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Rev Neurol ; 26(154): 956-9, 1998 Jun.
Artigo em Espanhol | MEDLINE | ID: mdl-9658467

RESUMO

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/complicações
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