Clinico-electroencephalographic features of 96 cases of occipital lobe epilepsy whose initial sign was evidently elementary visual were investigated. Mean age at the onset was 8.2 years and the present age 20.9. More than one half of the patients had neu rologic and/or CT abnormalities and mental retardation. Besides the elementary visual seizure manifestations the ictal symptoms consisted of uni-or bilateral generalized tonicclonic convulsions, impaired consciousness, ocular movements, autonomic signs, automatisms, fallings, focal motor or sensory signs listed in decreasing order.
These complicated features were attributed to the spread of discharges from the occipital lobe to the other areas. The interictal EEG focus was localized in the occipital region in 25 cases. In 59 cases the EEG foci were found in the occipital as well as in other areas. In more than half of them temporal spikes were identified. 20 cases showed extraoccipital discharges which were contralateral to the occipital focus. In 12 cases no occipital focus was found. Typical patterns of focal interictal discharges were spike, sharp wave and polyspikes. It was illustrated that the ictal discharges spread anteriorly from the occipital focus to the ipsilateral or contralateral areas.
From an analysis of 63 cases whose clinical course was followed up for more than three years, factors relevant to unfavorable seizure outcome were: the presence of known etiology, neurologic or CT abnormalities, existence of epileptic foci in other than occipital regions and of focal polyspikes, and a certain seizures represented by falling, automatisms, focal motor sign and ocular movement.

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The clinical features and prognosis were studied in 6 patients with epilepsia partialis contunua (EPC) in terms of seizure manifestations and neuropsychiatric deficits. The diagnostic criteria of EPC were as follows; manifested by a form of epilepsy with somatomotor epileptic seizures characterized by a Jacksonian march during which intervals localized myoclonus persisted in the area of origin of the convulsions for more than one week.
Of the 6 patients, 4 males and 2 females, the ages of onset of EPC ranged from 2 to 19 years. Disappearance of EPC within one month as a result of intensive pharmacotherapy was obtained in 2 patients. Clinical manifestations of these patients were; the onset ages of EPC were 15 years (case 1) and 19 years (case 2), EPC disappeared during sleep, no combined seizures were noticed during the entire course of EPC, and no neuropsychiatric deficits ensued.
EPC persisted for more than 3 years in 4 cases out of the 6. The clinical features of the 4 cases were characterized as follows; the onset ages of EPC were 15 years (case 3), 12 years (case 4), 2 years (case 5) and 2 years (case 6), respectively. EPC subsided during sleep in case 3, but continued to repeat in other 3 cases, combined seizures which were occurring independently from EPC and obviously intractable were observed in case 5 and 6, hemiplegia was observed in case 3, 4 and 5, quadriplegia in case 6, sequential mental deficit was noted in 3 patients (case 4, 5 and 6). These neuropsychiatric impairments were non-progressive in case 3, 4 and 5, but unequivocally progressive in case 6.
The unfavorable neuropsychiatric outcome was found especially in cases with onset of EPC in early childhood and with intractable combined seizures. It is suggested that the immaturity of brain development in early childhood may play a pathogenetic and/or pathoplastic role in the progressive epileptic process of EPC.

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Thirty four patients with intractable temporal lobe epilepsy who had complex partial seizures more than once a month over one year despite anti-epileptic drug treatment were tried to be treated with monotherapy of phenytoi (PHT) and/or carbamazepine (CBZ). The doses of PHT and CBZ were increased until complex partial seizures were controlled longer than one month or toxic manifestations occurred. As the result, 13 patients were effective by PHT monothrapy and 3 patients were effective by CBZ mono-therapy. 12 patients were tried to be treated with both monotherapies; 3 were ineffec-tive by CBZ and effective by alternative PHT, and 9 were ineffective by both mono-therapies. The mean plasma level necessary for seizure control were 26.9μg/ml of PHT and 9.6μg/ml of CBZ. The effective cases of PHT monotherapy were more than those of CBZ monotherapy. The therapeutic plasma level of PHT for intractable complex partial seizures was above the so-called optimum plasma level (10-20μg/ml). The ineffective cases of PHT monotherapy had higher seizure frequencies and more complex partial seizures than grand mals as the initial seizure manifestations.

