Hipermetabolismo temporal izquierdo en síndrome de Landau-Kleffner demostrado con PET F18-FDG: reporte de un caso con remisión metabólico-clínica postratamiento / Left temporal hypermetabolism detected in Landau-Kleffner syndrome with F18-FDG PET/CT: report of a case with metabolic and clinical remission after treatment

Brain F18-FDG Positron Emission Tomography (PET) has been used for studying focal epilepsy, with high sensitivity in detection of epileptogenic foci, even with normal magnetic resonance imaging (MRI). Some cases of Landau-Kleffner Syndrome (LKS) have shown PET abnormalities, mostly showing uni- or bilateral temporoparietal hypometabolism, although a heterogeneous group of alterations have been described. We report a case of LKS with a left hypermetabolic temporo-occipital area that responded to treatment, with clinical improvement and remission of PET hypermetabolic focus.

La positron emission tomography (PET) cerebral con F18-FDG ha sido utilizado para estudiar epilepsias focales con alta sensibilidad en la detección del área epileptógena, aun con resonancia magnética (RM) normal. La PET ha mostrado positividad en algunos casos de síndrome de Landau-Kleffner (SLK), la mayoría de las veces evidenciando hipometabolismo temporoparietal uni o bilateral, aunque un grupo heterogéneo de alteraciones asociadas ha sido descrito. Presentamos un caso de SLK con un área hipermetabólica temporooccipital izquierda, que respondió al tratamiento, con mejoría clínica y regresión del foco hipercaptante a la PET.

Efficacy of methylprednisolone therapy for electrical status epilepticus during sleep in children / 中华儿科杂志

<p><b>OBJECTIVE</b>To evaluate the therapeutic effect of methylprednisolone for electrical status epilepticus during sleep (ESES) in children.</p><p><b>METHOD</b>The clinical and EEG data of 82 epilepsy patients with ESES, which included benign childhood epilepsy with centro temporal spikes (BECT) variants, epilepsy with continuous spikes and waves during slow sleep (CSWS) , Landau-Kleffner syndrome (LKS) collected from department of pediatrics, Peking University First Hospital were analyzed from July 2007 to September 2012. During ESES period, all patients received methylprednisolone treatment for three courses, which included methylprednisolone intravenous infusion for three days, followed by oral prednisone for four days every time. After three courses, prednisone [1-2 mg/(kg × d)] were taken by all patients for 6 months. The ESES phenomenon and seizures were observed before and after treatment. The efficacy of corticosteroid on ESES suppression, seizure control of three epilepsy syndrome were analyzed.</p><p><b>RESULT</b>Thirty-nine cases were male and 43 cases were female. The epilepsy syndromes included 49 patients diagnosed as benign childhood epilepsy with centrotemporal spike (BECT) variants, 27 patients diagnosed as epilepsy with continuous spikes and waves during slow sleep (CSWS), and 6 patients diagnosed as LKS. Age of onset ranged from 1 year and 4 months to 11 years. The age of ESES newly monitored was from 2 years to 10 years and 8 months. The total effective rate of corticosteroid was 83% (68/82) for ESES, BECT variants was 82% (40/49), CSWS was 81% (22/27), LKS was 100% (6/6). There was no statistically significant difference in effective rates between the front two (χ² = 0.09, P > 0.05). The seizures were improved in the first month after methylprednisolone treatment in 3 epilepsy syndromes. The recurrence rate of BECT variants was 47% (23/49) , CSWS was 59% (16/27) , LKS was 50% (3/6) after 1 year follow up. There was no association between disease parameters, including age at seizure onset, duration of ESES and the treatment effect of ESES examined by Kruskal-Wallis method (χ² = 3.585, 0.932, P > 0.05).</p><p><b>CONCLUSION</b>Methylprednisolone was effective for improving ESES and seizures in 3 epilepsy syndromes combined with ESES. There was no significant correlation between age at seizure onset, duration of ESES and treatment effect of ESES.</p>

Landau-Kleffner syndrome without seizures: would speech delay justify the treatment with antiepileptic drugs?: [review] / Síndrome de Landau-Kleffner sem crises: atraso na aquisição da fala justifica o tratamento com drogas epilépticas?: [revisão]

