[in Japanese]

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510 children with grand mal (first diagnosed at the age less than 15 years), who were followed up for over 10 years were studied.
By their prognosis, the subjects were divided into three groups: group A (favorable outcome): 276 cases (54%), who were off-medcation without relapse for at least one year.
group B (moderate outcome): 159 cases (31%), who had been seizure-free for more than 5 years but dependent on medication or reducing the dosage of medication.
group C (poor outcome): 75 cases (15%), who were seizure-free for less than 5 years.
The remission rate was 85% for the five-years period. The relapse rate was 9% after withdrawal of drugs.
Factors for favorable outcome (group A) were as follows:
1) The onset of seizure at less than 6 years of age.
2) Duration of seizures less than 5 years.
3) Seizure control by the age of 10 years.
4) Normal mentality.
5) Seizure attack only with fever.
6) Disappearance of seizure discharge (focal or multi focal spike, diffuse spike and wave, their complications, etc.) in EEG by the age of 15 years.
On the contrary, factors of poor outcome (group C) were as follows:
1) The onset of seizures at more than 10 years of age.
2) Duration of seizure more than 10 years.
3) Mental retardation.
4) Afebrile seizure.
5) Later disappearance of seizure discharge in EEG.
In the relapse cases, there was a tendency to earlier reduction of medication in spite of no disappearance of seizure discharge in EEG, which were compared with complete remssion cases.

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A 14 years old girl whose chief complaints were epileptic seizures and dysarthria was reported. In infancy, retardation of verbal development and clumsiness of oral movements were noticed. By speech therapy her vocabularies increased and her speech became more fluently than before, however dysarthria was retained. At age of 13 years epileptic seizures began, then abnormal findings of brain CT and EEG were found. By neurological examination during admission at our hospital, it was clarified that her dysarthria was caused by bucco-facial apraxia.
The CT finding which showed abnormal infolding of cortical gray matter along the bilateral symmetrical clefts was considered to be one type of prenatal dysgenesisschizencephaly.
The EEG showed repetetive bilateral independent or dependent centro-midtemporal spikes during sleep. The epileptic seizures which occurred at frequency of several times per month were regarded as complex partial seizures with salivation at onset.
From the findings of brain CT, EEG and clinical symptoms, the bilateral centromidtemporal cortical regions were considered to be responsible for the etiological localized lesion.
And from the viewpoint of apasia-epilepsy syndrome, it was discussed that the developmental dysphasia of our case was differentiated from auired aphasia syndrome of Landau-Kleffner.

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A case of 28 year-old-woman withpartial statusepilepticus occurringfrom two different foci was reported. She has suffered from systemic lupus erythematosus for 6 years and had one generalized tonic clonic convulsion with prolonged comatose state at the age of 23, and the same complex partial status epilepticus at 23, 24 and 25.
She had two types of seizures in the reported status epilepticus; complex partial seizures with oral, verbal and gestural automatism, and visual seizures with eyes deviations. They occurred independently, but sometimes two seizures combined with each other; a complex partial seizure was followed by a visual seizure and vice versa. The state of consciousness in the interval between seizures, and during visual seizures was fairly normal.
Electroencephalogram showed epileptic discharges localized in the left occipitoposterio-temporal areas during visual seizures and those predominantly in the left anterior temporal areas during complex partial seizures. These findings indicate that visual seizures may start from occipital lobe focus, and complex partial seizures from temporal lobe focus, independently.
This is a rare type of partial status epilepticus, associated with systemic lupus erythematosus, and induced by the reduction of steroid medication.

