特点.方法 回顾性分析2002年2月至2008年2月于我院住院的,伴有神经系统受累的艾滋病患者15例,其中8例因神经系统症状而首诊于神经科.结果 15例患者中人类免疫缺陷病毒(HIV)脑病者4例,其中1例表现为舞蹈症,1例HIV脑病合并HIV相关肌病;进行性多灶性白质脑病1例;进行性多灶性白质脑病合并弓形体脑病1例;HIV相关肌病1例;多脑神经损伤合并脊髓病1例;周围神经病1例;药物相关的神经肌肉病1例;脑膜脑炎4例;腑脓肿1例.结论 艾滋病累及神经系统的部位及表现形式多样,在临床工作中应提高警惕,注意筛查.     

Landau-Kleffner syndrome: a case report

A healthy 5 year old boy developed aphasia, attention disorder and hyperkinesia preceded by transient formed visual hallucinations and emotional outburst, immediately after a stressful event of forced separation from his father. EEG showed generalized epileptiform activity. He was diagnosed as Landau-Kleffner syndrome (LKS). CT and MRI of the brain were normal. SPECT showed left mesial temporal hypoperfusion. He improved on antiepileptics and ACTH.     

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.     

Prosodic preservation in Landau-Kleffner syndrome: a case report

Landau-Kleffner syndrome (LKS) is a rare acquired condition of auditory verbal agnosia and convulsive disorder in children. It has been proposed that there is a functional relationship between electrical disturbance and the speech defect. Prosody or the melody of language, as described by Monrad-Krohn (1947), is one aspect of non-verbal communication which is distributed bilaterally in the brain. Prosodic parameters of expression and perception in one 7.5-year-old child were tested to see if they were preserved as a means of communication. The child was observed during video-electroencephalogram (EEG) monitoring over a 48-hour period. All utterances were recorded and subject to analysis for the salience and variation of acoustic correlates of prosody. Prosodic comprehension was measured using specific perceptual tasks previously presented to normal children between the ages of 5.5 and 8.5. Despite being unable to meaningfully use or perceive phonemes, the child was able to use variations in fundamental frequency, duration and intensity of utterances, to convey both emotional and propositional intent. Similarly, the child was able to discriminate prosodic contours of a male adult voice to an age equivalent to 5.5 to 6.5 years. This argues in favour of the notion for educating such children not only through the visual channel but also through the auditory channel.     

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.     

The Landau-Kleffner syndrome: A review

The Landau-Kleffner syndrome or the syndrome of acquired epileptic aphasia was first described in 1957. The disorder is characterised by gradual or rapid loss of language in a previously normal child. All children have abnormal EEG compatible with the diagnosis of epilepsy, however, only 70% have clinical seizures. The present article presents a review of the current knowledge concerning this disorder. Information is provided related to the clinical picture, etiology, pathogenesis, treatment and outcome.

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Zusammenfassung Das Landau-Kleffner-Syndrom oder das Syndrom der erworbenen epileptischen Aphasie wurde erstmals 1957 beschrieben. Die Störung ist charakterisiert durch den allmählichen oder raschen Verlust der Sprache eines zuvor normal entwickelten Kindes. Alle Kinder haben abnorme EEG-Befunde, die mit der Diagnose einer Epilepsie vereinbar sind. Jedoch haben lediglich 70% der Betroffenen klinisch apparente Krampfanfälle. Der vorliegende Artikel faßt den gegenwärtigen Wissensstand zu dieser Störung zusammen. Es werden Informationen zum klinischen Bild, zur Ätiologie, Pathogenese, Behandlung und Verlauf vermittelt.

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Résumé Le syndrome Landau-Kleffner, ou bien le syndrome de l'aphasie épileptique acquise, a été découvert en 1957. Ce dysfonctionnement se charactérise par la perte graduelle ou rapide du langage chez un enfant jusque là normal. Tous les enfants présentent des électro-encéphalogrammes anormaux correspondant à des critères diagnostiques de l'épilepsie, mais 70% d'entre eux seulement ont des attaques cliniques. Cet article présente un survol des connaissances actuelles relatives à ce dysfonctionnement, et se propose de donner des informations quant à son aspect clinique, son étiologie, sa pathogénie, son traitement ainsi que son issue.

     

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.     

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.