INTRODUCTION: Landau-Kleffner Syndrome is a rare epileptic syndrome characterized by the association of receptive aphasia, epileptic seizures, behavioral disorders and electroencephalographic changes with discharges at the temporal lobe unilaterally or bilaterally. Seizures are not essential for diagnosis. CASE REPORT: J. V. S. S., 3 y/o male, with delay in acquirement of speech, psychomotor agitation and sleep disorder (sleeplessness). Attempt to communicate was established by gestures and pointing at things. His behavior characterized by agitation, restlessness, aggressiveness and difficulty to establish social contact with other children by the same age. MRI was normal and the EEG showed sharp-wave discharges in the left medial and posterior temporal regions. After three months of treatment with carbamazepine the child returned to an evaluation, presenting substantial improvement at speech, speaking simple words and with meaningful improvement on both behavioral and sleep patterns, as well as, social interaction. DISCUSSION: Expression and reception speech disorders are relatively common in children with different epileptic syndromes, particularly in Landau-Kleffner syndrome. Epileptiform discharges can occur in people without epilepsy and use to be called subclinical discharges. What would be the impact of subclinical discharges on this population remains uncertain at the present time. We reviewed these interactions in the light of a representative case, showing favorable outcome following introduction of an antiepileptic drug.

INTRODUÇÃO: Síndrome de Landau-Kleffner é uma síndrome epiléptica rara caracterizada pela associação de afasia de recepção, crises epilépticas, distúrbios do comportamento e alterações eletrencefalográficas com descargas nos lobos temporais unilaterais ou bilaterais. Crises convulsivas não são essenciais para o diagnóstico. RELATO DO CASO: J.V.S.S., masculino, três anos de idade, com atraso na aquisição da fala, agitação psicomotora e distúrbio do sono. Ele usava gestos e apontava para os objetos tentando se comunicar. Seu comportamento era muito agitado, impaciente, períodos de agressividade e muita dificuldade em estabelecer contato social com outras crianças da mesma idade. RNM foi normal e o EEG mostrou descargas de ondas agudas nas regiões temporais média e posterior esquerda. Após três meses de tratamento com carbamazepina a criança retornou para reavaliação, apresentando importante melhora da fala, falando palavras simples, melhora do comportamento e do padrão de sono e da interação social. DISCUSSÃO: Alterações na linguagem de recepção e de expressão são relativamente comuns em crianças com diferentes síndromes epilépticas, particularmente na síndrome de Landau-Kleffner. Descargas epileptiformes podem ocorrer em pessoas sem epilepsia sendo denominadas descargas subclínicas. Qual o impacto das descargas subclínicas para estas pessoas é incerto até o momento. Relatamos o caso de um menino com descargas epileptiformes no EEG, sem história de crises convulsivas e excelente evolução clínica após tratamento com droga antiepiléptica.

Síndrome de Landau-Kleffner / Landau-Kleffner syndrome

El síndrome de Landau-Kleffner es un trastorno poco frecuente en niños, que debe sospecharse en pacientes que presentan regresión del lenguaje adquirido y muchas veces se asocian otras manifestaciones como crisis convulsivas, trastornos de conducta y alteraciones psiquiátrica. En este artículo se publica una revisión actualizada de esta patología, para que los pediatras consideren este diagnóstico en un niño con afasia.

Utilidad de la electroencefalografía en las epilepsias y síndromes epilépticos de la infancia / Utility of electroencephalography in epilepsy and epileptic syndrome of childhood

Se revisó la utilización del electroencefalograma (EEG) en el manejo de las epilepsias y síndromes epilépticos, como una herramienta para el diagnóstico y pronóstico del paciente con crisis epilépticas en el área de la neuropediatría, correlacionando las manifestaciones clínicas, edad de inicio, frecuencia, pronóstico y características electroencefalográficas de los trazados de cada una de ellas. El electroencefalograma goza de una extraordinaria vigencia en las neurociencias, dado que es un instrumento del que disponemos para aproximarnos realmente al funcionamiento cerebral en tiempo reales decir, a escala de milisegundos. A pesar de que en el último decenio se ha producido un desarrollo espectacular de las técnicas de neuroimágenes y funcionales, como la tomografía axial computarizada (TAC), resonancia magnética cerebral (RM) con sus variantes funcionales, la tomografía de emisión de positrones (PET), la tomografía computarizada de emisión de fotones (SPECT) y la magnetoencefalografía (MEG), la llegada de esas técnicas no ha conseguido relegar al EEG a un papel secundario. La calidad del EEG va a depender de los datos obtenidos, de una evaluación adecuada para llegar a conclusiones valederas que se obtengan como producto de la habilidad, entrenamiento y experiencia de la persona a cargo de realizarlo. El EEG aporta el diagnóstico definitivo en muchas de las epilepsias y síndromes epilépticos de la infancia.

