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.     

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.     

Multiple subpial transection in Landau-Kleffner syndrome

We have considered multiple subpial transection (MST) as a treatment option for Landau-Kleffner syndrome (LKS) for the past 6 years. The effect of this technique on language and cognitive ability, behaviour, seizures, and EEG abnormalities is analysed here. Five children (4 males, 1 female; aged 5.5 to 10 years) underwent MST with sufficiently detailed pre- and postoperative data for analysis. Behaviour and seizure frequency improved dramatically after surgery in all children. Improvement in language also occurred in all children, although none improved to an age-appropriate level. All five had electrical status epilepticus in sleep (ESES) before surgery, which was eliminated by the procedure. One child has had an extension of his MST due to the recurrence of ESES and accompanying clinical deterioration with good effect. An attempt is made to set the effect of MST against the natural history of the condition. MST is an important treatment modality in LKS, although the timing of this intervention and its effect on final language outcome remains to be defined.     

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.     

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.     

Changing perspectives on Landau-Kleffner syndrome

Landau-Kleffner syndrome (LKS) is a childhood disorder characterized by an acquired aphasia that emerges in association with epileptiform electroencephalographic abnormalities. The language loss is often characterized by a severe disturbance of auditory language comprehension (verbal auditory agnosia) combined with a substantial disruption of expressive language. Comorbid behavioral disturbances commonly involve hyperactivity and attentional problems but sometimes encompass a more pervasive pattern of difficulties resembling an autism spectrum disorder. Now one the most frequently described forms of acquired aphasia in children, LKS has had a profound influence on both neurological practice and cognitive neuroscience. Here, we review current conceptualizations of LKS, consider its pleomorphic manifestations and discuss existing and future diagnostic issues and dilemmas. The potential relevance of LKS to understanding other disorders, including autistic regression, is considered.     

Efficacy of intravenous immunoglobulin in Landau-Kleffner syndrome

We administered 2 gm/kg of intravenous gamma globulin (IVIG) to each of five consecutive patients with Landau-Kleffner syndrome, over 4 days. We compared the 1-month baseline to that following IVIG using a severity score assessing speech, comprehension, behavior, seizures, and electroencephalography. There was a significant drop in this score after IVIG (P = 0.025). Two patients had a dramatic response to IVIG, with complete resolution of symptoms. This finding suggests that IVIG has at least some efficacy for the therapy of Landau-Kleffner syndrome.     

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.     

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。     

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.

     

The surgical treatment of Landau-Kleffner syndrome

The medical management of Landau-Kleffner syndrome is usually effective for seizure control and eventual seizure remission. However, the response for language and behavior is often poor. Surgery, in the form of multiple subpial transections (MSTs) to include Wernicke's area has been suggested as a way forward if electrophysiologic lateralization can be demonstrated. Surgical series in the literature are few and outcome from surgery variable     

Specific language impairment versus Landau-Kleffner syndrome

The occurrence of sleep electroencephalography (EEG) abnormalities in some children with specific language impairment (SLI), the various forms of language dysfunction patterns seen in children with benign childhood epilepsy with centrotemporal spikes (BECTS), and finally the acquired aphasia in Landau-Kleffner syndrome (LKS) indicate a large spectrum of interactions between language and epilepsy. As such, the question is whether SLI and LKS should rather be considered along a continuum or as two entirely distinct entities. In addition, the rationale for using antiepileptic medications in rare forms of SLI is discussed.     

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.     

Auditory processing deficits in a teenager with Landau-Kleffner syndrome

Abstract

This study explores the auditory processing skills of a 14-year-old boy (William) with an acquired aphasia, diagnosed as Landau-Kleffner syndrome. A single case study design was implemented, with the use of both chronological age-matched and receptive-language-matched control participants. Tasks were designed to investigate non-linguistic and linguistic auditory processing. The results indicated that William had intact peripheral hearing and gap detection abilities. However, William was significantly impaired at detecting a tone presented before or during some masking noise. William also showed significant impairments in three auditory processing tasks, requiring discrimination of both word and non-word stimuli. Findings suggest that William has an auditory processing deficit affecting the perception and discrimination of linguistic and some non-linguistic stimuli.