A total of 25 peculiar ictal automatisms were documented by simultaneous recording in 4 patients, ranging from 9 to 18 years of age, and 3 to 8 years of duration of epilepsy. The modes of the seizures were characterized by: abrupt onset and termination, brief duration lasting at longest for 30 sec., probable lack of impairment of awareness, remarkable tendency to repeating seizures with short intervals particularly during light sleep.
The seizure manifestations were characterized by: vocalization of screaming or yelling, laughter, vigorous and usually reciprocal movements of legs, rolling shaking of trunk and/or hip, tonic flexion of arms.
These characteristic automatisms were recognized as of frontal lobe origin based on the EEG expressions: interictally, focal spikes or sharp waves occasionally accompanied by slow waves in unilateral frontal region clearly recorded in all the 4 patients. Ictal EEGs revaled run of rapid spiking initiating from uni-frontal area in 3 cases and from bi-frontal area in 1 case.
A monosymptomatic course was evident in all cases, except for one who experienced a generalized convulsion as a result of drug incompliance. A tendency toward smedication refractoriness was also obvious.
All cases had been mistakenly regarded and treated as hysterical attacks.

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To investigate the long-term prognosis of childhood epilepsy, 99 patiens from our pediatric outpatient clinic who fullfill all of the three conditions described below were selected.(1) patients with epilepsy whose age of onset is under 10 years of age.(2) patientswho were followed over 10 years.(3) patients whose age is over 20 years.
63 cases are adapted almost normally in the society. 36 cases are adapted poorly in the society. In these two groups, seizure remain in 11% and 56% of the case srespectively. Social adaptation is well correlated with seizure control.
22 cases in the former group and one in the latter group are attending University 37 cases in the former group and 23 cases in the latter group are employed.
The correlating factors with social adaptation are age of onset of seizures, specific hereditary neurological diseases and brain damages due to external causes, developmental delay and neurological abnormalities before onset, epileptic types and frequency of seizures in recent 3 years (p<0.01). Correlating factors with seizure control are specific hereditary neurological diseases and brain damages due to external causes, developmental delay and neurological abnormalities before onset, epileptic types and frequency of seizures in onset (p<0.01).

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Long-term aministration of anticonvulsant is thought to affect calcium and vitamin D metabolism, and to cause rickets or osteomalacia. It was the purpose of this study to elucidate the factors which lead the bones to rachitic cahnges, and to evaluate the effects of 1α-hydroxy vitamin D₃ (1α-OH-D₃) on patients suffering from mild or moderate osteomalacia. The study was performed on 92 epileptic patients aged 3 to 22 years (mean 10.5) who had been receiving anticonvulsant drugs for 3 to 19 years (mean 5.8). The authers measured the serum concentrations of calcium (Ca), phosphorus (P), alkalinephosphatase (Al-p), 25-hydroxyvitamin D (25-OH-D), and evaluated ΣGS/D (index of bone density) by microdensitometry. 1α-OH-D₃ was given to the 16 patients of 92, whose serum concentrations of 25-OH-D or the values of ΣGS/D showed the lower levels than the normal ranges respectively.
Results: 1) In 21 of the 92 patients, serum calcium levels were lower than the lower limit of normal values obtained in age-matched controls. In 17 of 92, serum 25-OH-D levels were lower than the lower limit of normal values (13.8 ug/ml), and in 8 of 17, the values of ΣGS/D were lower than -1.65 S. D. from the mean value. 2) In 11 of 82 patients, the values of ΣGS/D were lower than -1.65 S. D. from the mean value, and all of 11 had been received multiple anticonvulsants concurrently for more than 7 years. In 8 of 11, serum 25-OH-D levels were lower than the lower limit of normal values. 3) Mean serum Ca levels of the 16 patients whose 25-OH-D levels of ΣGS/D values were lower than normal ranges, showed significant increse from 9.0 to 9.3mg/dl by administrating 1α-OH-D₃ for 3 months. The mean serum 25-OH-D level significantly increased from 15.8 to 26.7ng/ml by treatment for 6 months.Increasing rate of ΣS/D values of patients taking 1α-OH-D3 for 18 months was more than that of age-dependent controls.
It was concluded that long-term treatment with concurrent use of multiple drugs was the factor leading to rachitic changes, and that evaluation of ΣGS/D with serum Ca and 25-OH-D was useful for early detection of rachitic changes. Treatment with 1α-OH-D₃ improved the bone density in epileptic children receiving long-term anticonvulsant drugs.

