The proper classification of non-convulsive status epilepticus is a very important problem in the field of epileptology, but it has not been fully studied as yet. The purpose of this paper is to present a newly recognized, peculiar type of non-convulsive status epilepticus in childhood.
The clinical manifestation consisted of frequent brief atonic episodes without loss of consciousness. These episodes were only a few seconds' duration and appeared as frequently as several hundreds times a day. Such a condition of numerous episodes lasting for about one or two months recurred at intervals of several months. During the cluster of episodes, decreased dexterity, mental deterioration and hyperkinetic behavior were observed in all cases, and the motor disturbance of the left arm also appeared in one case.
Regarding the epileptic seizures, nocturnal focal motor seizures were observed in five among seven cases. The remaining two cases had generalized tonic clonic seizures.
During the cluster of episodes, repetitive focal spike-and-waves with marked tendency of generalization were noted in waking EEG records. As clinical episodes did not always occur in accordance with spike-and-wave discharge, each episode could not be considered one epileptic attack. However, clinical episodes increased with the increase of spike-andwave discharges in EEG. Another finding was the disappearance of clinical episodes with the decrease of spike-and-wave discharges. During the cluster of episodes, sleep EEGs showed a characteristic pattern with continuous diffuse spike-and-waves which resembled those of subclinical electrical status epilepticus induced by sleep (ESES). During the period of remission, focal spikes were the main feature and diffuse spike-and-waves were not seen continuously even during sleep. The spike foci were multiple; midtemporal central, anterior temporal, parietal, frontal and occipital.
These findings indicate the condition observed in our cases as a new type of nonconvultive status epilepticus, which is closely related to ESES.

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Long-term prognosis was studied in 103 cases with generalized tonic and/or clonic convulsions in the first year of life (boys 49, grils 54). All cases were aged 6 years or more at the time of the final follow-up. According to their mental and physical status, the subjects were divided into two groups; mentally and physically normal (N), mentally and/or physically handicapped (H). Each group was further divided into two subgroups A (38) and B (19) in group N (57), C (8) and D (38) in group H (46). In cases of A and C, seizures ceased before 3 years of age and seizures persisted at or over 3 years of age in B and D.
Sixty-five cases (63%) were free from seizures for more than three years. 55% showed normal development and 37% normal mental and physical development and complete remission of seizures. The latter can be called benign infantile convulsions.
Following five factors were found to be associated with good prognosis for mental and physical development: 1) cryptogenic, 2) normal development before the onset, 3) noneurological abnormality, 4) no evolution into other types of seizures, and 5) normal EEG course. If a patient has three factors, above-mentioned 1)-3), in the early stage of infantile convulsions, good prognosis will be expected. However, no significant difference in prognostic factors was found between subgroup A and B.

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In order to study whether associations exist between HLA antigens and epilepsy, HLA-A, B, and C antigens were determined in 257 patients with epilepsy.
HLA antigens of the A, B and C locus were evaluated in 110 controls with no history of epilepsy or febrile convulsion. Epileptic patients were classified following the International Classification of ILAE: partial seizures (N=144)[partial seizures with elementary symptomatology (N=32), partial seizures with complex symptomatology (N=14), partial seizures secondarily generalized (N=98)], primary generalized epilepsy (N=56)[absence seizures (N=27), generalized tonic clonic, clonic, or tonic seizures (N=29)], secondary generalized epilepsy (N=27), hemiconvulsion (N=13) and unclassified seizures (N=17). Six HLA-A, 17 HLA-B, and 4 HLA-C antigens were defined using the standard NIHTerasaki microlymphocytotoxity test. The most of typing sera were qualified for their specificities in the Japanese and the International Histocompatibility Workshop. Relative risks and difference in HLA antigens between patients and controls were calculated by chi-square analysis and P values were corrected by multiplying P by the number of antigens tested (Pc).
Among number of antigens tested, the phenotype frequency of Bw 60 was increased in patients with several seizure types and in patients with epilepsy as a whole. Bw 60 was found in 35.4% of all the epileptic patients (X²=20.1, Pc<0.001), in 38.8% of the patients with partial seizures secondarily generalized (X²=20.35, Pc<O.001), in 40.7% of the patients with absence seizures (X²=12.55, Pc<0.02), in 40.7% of the patients with secondary generalized epilepsy (X²=12.55, Pc<0.002), compared with 11.8% of the control and showed relative risks of 4.1, 4.7, 5.1 and 5.1 respectively.
This investigation showed that HLA Bw 60 may be a marker of genetic characteristic which seems to have some precipitating effects on the manifestation of epileptic seizures.

