MIGRATING PARTIAL SEIZURES IN INFANCY - A MALIGNANT DISORDER WITH DEVELOPMENTAL ARREST

被引:186
作者
COPPOLA, G
PLOUIN, P
CHIRON, C
ROBAIN, O
DULAC, O
机构
[1] UNIV PARIS 05,DEPT NEUROPEDIAT,PARIS,FRANCE
[2] HOP ST VINCENT DE PAUL,DEPT NEUROPHYSIOL,F-75674 PARIS,FRANCE
[3] HOP ST VINCENT DE PAUL,INSERM,U29,F-75674 PARIS,FRANCE
关键词
CONTINUOUS MULTIFOCAL SEIZURES; ANTIEPILEPTIC DRUG INTRACTABILITY; PSYCHOMOTOR DEVELOPMENTAL ARREST; EPILEPTIC SYNDROME; INFANTILE EPILEPSY;
D O I
10.1111/j.1528-1157.1995.tb00961.x
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Fourteen infants of both sexes had a previously unreported epileptic condition characterized by nearly continuous multifocal seizures. The first seizures occurred at a mean age of 3 months, without antecedent risk factors. At 1 to 10 months, the seizures became very frequent. They were partial with variable clinical expression, and the EEG showed that the discharges randomly involved multiple independent sites, moving from one cortical area to another in consecutive seizures. Although their topography varied, the EEG ictal pattern of each seizure was very similar. It consisted of rhythmic alpha or theta activity which spread to involve an increasing area of the cortical surface. Patients regressed developmentally and became quadriplegic with severe axial hypotonia. Three patients died at age 7 months and at age 7 and 8 years, respectively. Seizures were controlled in only 2 patients, and only 3 children resumed psychomotor development. Extensive investigation failed to determine an etiology, and there was no familial recurrence. Neuropathological examination of the brain in two cases showed only severe hippocampal neuronal loss and accompanying gliosis.
引用
收藏
页码:1017 / 1024
页数:8
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