Phonological short-term memory networks following recovery from Landau and Kleffner syndrome

Landau-Kleffner syndrome (LKS) is a rare acquired aphasia occurring in otherwise healthy children, together with spike-wave discharges predominating over superior temporal regions and activated by sleep. Although the outcome of language abilities is variable, a residual impairment in verbal short-term memory (STM) is frequent. This STM deficit might be related to the persistent dysfunction of those temporal lobe regions where epileptic discharges were observed during the active phase of the disorder. We tested this hypothesis by measuring brain activation during immediate serial recall of lists of 4 words, compared to single word repetition, using H(2) (15)O positron emission tomography (PET), in 3 LKS patients after recovery and in 14 healthy controls. The patients (TG, JPH, and DC) had shown abnormally increased or decreased glucose metabolism in left or right superior temporal gyrus (STG) at different stages during the active phase of their disease. At the time of this study, the patients were 6-10 years from the active phase of LKS. Results showed that Patients JPH and DC had impaired performance in the STM condition, whereas TG showed near normal performance. PET data showed that JPH and DC activated significantly less than controls left and right posterior STG. TG, having near normal STM performance, showed increased activity in the posterior part of the right STG. These data suggest that impaired verbal STM at late outcome of LKS might indeed be related to a persistent decrease of activity in those posterior superior temporal gyri that were involved in the epileptic focus during the active phase.     

Linguistic deficits in the acute phase of stroke

Background and Purpose For the diagnosis of aphasia early after stroke, several screening tests are available to support clinical judgment.None of these tests enables the clinician to assess the underlying linguistic deficits, i. e. semantic, phonological and syntactic deficits, which provides indispensable information for early therapeutic decisions. The ScreeLing was designed as a screening test to detect semantic, phonological and syntactic deficits. The ScreeLings sensitivity, specificity and accuracy in detecting aphasia and semantic, phonological and syntactic deficits were determined.Methods The ScreeLing was validated in an acute stroke population against a combined reference diagnosis of aphasia (aphasia according to at least two of the following measures:ne urologists judgment, linguists judgment, Tokentestscore). The three ScreeLing subtests were validated in the aphasic population against the presence or absence of a semantic, phonological and/or syntactic deficit according to an experienced clinical linguist.Results From a consecutive series of 215 stroke patients, 63 patients were included. The ScreeLing was an accurate test for the detection of aphasia (0.92),with a sensitivity of 86% and specificity of 96%. Sensitivity of subtests was 62 % for semantics, 54 % for phonology and 42 % for syntax. Specificity was 100 % for semantics and phonology and 80 % for syntax, and accuracy 0.84 for semantics, 0.87 for phonology and 0.64 for syntax.Conclusions The ScreeLing is an accurate test that can be easily administered and scored to detect aphasia in the first weeks after stroke. Furthermore, the ScreeLing is suitable for revealing underlying linguistic deficits, especially semantic and phonological deficits.     

Language Disturbances as Side Effects of Topiramate and Zonisamide Therapy

Reversible side effects of two sulfa-containing antiepileptic drugs (AEDs), topiramate (TPM) and zonisamide (ZNS), are reported. These effects differ from those of other AEDs in that language impairment was the predominant cognitive complaint. Information was available for 42 patients exposed to TPM. Twenty-two (52%) complained of adverse effects; 12, specifically of deficits in language-related functions. Brief neuropsychological testing in four patients on TPM confirmed verbal deficits. These deficits could appear shortly after initiating TPM and disappear variably after drug withdrawal. Similar complaints were seen in a pilot study of ZNS monotherapy, administered in supratherapeutic doses, confirmed by neuropsychological testing. TPM and ZNS both contain a sulfa moiety, suggesting that verbal processing is especially sensitive to these sulfa-containing AEDs.     