The utility of the electroencephalogram (EEG) in handling childhood epilepsies and epileptic syndromes was reviewed as a diagnosis and prognosis tool in the patient with epileptic crisis. Correlations were stablished between clinical manifestations, age of onset, frequency, prognosis and electroencephalographic characteristics of the layouts of each one of the different types. EEG is extremely useful in neurosciences; since it is the only instrument that we have to assess the brain´s function in real time, that is to say, at the scale of milliseconds. Although in the last decade a spectacular development of neuroimages and functional techniques has taken place, as with computerized axial tomography (CAT), cerebral magnetic resonance (MR) with its fuctional variants, positron emission tomography (PET), photon emission computerized tomography (SPECT) and magnetoencephalography (MEG), the arrival of those techniques has not been able to relegate EEG to a secondary place. The utility of the EEG depends on an adequate evaluation that will lead to valid conclusions. The accuracy of this evaluation is related to the ability, training and experience of the operator. The EEG contributes to the definite diagnosis in many of childhood epilepsies and epileptic syndromes.

Crise febril: correlação clínico-eletrencefalográfica em 1162 crianças / Febrile seizures: clinical-electroencephalographic correlation in 1162 children

INTRODUÇÃO: Crise febril (CF) é comum, e ocorre em aproximadamente 2 a 5 por cento das crianças até 5 anos de idade. OBJETIVO E MÉTODOS: Avaliar a relação entre aspectos clínicos e ocorrência de atividade epileptiforme (AE) no eletrencefalograma (EEG) em crianças com CF e, naquelas em que houve seguimento, observar, também, o aparecimento de crises epilépticas não febris (CNF). RESULTADOS: Foram avaliadas 1162 crianças de 4 meses a 5 anos de idade. Houve predomínio de CF simples (82,4 por cento) e de episódio único (62,0 por cento). Houve maior recorrência de CF quando a primeira crise ocorreu antes dos 12 meses de idade ou quando era complexa. AE foi encontrada em porcentagem crescente com a idade da criança; em 3 por cento dos pacientes até os dois anos de idade e 33 por cento após os 4 anos. Pontas evocadas pela percussão de pés ou mãos (PE) ocorreu em 3,4 por cento dos casos. CNF foi relatada em 38 (9,5 por cento) crianças. Não houve correlação estatisticamente significativa entre o tipo e número de CF, idade de ocorrência da primeira CF, presença de AE e de PE no EEG e a ocorrência ou o tipo de CNF, no seguimento. DISCUSSÃO: Há aspectos ainda controversos na literatura, como o prognóstico em relação ao desenvolvimento de epilepsia e o eventual efeito de AE persistente quanto à cognição e comportamento nas crianças que apresentam CF, a exigir pesquisas prospectivas longitudinais.

INTRODUCTION: Febrile seizures (FS) occur in about 2-5 percent of the children from the age of 6 months do 5 years of age. OBJECTIVE: The aim of this study is to analyze clinical and EEG aspects of children with febrile seizures. Methods: We retrospectively studied 1162 children (age range: 4 months to 5 years) with FS. The relationship between clinical and EEG abnormalities was statistically analyzed. RESULTS: In the majority of cases there were simple febrile seizures (82 percent) and unique seizures (62 percent). The recurrence rate of FS was higher for children with the first seizure bellow 12 months of age, and when the FS were complicated. Ninety-five (8.1 percent) children showed focal epileptiform activity (EA) in the first EEG, and 3.4 percent had somatosensory evoked spikes by foot or hand stimulation. There was a increase in percentage of EA with the increase of age (3 percent until 12 months and 33 percent after 48 moths). Afebrile seizures subsequent to the FS occurred in 9,4 percent of the cases. EA, a family history of seizures, and the type of FS were not significant risk factors for subsequent afebrile seizures. DISCUSSION: In order to understand the relationship between FS and the occurrence of epilepsy, and to evaluate possible cognitive and behaviour problems associated to the persistent epileptiform activity there is a need of longitudinal studies.

