Since the introduction of the International Classification of Epilepsies and Epileptic Syndromes in 1989, a number of new syndromes have been proposed, some of which need further clarification and confirmation. Some of the syndromes listed in the 1989 Classification have been further delineated, while other should be considered for reclassification. The most exciting recent advance has been a discovery of mutant genes in some of idiopathic epilepsies, which have been found to be grouped in the category of channelopathies.

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We describe four patients with dentatorubral-pallidoluysian atrophy (DRPLA), who showed marked deterioration involving dementia, choreoathetosis and cerebellar ataxia, corresponding to the appearance of epileptic seizures. The patient in case 1 with the juvenile type, showed clustering of focal and generalized clonic seizures over a one-month period. During this episode, her clinical condition deteriorated, and slowing of background EEG activity and exaggeration of paroxysms was also observed. In the other three cases, who showed the adult type, the clinical conditions deteriorated soon after the clustering of clonic seizures or the appearance of tonicclonic convulsions, making the patients unable to walk, sit, eat or communicate. It is suggested that epileptic seizures may herald acute deterioration of the clinical symptoms and reflect the acute progress of DRPLA pathophysiology.

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This is a case of mentally retarded 26 year-old woman who has an intractable epilepsy with the onset in childhood. The epilepsy was classified as an undetermined epilepsy. From the age of 20 years, she was administered zonisamide increasing up to 400mg per day for about two years in addition to valproic acid and clonazepam. The serum concentration of zonisamide was 36.7μg/ml, but the seizures were not controlled sufficiently. Moreover, because the patient complained of sleepiness and loss of appetite after she was administered full dose of zonisamide, the patient was stopped to be administered zonisamide only by the decision of her mother.
At the age of 26 years, she was administered acetazolamide with the gradual increase of the dose combined with phenobarbital and phenytoin. She was admitted to the hospital because she showed frequent seizures with no specific reasons. On the first day of admission, acetazolamide was stopped and zonisamide was introduced again with the intial daily dose of 160mg. The seizures were not controlled with the intravenous administration of diazepam and phenytoin, but the seizures disappeared almost completely with the intravenous administration of lidocaine. After a week of readministration of zonisamide, generalized choreoathetotic involuntary movements were recognized continuously during the wakening stage. At this moment, the serum concentrations of phenobarbital, phenytoin and zonisamide were 28.5μg/ml, 8.9μg/ml and 5.2μg/ml, respectively. The choreoathetotic movements were considered as a side effect of zonisamide and the movements disappeared four days after discontinuing the medical agent.

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