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To clarify the actual conditions of the refractory epilepsies in childhood, a comparative study was made between intractable cases and favourable cases in which seizures were suppressed for over three years at the time of follow-up. Refractoriness was defined as no change or aggravation in seizure frequency at the last visit comparing with that of initial visit.
The refractory and the favourable groups consisted of 41 cases (7.3%) and 375 cases (66.8%), respectively, among 561 cases followed-up for over three years after the initial visit from 1976 to 1979. With the type of epilepsy at the initial visit, the rate of intractable cases was high in Lennox-Gastaut syndrome (28.2%) and West syndrome (10.5%). All 5 cases of severe myoclonic epilepsy in infancy were classified into refractory group. On the other hand, the rate of the favourable cases was high in idiopathic generalized epilepsy (85.6%).
Compared with the favourable group, the refractory group comprised significantly more cases with seziure onset within six months of life, those with underlying pathologies, those with prenatal causative factors, those associated with mental retardation and/or motor disabilities. On the other hand, family history of convulsions was noted signnificantly frequently in the favourable group.
In the evaluation of therapy, it was noteworthy that considerable number of cases were revealed to be quasi-refractory cases.

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To settle the commonly available criteria of the refractory epilepsy, a comparative study was carried out on the two groups of intractable cases selected by different criteria, concerning the change in seizure frequency at the last visit comparing with that at the initial visit; Group I with no change or increase and Group II with persistence of seizures more than once a month at the last visit in spite of decrease in seizure frequency.
Intractable cases in both Groups I and II shared many characteristics such as clinical seizure patterns at the initial visit, classification of epilepsies, age of onset, associated mental and/or motor disabilities, mental prognosis, EEG findings, although symptomatic generalized epilepsies such as Lennox-Gastaut and West syndromes were more frequently observed in Group II than in Group I. Accordingly, the criteria of Group I will be usable for the collaborative studies on the refractory epilepsies.

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The clinical effects and plasma levels were investigated in a prospective randomized study when clonazepam (CZP) was administered as a single drug to children with absence seizures.
The patients comprised 20 previously untreated children with absence seizures aged 5 to 10 years, who were newly referred to our pediatric seizure clinic. Seventeen of the 20 patients suffered from absence seizures only, and the other 3 showed generalized tonic-clonic seizures combined with absence seizures.
A daily dose of 0.025mg/kg of CZP was given at first and then the dosage was doubled at weekly intervals until the initial maintenance daily dose of 0.1mg/kg was reached. However, in 3 patients who showed high plasma levels of CZP (50.7-65.7ng/ml), the dosage was decreased to tolerable levels because of continuous parental complaiants of hyperkinetic behavior problem. CZP was prescribed in two divided doses per day. The plasma levels selected for the study were determined 4 weeks after starting the maintenance dosage, and all blood samples were taken 2 to 3 hours after the morn-ing dose.
The maintenance daily dosage of CZP (0.10±0.01mg/kg) gave plasma levels of 44.8±6.3ng/ml.
Of the total of 20 patients, absence seizures were not controlled in one. Generalized tonic-clonic seizures occurred in one patient with no absence seizures, and in another they changed to partial seizures. However, for the period of treatment, ranging from 12 to 56months (mean, 27 months), complete seizure control was obtained in 17 out of the 20 patients on the maintenance dosage.
As a wide range of plasma levels (34.0-56.3ng/ml) was associated with complete freedom from seizures, and the plasma levels of patients who did not respond to CZP also fell to within the same range, it was not possibe to define a therapeutic range for CZP.
In 14 out of the 17 patients who responded well to CZP therapy, paroxysmal discharges seen on EEG had disappeared after 6 months of therapy.
CZP is a drug of choice for the treatment of absence seizures in children.

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