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In order to elucidate the role of psychic tension in the precipitation of epileptic seizures, a precise analysis of provoked paroxysmal activities during EEG activation by means of speaking, reading, writing, calculation, and constructive acts was made. This examination, which we call “neuropsychological EEG activation” (NPA), was carried out in a total of 455 patients with epilepsy. In 25 patients out of them, paroxysmal activities, most often associated with myoclonic seizures, were apparently induced by NPA. The factors which were related to the provocation of paroxysmal activities and habitual sei-zures in these 25 patients were:(1) each performance of NPA (16 patients);(2) psychic tension alone (3 paticnts);(3) each performance of NPA as well as psychic tension (6 patients).
A total of 9 patients who belonged to the above mentioned group (2) and (3) were main subjects of this study. In 3 patients of group (2), paroxysmal activities which appeared during NPA were considered to be induced by psychic tension when they were listening to an explanation for tasks and/or when these tasks seemed to puzzle them. Based on such clinical and EEG findings, a proposal for naming these patients as “reflex epilepsy induced by psychic tension” was made. In the other 6 patients of group (3), it was considered that in addition to each task of NPA (e.g. writing, etc.), psychic tension as mentioned above also could play a significant role in the provocation of paroxysmal activities.
The provoked paroxysmal activities were focal (1 patient) and generalized (8 patients): in the former, rhythmic slow activity with spike over the RFp-aT areas could be induced by psychic tension alone; the latter was of simultaneous occurrence over all the areas.
These results suggest that the role of psychic tension cannot be disregarded in the provocation of epileptic seizures precipitated during mental activity.

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A 40 years old man with tonic clonic seizures, ataxia and involuntary movements was investigated clinically. Computed tomography showed diffuse brain atrophy and CSF examination revealed a low content of GABA. The latter is supported by the evidence of a low activity of GAD in the brain of 3 autopsied cases suffered from the similar disease, which was investigated by Dr. I. Kanazawa. Abnormal brain stem function was revealed by REM stage in sleep EEG and auditory evoked potential.
This case was diagnosed as a peculiar degenerative disease-dentatorubropallidoluysian atrophy (DRPLA). It is emphasized that the case suffered from this disease would be found clinically by co-existence of epileptic seizures and ataxia or choreic movements. Familial outbreak of the patient with such symptoms, even if it was recessive or dominant, would have the confidence to diagnose the case as this peculiar disease.

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This report described a case of SSPE, who repeatedly manifested minor epileptic status (MES); a special form of non-convulsive status epilepticus.
Her psychomotor development had been normal until four years five months, when she developed frequent brief episodes of falling down and mental deterioration. On her admission at four years and eleven months, she was diagnosed as SSPE because of the periodic bursts in the EEG and a high titer of measles antibodies and a high IgG level in CSF.
She abruptly manifested confusional state twice during the course of the illness; at four years and seven months, and four years and eleven months, respectively. The first episode lasted for two weeks and the second, for two days. These were regarded as MES because the EEG during the confusional state, revealed irregular high voltage slow waves accompanied by multifocal spikes.
Various kinds of epileptic attacks can occur during the course of SSPE and epileptic discharges such as diffuse slow spike-and-wave burst are often observed at some stages. Her EEG on admission also revealed diffuse slow spike-and-wave bursts frequently but she did not have epileptic attacks. MES in this case was considered to be manifested based on the epileptic mechanism which was caused by SSPE.
In general, MES occurs in the cases of secondary generalized epilepsy, especially in that of the Lennox syndrome. Therefore, the epileptic mechanism in this case may be similar to the pathophysiology in the Lennox syndrome.
Although it is known that MES can occur in the cases with underlying diseases other than epilepsy, this is the first reported case who manifested MES during the course of SSPE. Sufficient examinations of underlying diseases including SSPE should be done at the time of the diagnosis of MES.

