[Landau-Kleffner syndrome: an analysis of 10 cases in Venezuela]. / Síndrome de Landau-Kleffner: análisis de 10 casos en Venezuela.

INTRODUCTION: Landau-Kleffner syndrome is characterised by acquired aphasia and encephalographic alterations that may or may not be accompanied by epileptic seizures. AIM. To analyse the clinical and encephalographic features and response to treatment of 10 patients with Landau-Kleffner syndrome. PATIENTS AND METHODS: We reviewed the patient records, encephalograms and treatment administered to patients catalogued as having Landau-Kleffner syndrome. RESULTS: The mean age of the patients was 44 months. Of these cases, 60% presented epilepsy when the diagnosis was established and 70% were found to have epileptic status during slow-wave sleep in the encephalographic study. Results showed that 40% corresponded to variants of Landau-Kleffner syndrome. No cause of the disease could be established in any of the patients. In the neuroimaging study, only one patient displayed abnormalities in the magnetic resonance imaging of the brain. All the patients received adrenocorticotropic hormone (ACTH)-based treatment, at a dose of 1 IU/kg/day for one month, administered together with antiepileptic drugs such as valproic acid and clobazam. Convulsive seizures and epileptic status during slow-wave sleep disappeared in all the patients. In the patients without epileptic status, epileptic activity became less frequent, although it did not completely disappear. Aphasia improved considerably, which meant that all the patients were able to enroll in normal schools. CONCLUSIONS: We believe that early diagnosis, together with suitable and timely management of aphasic patients with encephalographic alterations that allow ACTH to be used at low doses, make it possible to offer an early education so as to provide maximum recovery from the disease.

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

Some neurological disorders may present psychiatric signs and symptoms, therefore the search for an etiological diagnosis is crucial. The aim of this study is to report the case of a patient with a neurological disorder, diagnosed during a psychiatric admission. A boy with normal neuropsychomotor development until the age of 3 years, started presenting epileptic seizures, followed by behavioral disorder and language deterioration. During neurologic follow-up, the patient was referred to the Psychiatry Department with a diagnosis of autism, in this case an autistic regression (AR). During his admission, diagnosis of Landau-Kleffner syndrome (LKS) was established on clinical and EEG grounds. LKS is characterized by acquired aphasia, epilepsy, EEG abnormalities and behavioral changes, including autistic traits. Language regression is observed LKS and AR. We stress the main differences between these two entities because misdiagnosis may postpone early intervention and consequent benefits, as observed in our case.

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.

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

Sindrome de Landau-Kleffner (a propósito de 2 casos) / Landau-Kleffner syndrome (apropos of 2 cases)

Reportamos el caso de un niño de 4 años de edad, y de una niña de 2 años 5 meses, que fueron admitidos en el Hospital Guillermo Almenara Irigoyen -IPSS (Lima-Perú) por presentar crisis convulsivas parciales motoras sin generalización secundaria, y que cursaron con un disturbio afásico del lenguaje, cambios en la conducta y pérdida del control de esfínteres. Presentamos datos clínicos y exámenes auxiliares que indujeron a sospechar del sindrome de Landau-Kleffner, una entidad bastante infrecuente y caracterizada principalmente por afasia neuropsicológicos y que en uno de los casos reportados la aparente etiología es una infección por Citomegalovirus. Se revisa y actualiza la bibliografía al respecto(AU)

Sindrome de Landau-Kleffner (a propósito de 2 casos) / Landau-Kleffner syndrome (apropos of 2 cases)

Reportamos el caso de un niño de 4 años de edad, y de una niña de 2 años 5 meses, que fueron admitidos en el Hospital "Guillermo Almenara Irigoyen" -IPSS (Lima-Perú) por presentar crisis convulsivas parciales motoras sin generalización secundaria, y que cursaron con un disturbio afásico del lenguaje, cambios en la conducta y pérdida del control de esfínteres. Presentamos datos clínicos y exámenes auxiliares que indujeron a sospechar del sindrome de Landau-Kleffner, una entidad bastante infrecuente y caracterizada principalmente por afasia neuropsicológicos y que en uno de los casos reportados la aparente etiología es una infección por Citomegalovirus. Se revisa y actualiza la bibliografía al respecto

Brain single photon emission computed tomography imaging in Landau-Kleffner syndrome.

Five right-handed children with Landau-Kleffner syndrome (LKS) who had disease onset between the ages of 3 and 9 years were studied with EEG and single-photon emission computed tomography (SPECT) before and, in four cases, after 6 months of corticosteroid treatment. EEG findings included both focal and generalized spikes as well as spike-wave discharges with bilateral temporal predominance. These increased markedly during sleep in 1 child, and continuous spike-and-wave complexes appeared during slow-wave sleep in another patient. Neuropsychological testing demonstrated verbal auditory agnosia. Magnetic resonance imaging (MRI) was performed in 4 children and was normal. Brain SPECT imaging demonstrated abnormal perfusion in the left temporal lobe in all patients. The response to corticosteroid therapy was mixed. Our findings reinforce the concept that LKS is a functional disease affecting the language-dominant brain areas. We conclude that SPECT imaging may be of diagnostic assistance in the evaluation of this syndrome of unknown etiology.