Landau-Kleffner综合征1例

病例报告,女,9岁,因发作右侧肢体抽搐伴言语障碍2年于2003年9月13日入院.患儿于2年前因发热、头痛、呕吐,在当地医院诊断为"脑炎",经治疗后头痛消失,体温正常,逐渐出现言语不清,发单声,不能连贯表达自己的思想,不能理解他人话语.     

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     

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.     

Landau-KIeffner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.     

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...     

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。     

Landau-Kleffner syndrome: study of four cases

We describe four patients with clinical features of Landau-Kleffner syndrome and discuss electroencephalographic features, treatment and prognosis. Anticonvulsants and prednisone were used for treatment with good control of seizures in all cases and a less effect response in acquired aphasia. Further studies are necessary to elucidate the causes and management of this syndrome.     

Landau-Kleffner syndrome with unilateral EEG abnormalities--two cases from Beijing, China

We reported 2 cases of Landau-Kleffner syndrome from Beijing whose clinical manifestations were very similar to ones previously reported. The onset of symptoms in these 2 cases was at 3 and 4 years of age, respectively, and both were characterised by unilateral focal epileptiform EEG discharges, without remarkable clinical seizures. One patient had normal SPECT study in addition to normal CT scan, BAER and VER results; the other had a family history of epilepsy. Since this syndrome may involve a primary brain disturbance responsible for both the epilepsy and aphasia without clear structural brain lesions, we wonder whether it can be classified as one of the benign functional partial epilepsies with genetic factors.     

Landau-Kleffner syndrome responsive to levetiracetam

A 5-year-old girl with Landau-Kleffner syndrome is discussed. The child began having seizures at age 4 associated with language deterioration despite anticonvulsant therapy. With levetiracetam monotherapy to a dose of 60 mg/kg/day and discontinuation of carbamazepine and valproic acid, her language has improved and seizures are controlled. Levetiracetam should be considered as therapy for Landau-Kleffner syndrome.     

Sign language in Landau-Kleffner syndrome

This article reviews the history of sign language (SL) and the rationale for its use in children with profound auditory agnosia due to Landau-Kleffner syndrome (LKS), illustrated by studies of children and adults followed for many years and rare cases from the literature. The reasons that SL was successful and brought some children out of isolation while it could not be implemented in others are discussed. The nowadays earlier recognition and treatment of LKS and better awareness of the crucial need to maintain communication have certainly improved the outcome of affected children. Alternatives to oral language, even for less severe cases, are increasingly accepted. SL can be learned at different ages with a clear benefit, but the ambivalence of the patients and their families with the world and culture of the deaf may sometimes explain its refusal or limited acceptance. There are no data to support the fear that SL learning may delay or prevent oral language recovery in children with LKS. On the contrary, SL may even facilitate this recovery by stimulating functionally connected core language networks and by helping speech therapy and auditory training.     

Landau-Kleffner syndrome: long-term follow-up

Purpose

Landau–Kleffner syndrome (LKS) is a rare entity characterized by epilepsy and aphasia. It occurs in previously normal children, usually between three and seven years of age. The long-term outcome of LKS is not completely clear. The aim of this study is to verify the long-term follow-up of a group of patients with LKS, focusing on clinical and electroencephalographic (EEG) aspects, and quality of life. Methods: This was a transversal study. Between November 2006 and April 2007 seven patients with previous diagnosis of LKS were interviewed. They had had a follow-up of three to 16 years after their disease onset. They were all males between the ages of eight and 27 years old. All patients had normal MRI. Parents and/or patients were interviewed by one of the authors using a structured questionnaire. The Vineland Adaptive Behavior Scales, the Conner’s Rating Scales – Revised, and Short-Form Health Survey (SF 36) were used. Each patient had a prolonged interictal EEG recording. All patients had normal MRI. Results: The present investigation revealed that two patients still have seizures several years after epilepsy onset. One patient had total and three others had partial remission of language disturbance, while three patients still have aphasia and verbal auditory agnosia. With respect to quality of life, only one of our patients has a normal life at present. The remaining six patients with some sort of limitation consider the aphasia/agnosia to be the main difficulty in their lives. Five patients have normal EEGs. Conclusions: The long-term follow-up of patients with LKS shows that epilepsy and EEG abnormalities do not always disappear. Language disturbances tend to persist in most patients. The age of onset of language dysfunction does not seem to correlate with the prognosis for recovery of language function. Patients with LKS have an overall poor quality of life, mostly due to language difficulties.