Assuntos
Encefalopatias/congênito , Encefalopatias/patologia , Epilepsia Parcial Complexa/diagnóstico , Atrofia/patologia , Encefalopatias/complicações , Eletroencefalografia , Epilepsia Parcial Complexa/etiologia , Epilepsia Parcial Complexa/fisiopatologia , Lateralidade Funcional/fisiologia , Humanos , Lactente , MasculinoRESUMO
No disponible
Assuntos
Humanos , Masculino , Lactente , Encefalopatias/complicações , Transtornos Psicomotores/etiologia , Microcefalia/diagnóstico , Tomografia Computadorizada por Raios XAssuntos
Doenças Cerebelares , Neoplasias Cerebelares , Doenças Cerebelares/diagnóstico , Doenças Cerebelares/etiologia , Doenças Cerebelares/patologia , Doenças Cerebelares/fisiopatologia , Neoplasias Cerebelares/complicações , Neoplasias Cerebelares/diagnóstico , Neoplasias Cerebelares/patologia , Neoplasias Cerebelares/fisiopatologia , Pré-Escolar , Feminino , Humanos , Imageamento por Ressonância MagnéticaRESUMO
No disponible
Assuntos
Feminino , Pré-Escolar , Humanos , Encefalite/complicações , Neoplasias Cerebelares/complicações , Linfangioma/patologiaRESUMO
INTRODUCTION: The childhood opsoclonus-myoclonus or Kinsbourne syndrome, is a uncommon process, of acute or subacute beginning, which affects infant and children. It's course is characterized by opsoclonus, polimyoclonias and cerebellar ataxia. The disease is frequently associated to neuroblastoma (46%). MATERIAL AND METHODS: We present a retrospective study on 9 patients, emphasizing the clinical presentation and the evolution aspects. RESULTS AND CONCLUSIONS: We found changes in the EEG in three cases. Most surprising is the scarce incidence of neuroblastoma, which has been found only in a one out of nine patients. We found three cases with relapse during the treatment or on withdrawal and one of them relapsed twice again. The evolution has been variable, since 5/9 patient have presented some type of mild or moderate neuro-psychological sequelae. Out of three patient with relapses, two presented permanent neurological sequelae. A patient which suffered three relapses, is also the one which presents more serious sequelae.
Assuntos
Ataxia Cerebelar/epidemiologia , Mioclonia/epidemiologia , Transtornos da Motilidade Ocular/epidemiologia , Neoplasias Abdominais/complicações , Neoplasias Abdominais/epidemiologia , Adolescente , Hormônio Adrenocorticotrópico/uso terapêutico , Dano Encefálico Crônico/etiologia , Ataxia Cerebelar/tratamento farmacológico , Ataxia Cerebelar/etiologia , Criança , Pré-Escolar , Eletroencefalografia , Feminino , Seguimentos , Ganglioneuroblastoma/complicações , Ganglioneuroblastoma/epidemiologia , Humanos , Masculino , Mioclonia/tratamento farmacológico , Mioclonia/etiologia , Transtornos da Motilidade Ocular/tratamento farmacológico , Transtornos da Motilidade Ocular/etiologia , Recidiva , Estudos Retrospectivos , Síndrome , Viroses/complicaçõesRESUMO
INTRODUCTION AND MATERIAL: Between 369 cases of benign partial epilepsy of the childhood with Rolandic spikes (BECRS) diagnosed in our hospital, we have been able to study three patients that have presented a neurophysiological and clinical presentation that was compatible with the form described by Aicardi and Chevrie in 1982. RESULTS: None of our patients had familial epilepsy precedents. There were no pathological birth or previous neurological disorders. The neurophysiological development was normal until the beginning of the clinical picture. The seizures began when the patients were 3 years and 4 months, and 5 years, with partial seizures of the same characteristic that present patients with BECRS. Two of them had generalized tonic-clonic seizures during sleep. All the patients presented throughout the evolution absences myoclonic and/or atonic seizures. The awake EEG patterns showed normal background activity and paroxysms of focal spike were complexes with diffusion to central areas. During the slow wave sleep, every cases showed paroxysms of diffuse and generalized slow spike and wave complexes practically continuous. The partial seizures were scarce, but the absences and the atonicas seizures presented several times every day, and in one case appeared in the form of a grand mal state. CONCLUSIONS: The evolution of the patients was favourable, as they were free of seizures, with a normal neurophysiological development. At the beginning, the seizures were resistant to treatment with several antiepileptics drugs (AED). The treatment with valproic acid (VPA) and clonazepam (CZP), has been very effective.