RESUMO
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.
Assuntos
Epilepsia/fisiopatologia , Síndrome de Landau-Kleffner/fisiopatologia , Transtornos da Linguagem/fisiopatologia , Adolescente , Adulto , Fatores Etários , Agnosia/etiologia , Agnosia/fisiopatologia , Afasia/etiologia , Afasia/fisiopatologia , Criança , Eletroencefalografia/métodos , Epilepsia/etiologia , Seguimentos , Humanos , Síndrome de Landau-Kleffner/complicações , Síndrome de Landau-Kleffner/diagnóstico , Transtornos da Linguagem/etiologia , Imageamento por Ressonância Magnética , Masculino , Prognóstico , Qualidade de Vida , Inquéritos e Questionários , Fatores de Tempo , Adulto JovemRESUMO
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.
Assuntos
Síndrome de Landau-Kleffner , Convulsões , Hormônio Adrenocorticotrópico/uso terapêutico , Anticonvulsivantes/uso terapêutico , Afasia/diagnóstico , Afasia/tratamento farmacológico , Afasia/fisiopatologia , Pré-Escolar , Eletroencefalografia , Humanos , Lactente , Síndrome de Landau-Kleffner/complicações , Síndrome de Landau-Kleffner/diagnóstico , Síndrome de Landau-Kleffner/tratamento farmacológico , Síndrome de Landau-Kleffner/fisiopatologia , Estudos Retrospectivos , Convulsões/tratamento farmacológico , Convulsões/etiologia , Convulsões/fisiopatologiaRESUMO
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
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Síndrome de Landau-Kleffner/fisiopatologia , Síndrome de Landau-Kleffner/complicações , Síndrome de Landau-Kleffner/tratamento farmacológicoRESUMO
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)