针药结合治疗急性期缺血性脑卒中失语症的临床研究

目的观察针药结合治疗急性期缺血性脑卒中失语症的疗效。方法急性期脑卒中失语症患者63例,按就诊先后顺序随机分为观察组(针灸 中药 常规西药)和对照组(常规西药治疗),其中观察组32例,对照组31例.观察比较2组病例治疗前后自发谈话、理解、复述、命名4项言语功能的得分和患者神经功能(NIHSS)积分变化,以及中医证候学和构音器官的症状变化。结果观察组临床疗效、自发言语和复述两项语言功能的改善情况显著高于对照组,证候学及构音器官症状的改善率观察组也有更好的变化趋势。结论针药结合治疗急性期缺血性脑卒中失语症可明显改善患者的言语功能和临床症状,明显提高临床疗效。     

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.     

A case of “crossed aphasia” in which the integrity of the left hemisphere is assessed by MRI

We describe a 65-year-old right-handed (on the Edinburgh Inventory Test) woman who developed left hemiplegia and language disturbances after right hemisphere lesion. She showed global aphasia and left spatial neglect. To our knowledge, this is the first case of so called “crossed aphasia” in which the integrity of the left hemisphere is assessed by Magnetic Resonance Imaging (MRI). The association of aphasia and neglect is briefly discussed.

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Sommario Descriviamo il caso di una donna destrimane che, in seguito a una lesione emisferica destra, sviluppò una emiplegia sinistra e disturbi del linguaggio. La paziente presentava afasia globale ed eminattenzione spaziale sinistra. L'interesse di questo caso deriva dal fatto che è il primo caso di cosiddetta “afasia crociata” in cui l'integrità dell'emisfero sinistro è stata valutata con la Risonanza Magnetica per Immagini (MRI). La concomitanza di afasia e neglect viene brevemente discussa.

     

脑外伤后失语症的非药物治疗方法综述

脑外伤后失语症的治疗尚无有效药物，总结除药物以外的有效治疗方法。     

汉语-朝鲜语-日语多语失语症治疗1例报道

目的探讨多语性失语症的临床特征及康复。方法报道1例母语为朝鲜语和汉语且日本语流利的在日本留学的中国留学生因车祸颅脑外伤导致多语失语症的临床康复过程。结果和结论经2～3个月康复治疗后,患者沟通能力进步,主要以理解能力及表达能力恢复为主。各种语言呈现不同的恢复。     

失语症命名障碍的类型及症状

本文综述了失语症命名障碍的常见症状以及基于临床特点的命名障碍的分类,包括产词性命名障碍、选词性命名障碍、语义性命名障碍、特殊范畴命名障碍、特殊传导通道命名障碍。     

语言中枢的低灌注、低代谢与失语症发病关系的临床研究

目的：通过功能磁共振揭示的语言功能区血流量及代谢的变化，探讨失语症可能的发病机制，为语言康复训练提供理论指导。方法：对10例有明确失语症临床表现。CT或MRI证实为脑卒中的患者进行北京医科大学第一医院的汉语失语症成套测验中的利手评定标准进行利手评定、西部失语评定标准判断失语症类型，采用Frenehy构音障碍评定标准进行构音障碍的评定，采用普通磁共振确定病变部位，并用功能磁共振对病变可能波及的语言功能区进行磁共振波谱分析及磁共振灌注成像分析，并与对侧相应区域进行对比。结果：10例脑卒中患者利手评定，结果有8例为右利手，2例为左利手，经西部失语评定有5例为运动性失语，4例为感觉性失语，1例为传导性失语，MRI提示病变部位均为左侧大脑半球，MRS均显示语言功能区的N-已酰天门冬氨酸、胆碱、肌酸的代谢较对侧相应区域降低；PWI显示受累语言功能区的局部脑血容量、局部脑血流量较对侧减低，对比剂平均通过时间、达峰时间较对侧延长。结论：失语症患者语言功能区均呈低灌注、低代谢表现，这可能为失语症的发病机制，在制订语言康复计划时，可适当提高语言功能区的灌注与代谢，促进失语症的康复。