Avaliação audiológica e eletrofisiológica da audição na síndrome de Landau-Kleffner / Audiologic and electrophysiologic evaluation in Landau-Kleffner syndrome

OBJETIVO: Descrever os resultados obtidos nas avaliações audiológica e eletrofisiológica da audição, verificando a ocorrência de alterações auditivas periféricas e/ou centrais, de indivíduos com síndrome de Landau-Kleffner. MÉTODOS: Foram submetidos à avaliação audiológica (inspeção do meato acústico externo, medidas de imitância acústica, audiometrias tonal e vocal) e eletrofisiológica da audição (potenciais evocados auditivos de curta, média e longa latência), quatro indivíduos com diagnóstico de síndrome de Landau-Kleffner, na faixa etária de nove a 19 anos, encaminhados ao Laboratório de Investigação Fonoaudiológica em Potenciais Evocados Auditivos do Curso de Fonoaudiologia da Universidade de São Paulo. RESULTADOS: Os resultados mostraram que 100 por cento dos indivíduos apresentaram alteração em pelo menos uma das avaliações realizadas, sendo que houve uma maior ocorrência de alterações no potencial evocado auditivo de média latência (100 por cento dos indivíduos apresentaram alterações). CONCLUSÕES: Observou-se uma grande ocorrência de alterações nos resultados das avaliações audiológicas e eletrofisiológicas da audição em indivíduos com síndrome de Landau-Kleffner. Enfatiza-se a importância da investigação da função auditiva destes indivíduos a fim de verificar possíveis relações entre os déficits da comunicação e alterações auditivas que possam estar presentes nessa população.

PURPOSE: To describe the audiological and electrophysiological results of individuals with Landau-Kleffner syndrome, verifying the occurrence of peripheral and/or central auditory disorders. METHODS: Four individuals with Landau-Kleffner syndrome with ages ranging from nine to 19 years old, referred to the Auditory Evoked Potentials Laboratory of the Speech and Language Pathology and Audiology Course of the University of São Paulo, were submitted to audiologic (otoscopy, immitance measurements, pure tone and speech audiometry) and electrophysiological evaluation (short, middle and long-latency auditory evoked potentials). RESULTS: All individuals showed alterations in at least one of the evaluations performed, and it was observed a higher rate of alterations in the middle latency response (100 percent of the individuals showed alterations). CONCLUSIONS: A great occurrence of auditory disorders in audiological and electrophysiological evaluation of individuals with Landau-Kleffner syndrome was observed. It is emphasized the importance of the audiological investigation of these individuals, in order to verify possible relations between communication and auditory disorders in this population.

Clinical characteristics and long-term prognosis of Landau-Kleffner syndrome / 中华儿科杂志

<p><b>OBJECTIVE</b>To investigate the clinical and electroencephalographic (EEG) characteristics, therapeutic response and long-term prognosis of Landau Kleffner syndrome (LKS).</p><p><b>METHODS</b>The clinical and EEG data of 10 children with LKS were analyzed, and therapeutic response and long-term outcome were followed up.</p><p><b>RESULTS</b>The age of onset was from 2 to 10.5 years of age. All patients had acquired aphasia, characterized by verbal auditory agnosia. All patients had epileptic seizures. Partial motor seizures during sleep occurred in 8 patients, and other seizure type including atypical absence seizure and generalized tonic-clonic seizure were also observed. Psychological and behavioral abnormalities occurred in 9 patients. There were no abnormalities of hearing and neuro-imaging tests in all patients, and family histories were negative. All the patients had EEG abnormalities. Focal spike and waves of temporal lobe were recorded in 9 patients. Electrical status epilepticus during sleep (ESES) was observed on Video-EEG (VEEG) monitoring in 4 patients. Anti-epileptic drugs (AEDs) showed favorable effects on epileptic seizures, but no effects on aphasia. All patients responded to corticosteroid, and got language improved. Eight patients were followed up for long-term outcome. All patients were seizure free, while the level of language development was abnormal in 5 patients. The VEEG follow-up was conducted in 6 patients. Continuous epileptic discharges in slow sleep recurred in 2 patients after the discontinuation of steroid therapy.</p><p><b>CONCLUSIONS</b>LKS is one of the childhood epileptic encephalopathy, and acquired aphasia and epileptic seizures are two main clinical characteristics. Aphasia is characterized by verbal auditory agnosia. Psychological and behavioral abnormalities are very common in children with LKS. Focal epileptic discharges were often located in temporal area, and usually generalized, and could be continuous during sleep. AEDs could control seizure but had no effects on aphasia. Early use of full dose corticosteroids could improve the language significantly. Long-term follow up showed that language impairments often remained, but the outcome in terms of EEG and epileptic seizure was good.</p>

