A clinical and electroencephalographic study of myoclonic epilepsies in infancy and early childhood. Part 2. Idiopathic myoclonic epilepsy with benign prognosis.:Part 2. Idiopathic Myoclonic Epilepsy with Benign Prognosis

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We studied clinical and EEG features of 60 patients with idiopathic ME in infancy and early childhood with benign prognosis. They showed normal development before onset and a high incidence of either febrile convulsions or epilepsy in the family history (36%). The mean age of onset of myoclonic seizures (MS) was 30±13 months. In addition to MS, the patients often exhibited generalized tonic clonic seizurees (70%), myoclonic astatic or astatic seizures (64%), atypical absence (53%), minor epileptic status (20%) and nocturnal generalized seizures (NGS)(63%). Early in the evolution, frequency of the seizures increased and they became resistant to medical therapy. However, the long-term seizure prognosis ranged from fair to good. All patients entered remission within several years. The final intellectual outcome ranged from normal to mildly retarded. The patients could be placed into 3 subgroups according to the frequency of NGS: goup A 1 (without NGS), A 2 (rare NGS), A 3 (frequent NGS). Although much better prognosis in the seizure and intellectual outcome correlated inversely with the frequency of NGS, they form a continum. These findings lead us to conclude that idiopathic ME in infancy and early childhood is an epileptic syndrome with a relatively wide spectrum concerning the prognosis of the patient's seizures and intellectual development.
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