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Atypical absence status occurring in a patient with Fukuyama type congenital muscular dystrophy (FCMD) was detected by EEG and recorded on film video; the condition was completely controled by sodium valproate (VPA) syrup given rectally.
The patient was an 8-year-old girl diagnosed as having FCMD and the onset of her epileptic seizures was at age eleven months. Her epileptic seizures which were refractory to administration of many antiepileptic drugs, occurred about once in one or two months, and occassional status epilepticus was also observed. On this occasion we could observe the status epilepticus and regarded it as atypical absence status accompanied by facial myoclonia. Febrile or afebrile covulsions are reportedly present in more than half of the cases of FCMD. This is the first report in which a case of FCMD was accompanied by non-convulsive generalized seizures in the form of status epilepticus.
VPA aministered rectally was successful in the treatment of the status epleipticus, and complete seizure control was obtained without severe side effects. Although the rectal administration of VPA in the treatment of status epilepticus has been employed in only a few cases, the method seemed to be effective. In addition, we previously confirmed that there is no essential difference in pharmacokinetics between rectal and oral administration of VPA by the use of computerized simulation based on plasma concentration data of VPA after rectal administration to two volunteers.

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The diurnal oscillation of carbamazepine (CBZ) and the relationships between serum levels (Cp) of CBZ and the appearance of side effects were investigated in nine patients receiving chronic therapy with CBZ. Furthermore, in some cases, the Cp of CBZ-10, 11-epoxide (Epo) were also investigated. Cp of CBZ were measured 7-10 points throughout a day and analyzed pharmacokinetically by using the nonlinear least square regression program.
It was revealed that the mean diurnal oscillation of CBZ was 64±26% and the time courses of the CBZ Cp were simulated very well by one compartment open model. The minimal Cp were found in an early morning doses, while the maximum were observed between three and four hours later the last doses. The values in early morning doses were good reproducible in steady state conditions. However, in four patients, the absorption of CBZ after morning doses was delayed about one hour compared with other patients.
In all the cases, the mean absorption and elimination rate constants was 0.80±0.35 hr^-1 and 0.07±0.03 hr^-1, the values of clearance ranged from 0.051 to 0.255 liter/hr/kg and Vd/F ranged from 0.75 to 2.67 liter/kg.
The rate of the Cp of Epo to CBZ showed the interindividual variation from 10.5 to 46.9%.
In four patients, the side effects (diplopia, drowsiness, ataxia, dizziness, dysarthria, headache, blurred vision) were found especially for two or three hours after lunch and evening doses. Side effects mentioned above were invariably appeared at levels≥8.0μg/ml of CBZ.
These data underline the limitation of relying on a single drug determination of CBZ and emphasize the necessity of pharmacokinetic applications for the optimal dosing schedule of CBZ.

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Somatosatin (SRIF) has been proposed to be involved in the epileptogenic properties in rats, and DN-1417 (γ-butyrolactone-γ-carbonyl-histidyl-prolinamide citrate) and TRH-T (tartarate) have been reported to have an anticonvulsant action. We have investigated the effects of the drugs administered intraperitoneally (0.2 and 2.0mg/kg) on immunoreactive SRIF (IR-SRIF) cotents in the rat brain. IR-SRIF concentrations were found to be significantly decreased after chronic treatment with DN-1417 (10 days) in the striatum, amygdala, sensorimotor cortex, hippocampus and entorhinal and pyriform cortices. Acute treatment with DN-1417 failed to show any change in brain IR-SRIF. No change were seen after acute or chronic treatment with TRH-T. Changes in brain IR-SRIF after chronic DN-1417-treatment were more prominent at a dose of 0.2mg/kg as compared to 2.0mg/kg.
These results, together with the previous findings that somatostatin have an epileptogenic effect on animal models, suggest a possible role of somatostatin in the mechanisms underlying anticonvulsant action of DN-1417.

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The mechanism of the anticonvulsant effect of benzoidiazepine derivatives (BDZs) on amygdaloid-kindled convulsions of cat was investigated in the present study. Flunitrazepam, nitrazepam, clonazepam showed marked blocking of convulsive seizures and significant shortening of after discharge (AD) at both primary and secondary epleiptogenic sites. Diazepam blocked convulsive seizures and shortened AD at the secondary epileptogenic site, but only showed shortening of AD duration in the midbrain reticular formation (MRF). On kindled convulsions following splitbrain procedures, diazepam displayed a marked anticonvulsant effect which was similar to those of the three other BDZs. These findings suggest that BDZs have eminent anticonvulsant effects and act pharmacologically by blocking AD propagation in MRF.

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