[Landau-kleffner syndrome]

Landau-Kleffner syndrome is a rare disorder caraterised by acquired aphasia of children, paroxysmal electroencephalographic disturbance and in 75% of cases epileptic seizures and cognitives disturbances. We report a 6 year old daughter who presented epileptic seizures and six months later regression langage and a behaviour of deaf. This patient had a global aphasia. The understanding was affected because of the cortical deafness. The electroencephalogramme showed discharges in the temporal bilateral regions. The cerebral scan and the potential evoked auditory were normal. The Landau-Kleffner syndrome's diagnosis was confirmed. The patient was treated by valproic acid and corticosteroids with good evolution. The stoopage of treatment has brought more seizure and the worsening aphasia. The authors discussed the pathogenic hypothesis, the semiology and the treatment of the Landau-Kleffner syndrome

Afasia epiléptica adquirida o síndrome de Landau Kleffner: a propósito de un caso / Acquired epileptic aphasia or landau kleffner syndrome: a case study

La Afasia Epiléptica adquirida o Síndrome de Landau Kleffner, es una afección que se caracteriza por agnosia auditiva adquirida, EEG paroxísticos, crisis epilépticas (67,6 por ciento), problemas conductuales y cognoscitivos. La observación de un infante preescolar de 4 años de edad, sexo femenino, quien presentó crisis convulsiva de carácter tónico-clónico generalizadas, con dificultad para la comprensión del lenguaje hablado y presencia en el EEG de sueño compatible con status eléctrico durante el sueño lento. Con la administración de ácido valproico, clobazan, fenobarbital y ACTH, se obtuvieron resultados satisfactorios, desapareciendo el cuadro convulsivo y mejorando el lenguaje. El diagnóstico temprano de este síndrome permite una atención y manejo adecuado, ofreciéndole un mejor pronóstico al niño

Two Cases of the Landau-Kleffner Syndrome / 대한이비인후과학회지

Landau-Kleffner syndrome, or acquired epileptiform aphasia, is an epilepsy syndrome with variable disruption of acquired language and epileptiform discharges on electroencephalograph (EEG). Auditory agnosia can deteriorate into total unresponsiveness and impaired expressive communication. In spite of the presence of this condition, the pure tone audiometry, otoacoustic emission and brainstem evoked auditory potential could be normal. Therefore, in the above hearing tests which are the mainstream methods for pediatric hearing evaluation, otolaryngologists should have suspicion for the presence of this syndrome, although it is rare.

Síndrome de landau kleffner: a propósito de un caso / Syndrome of landau kleffner: a purpose of a case

Síndrome Landau Kleffner es una enfermedad neurológica poco común de la infancia, presente en niños 3-7 años con desarrollo normal, caracterizándose por perdida gradual del lenguaje, crisis convulsivas, regresión neurológica, anormalidades electroencefalográficas. Caso clínico; escolar masculino 7 años que acude a consulta privada presentando convulsiones tónico clónicas generalizadas, afasia, perdida gradual de mortalidad, desarrollo normal hasta los 4 años, posterior a crisis convulsiva evidenciamos regresión neurológica progresiva. Realizamos EEG observando complejos punta onda multifocales, disminución en el trazado, reafirmando el diagnóstico, instaurándose tratamiento: Valproato y Clonazepan, obteniendo buena evolución

Epilepsy : Other Supplementary Treatments

Conventional anti-epileptic treatments have limitations in treating many epileptic patients. Although there have been great advances in the management of epilepsy by application of newly developed anti-convulsants and surgery, some patients still remain in an intractable condition. Other supplementary treatments including immunoglobulin, steroids, and ketogenic diet have significant anti-epileptic potencies. Immunoglobulin treatment shows benefits in some autoimmune-related epileptic conditions such as Rasmussen's encephalitis, however, still has limitations in long-term efficacy. Steroids show significant improvements in many epileptic syndromes including infantile spasm, Lennox-Gastaut syndrome, Landau-Kleffner syndrome, and epilepsy with continuous spikes and waves during slow wave sleep, however, some of them recur during or after tapering of the drug. Ketogenic diet has become one of the most reliable treatments for intractable epilepsy especially in children. Ketogenic diet is difficult to maintain because of poor palatability, but shows a very high anti-epileptic efficacy. These supplementary treatments should be considered in various epileptic conditions especially in intractable patients.