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Eleven cases with focal epilepsy were treated surgically. All cases had epileptogenic foci. In four cases, focal epilepsy was caused by head injury and in seven cases, by cavernous angioma. These epileptogenic foci were recognized by conventional electroencephalography and confirmed by intraoperative corticography. In four cases with traumatic contusion which was epileptogenic, computerized tomography showed abnormal heterogenous (mixed by clear low and relative low) low density areas. Relative low density areas of them were almost the same portion as the areas of epileptogenic foci which were identified corticographically. In seven cases with cavernous angioma, computerized tomography demonstrated not only the lesion but the abnormal heterogenous low density areas in the cortex around the lesion.
On the operation, the epileptogenic foci were comfirmed by corticography and resected. Histogically the cortex of the focus was consisted of the atrophic ganglion cells, gliosis, infiltration of macrophages and proliferation of the capillaries.

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Zonisamide (3-sulfamoylmethyl-1, 2-benzisoxazole, AD 810, CI-912) is a new anticonvulsant compound, of which properties were demonstrated in several animal models and some adults with medically refractory partial seizures. We studied its effects 39 children with intractable epilepsy. They were between 2 and 18 years of age (mean 10.0 years old) and in most of them (87.8%) their seizures repeated weekly or daily in spite of the maximal therapeutic plasma level of two or three standard antiepileptic drugs. In regard to the seizure type, 28 cases had partial epilepsy (TLE; temporal lobe epilepsy in 9 and NTLE; non-temporal lobe eilepsy in 19) and 11 cases were in secndary generalized epilepsy (LGS; Lennox-Gastaut syndrome in 9 and SGE without LGS in 2). Zonisamide was administered 2-10 mg/kg/day in addition to the previous drug for one to ten months.
Ninetten of 39 cases (48.7%), 15 with partial epilepsy and 4 with generalized epilepsy showed an improvement. Nine of them (45%) improved only transiently, suggesting the development of tolerance to the drug. The average serum concentration of patients in whom the agent was effective was 16.5μg/ml, while those who did not respond had an average concentration was 16.7μg/ml. The lower limit of effective concentration was thought to be lower than in adults. Adverse effects such as drowsiness, ataxia, anorexia and liver dysfunction were common to antiepileptic drugs. On the contrary, such side effects as decreased volition and memory seemed to be related to Zonisamide. All side effects were transient or reversible and not severe.

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We reported the clinical characteristics of 3 patients with peculiar reflex epilepsy in which specific seizures were evoked exclusively by motivating non-linguistic higher cerebral function.
The first case, a woman of 47, had recurrent myoclonic jerks involving the upper extremities as well as generalized tonic-clonic seizures (GTC) since age 14. Through intensive monitoring with the use of CCTV-EEG, it was determined that myoclonic jerks or spike-wave-complexes (sp-w-c) were evoked only when she was subjected to certain neuropsychological tasks comprised of a combination of complex processes of thinking (decision-making) and voluntary motor activities specifically involving the fingers and arm such as calculation, game playing, writing, construction of designs.
The second case, a woman of 29, had experienced since age 11 myoclonic jerks compound or combined with GTCs evoked only when, for example, calculating, writing or playing piano. Her condition was well controlled by valproate and clonazepam. However, when she was placed at calculation tasks, burst of generalized high voltage slow waves took place.
The third case, a man of 25, had recurrent myoclonic jerks and GTCs since age 15. They were characterized by being precipitated by calculating, constructing designs, writing or playing games. Sp-w-c were also activated by posing tasks of calculation or construction of blocks.
By reviewing a total of 22 cases of reflex epilepsy evoked mainly by non-linguistic higher cerebral activities reported hereto in the literature, we were able to identify a unique group of reflex epilepsy of idiopathic nature, of which the clinical characteristics are summarized as follows: 1) seizure onset at around puberty, 2) seizure-inducing factors comprised of higher cerebral activities requiring a combination of complex thinking and voluntary movement, becoming more and more definitive as the clincal course progresses, 3) clinical seizures of generalized nature, especially of myoclonic jerks mainly involving the upper extremities and combined or compound GTCs, 4) generalized epileptic discharges predominating in the central area activated by posing particular neuropsychological tasks to the patient. There is some resemblance between this type of reflex epilepsy we have postulated and juvenile myoclonic epilepsy.
There exist two different types of reflex epilepsy in which clinical seizures are evoked by higher cerebral activities: language-induced epilepsy on the one hand and epilepsy induced by non-linguistic activities on the other. The significance of the latter within the realm of reflex epilepsy was emphasized.

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Schizencephaly is a congenitl anomaly with a cleft in the cerebral mantle. Developmental errors or the impairment of the cerebral circulation in the early embryogenic stage are suspected to cause this malformation.
We report here cases of 6 patients with partial epileptic seizures, diagnosed as schizencephaly on the bases of CT findings. These patients had their first seizure at the age of 2-23 years (mean of 12 years) and received their first neurological examination when 5-42 years old (mean 24.8 years). CT scanning revealed a unilateral cleft in 4 cases (a cleft in the right side in 2 cases and in the left in 2) and bilateral symmetrical clefts in 2 cases. These clefts were limited in the pre-central or centrl areas and were not accompanied by other overt malformations. All the patiens had focal neurological symptoms and seizures that started with a simple partial seizure followed by complex partial seizures or generalized convulsions. EEG examinations showed a focal epileptic discharge in 5 cases, a unilateral focus in 2 cases and bilateral independent foci in 3 cases.
The localization of the clefts corresponded to the focal symptoms, seizure manifestations and EEG findings in all cases. Thus the cleft of schizencephaly is potentially an epileptogenic focus that induces partial epileptic seizures.

