["Benign" form of myoclonic epilepsy in children].
Among 62 children with myoclonic epilepsy who had first seizures between 1 and 10 years, without clinical or radiological evidence of brain lesion, we selected the 16 patients who had exhibited several types of fits and had stopped having seizures for over two years. First seizures occurred between 18 months and 4 years, and they were generalized clonic, tonic-clonic or tonic. After a mean 3 months' period, patients started also to have absence and myoclonic fits. During the period with various types of seizures, that lasted a mean 10 months, patients were ataxic and hyperkinetic, and 11 of them suffered myoclonic absence status for several hours or days. The EEG showed a high voltage rhythmic slow-wave activity with spikes, differing from the slow spike wave tracing of the Lennox-Gastaut syndrome, and there was no photosensitivity. The mean duration of the epilepsy was 1 year and 4 months and the last seizures were convulsive, occurring mainly during sleep. The clinical and EEG pattern, the high familial incidence are shared by the Doose syndrome, of which the present series seems to be a subgroup, as are other well-defined syndromes: benign and severe myoclonic epilepsies of infancy.
Dulac
O
O
Service de neuropédiatrie, INSERM U 29, Hôpital Saint-Vincent-de-Paul, Paris, France.
Plouin
P
P
Chiron
C
C
D002675:Child, Preschool; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies
Forme "bénigne" d'épilepsie myoclonique chez l'enfant.