Clinical Reviews of Landau-Kleffner Syndrome

PURPOSE: We intended to describe the clinical features including characteristic etiologies, therapeutic approaches and outcomes for Landau-Kleffner syndrome(LKS). METHODS: A retrospective chart reviews were done to reveal the clinical and electrophysiological features in 5 patients who were diagnosed as LKS and undergone extensive diagnostic work-up and various therapeutic interventions. RESULTS: Among five LKS patients, 2 patients were males and 3 were females. All patients showed well controlled seizure outcomes but cognitive function including auditory and/or expressive aphasia were not improved in spite of using various anticonvulsants. Only two patients responded to steroid therapy but one patient showed repeated deterioration after discontinuation of the durg. Among 3 patients who were on ketogenic diet(KD), one patient showed a dramatic improvement in liguistic and cognitive functions in spite of underlying mitochondrial complex I deficiency. Multiple subpial transections (MST) were done in the other two patients because of incomplete recovery from ketogenic diet, and only one patient showed fairly successful improvement. CONCLUSION: Cognitive regression induced by LKS could be successfully improved by various therapeutic modalities including steroid, KD and palliative MST in most patients.

Audiometria de tronco encefálico e síndrome de Landau-Kleffner: estudo de caso / Auditory brainstem response and Landau-Kleffner syndrome: case report

Objetivo: Relatar o caso de um paciente com diagnóstico de Síndrome de Landau-Kleffner (SLK) que foi acompanhado fonoaudiologicamente. Método: Realização de Audiometria, Logoaudiometria, Medidas de Imitância Acústica, Audiometria de Tronco Encefálico (ABR) e avaliação de linguagem durante um período de 7 meses, pré e pós tratamento com corticóides administrados após crises convulsivas. Resultados: Observaram-se mudanças nos resultados das avaliações audiológica e de linguagem após tratamento medicamentoso. O resultado da Audiometria de tronco encefálico também mostrou melhora após o tratamento com corticóides. Conclusão: Necessidade de mais estudos que relacionem alterações de linguagem com alterações na audiometria de tronco encefálico, com o objetivo de obter um melhor prognóstico para os indivíduos portadores da Síndrome de Landau-Kleffner.

Landau Kleffner syndrome: electroclinical and etiopathogenic heterogeneity.

Landau - Kleffner syndrome is a rare, functional, age-related epilepsy with aphasia and epileptiform discharges on EEG. The heterogenity of clinical presentations, course, long-term outcome and response to treatment suggests multiple underlying etiologies. Normal children abruptly develop deterioration of language functions along with spike and wave discharges on EEG. Clinical seizures may or may not be present. The aphasia responds poorly to most drugs. Valproic acid and benzodiazepines are most effective. Steroids and intravenous immunoglobulins have shown a variable response. Long-term outcome of aphasia is variable, many patients persist with residual impairment. Important questions regarding etiopathogenesis are unanswered.

Síndrome de Landau-Kleffner e regressäo autística: a importância do diagnóstico diferencial / Landau-Kleffner and autistic regression: the importance of differential diagnosis

Algumas doenças neurológicas podem apresentar sinais e sintomas psiquiátricos, portanto a exploraçäo do diagnóstico etiológico é crucial. O objetivo deste estudo é relatar o caso de um paciente com um distúrbio neurológico, diagnosticado durante internaçäo psiquiátrica. Um menino com desenvolvimento neuropsicomotor normal até 3 anos, quando começou a apresentar crises epilépticas, seguidas por distúrbio de comportamento e deterioraçäo da linguagem. Durante o acompanhamento neurológico, o paciente foi encaminhado ao Departamento de Psiquiatria com a suspeita de autismo, regressäo autística (RA). Durante internaçäo, o diagnóstico de síndrome de Landau-Kleffner (SLK) foi estabelecido em bases clínicas e eletrencefalográficas. A SLK é caracterizada por afasia adquirida, epilepsia, anormalidades eletrencefalográficas e distúrbios de comportamento, incluindo traços autísticos. A regressäo da linguagem é observada na SLK e na RA. Enfatizamos as principais diferenças entre estas entidades, pois o diagnóstico errôneo adia a intervençäo precoce e benefícios, como observado em nosso caso

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