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Several features of amygdaloid kingling support that it is an animal model of complex partial seizures; first the behavioral pattern of kindled seizure resemble to those of the human complex partial seizure, and secondarily EEG abnormalities are similar to those recorded from electrodes in the amygdala and the hippocampus during the seizures. The aim of this paper is to study the neural mechanism of the epileptic automatism in kindled cats, by analizing correlation of behavioral automatism and EEG afterdischarges in the amygdala and other cerebral structures.
Daily electrical stimulation of the basolateral amygdaloid nucleus induced the development of seizures, generally evolving through the following stages: 1) unilateral facial twitching, 2) repetitive masticatory movement, 3) head-turning and circling to the contralateral side of stimulation, and, 4) generalized clonic or tonic-clonic convulsion. There was approximate correlation between development of behavioral automatism, and EEG afterdischarges of amygdala and of other cerebral structures. At the stage of the repetitive masticatory movement, the high amplitude rhythmic burst discharges of about 6 Hz appeared in the amygdala, which propagated to the basal forebrain (nucleus accumbens). At the next stage of the contraversive head-turning and circling, high voltage repetitive spike discharges developed prominently in the neostriatum, whereas amygdaloid discharges rather decreased in amplitude. During the progression from the head-turning to the generalized convulsion stage, there was a still more remarkable increase of afterdischarges in amplitude in all the structures, particularly in the neostriatum and the cerebral cortex.
These results suggest that the basal forebrain and the neostriatum are involved in producing the perioral automatism and the head-body turning, respectively, which are observed in the human complex partial seizures, and that the neostriatum might play an important role for producing the secondary generalized convulsion, too.

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Development of hippocampal kindling was compared with that of the amygdaloid kindling in suckling rats. In amygdaloid kindling rats (n=12), an hourly electrical stimulus (400μA, 1 ms, biphasic square pulses, 62.5 Hz for 2 sec), twenty times a day, was applied to the left amygdala at 16 days of age. In hippocampus, the other 20 rats received the fourty kindling stimuli (fifteen times a day at 16 and 17 days of age and ten times a day at 18 days of age) to the left dorsal (n=8) or the ventral (n=12) hippocampus. If afterdischarge (AD) was not evoked by an 400μA current, stimulus intensity was increased to 800μA. If it was still ineffective, the animals were discarded from the data. The results were as follows.
1) In the hippocampal kindling, sixteen of 20 rats (80%) developed a generalized convulsion with the mean number of 23.3 stimulations. However, the other rats (20%) remained a focal seizure within the 40 stimulations. In the amygdaloid kindling, all rats (n=20) developed a generalized convulsion within 20 stimulations (the mean number; 13.2).
2) The mean duration of AD changed from 43. 3 sec for the initial AD to 84.7 sec for the first generalized convulsion (two fold increase) in the hippocampal kindling. On the other hand, the change of AD duration was 4.3 fold (12.4 sec to 53. 6 sec) in the amygdaloid kindling.
3) A marked difference was observed in the behavioral seizures of wet dog shakes (WDS) between the hippocampal and the amygdaloid kindlings.
4) There was no apparent difference in the pattern of kindling progression between the dorsal hippocampal and the ventral hippocampal kindlings.
A discussion was made on the hippocampal and the amygdaloid kindlings to understand the basic mechanisms underlying the critical susceptibility of immature brain to seizures.

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A cannula communicating with the rat ventriculus was chronicaly implanted, and anticonvulsants were administered through the cannula. The effect of anticonvulsants applied through the cannula on decreased electroconvulsive threshould by pretreatments with a-methyl-p-tyrosine (α-MT), p-chlorophenylalanine (PCPA) or allylglycine was inves-tigated. DN-1417, sodium valproate (VPA) and phenobarbital (PB) antagonized the α-MT induced lowering of electroconvulsive threshold (E. C. T.). The reduced E. C. T. caused by PCPA was antagonized by DN-1417 but not by VPA and PB. Allylglycine induced lowering of E.C.T. was antagonized by VPA and PB but not by DN -1417. Phenytoin had no anticonvulsive effect on rats pretreated with these three drugs.
The contents of dopamine, serotonin and their matabolites in the striatum and nucleus accumbens were determined by high-performance liquid chromatography combined with electrochemical detector. PCPA reduced the DOPAC content of striatum, and then the administration of DN-1417 into rats ventriculus lateralis caused an in crease inDOPAC content.
These experiments suggested that the anticonvulsant action of DN-1417 might be due to enhance the dopamine turnover.

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