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This report concerns two six-year-old right-handed boys who had acqired expressive speech disturbance associated with paroxysmal EEG abnormalities. The EEG showed frequent spike and wave discharges maximally in the right mid-temporal region with transmission to the other area of the right hemisphere during wakefulness and subclinical bioelectrical status during sleep.
They were succesfully treated with ACTH-Z. But the EEG abnormalities and speech disturbance recurred about one month later, which again responded to ACTH-Z therapy. In these patients the degree of speech disturbance paralleled the EEG abnormalities. Persistent focal EEG abnormalities might be a cause of speech disturbance. But not only cortical but also subcortical dysfunction might be involved in this condition in the light of diffuse continuous sharp and slow wave discharges during sleep. At the present time the cause of the syndrome remains unknown.

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A specific binding activity for phenytoin (PHT) was determined by a modified receptor assay with 3H-PHT using the ammonium sulfate precipitation and the glass filter filtration method. Approximately 70% of the specific binding activity was observed to exist in particulate fractions of the rat brain. Approximately 40% of the binding activity in the particulates was solubilized by freezing-thawing, ultrasonication and detergent CHAPS (3-[(3-cholamidopropyl) dimethylamino]-1-propanesulfonate) treatments. The solubilized binding sites were partially purified by Phenyl-Sepharose gel chromatography and n-butanol treatment, and it was found that a specific binding activity/protein of the purified fraction increased up to about ten times as much as the activity with non-purified solubilized fraction.
The PHT, related hydantoins and barbiturates were compared in terms of their affinity for the PHT binding sites. It was found that PHT and MPPH (5-(p-methylphenyl)-5-phenylhydantoin) indicated the highest affinity among the drugs tested. The affinity of HPPH (5-(p-hydroxyphenyl)-5-phenylhydantoin), whose hydrophilic hydroxyl group is substituted for the methyl group of MPPH, was markedly decreased as compared to that of MPPH. Almost no affinity was shown in the case of ethotoin, pentobarbital and phenobarbital. It is proposed that the PHT binding sites prepared in this study recognize quite specifically the particular chemical structure of the drug.

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From the viewpoint that posttraumatic epilepsy might be caused by iron liberated from hemoglobin we conducted a histopathological study in which ferrous chloride was used as the inducing agent on forty Wistar rats at 8 to 9 weeks of age weighing from 150 to 200 grams.
1) Histological changes in the chronic epileptic foci of the cortex were studied and compared at each stage with changes observed during continuous electroencephalography.
2) Microscopical findings of the focal lesions were consistent with the continuous EEG changes on seizure discharge observed at each stage. Iron stored at the injection sites was beginning to be ingested by the macrophages when the first marked seizure discharge on the EEG developed. At the stage of maximal discharge, the macrophages had invaded deeper and were also detected around the white matter similar to the stage of focus completion.
3) Electron microscopical findings indicated that the nuclei of the nerve cells had become transparent due to chromatolysis and swelling of the protoplasm and the mitochondria was also detected. Many paired cisternae, a formation seen in cells and germ cells undergoing marked mitosis, were detected in the protoplasm of the nerve cells and they increased during the completion stage.
4) Ferrous chloride was detected in the macrophages in the white matter and in the secondary lysosomes of the neurons and the astrocytes. Many secondary lysosomes and fibrous structures were detected in the endfeet of swollen astrocytes, which also tended to increase toward the focus completion stage.
5) It may be presumed that the induced iron, remaining as a residual body, might be related to the causative mechanism in developing an epileptic discharge.

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Recent investigations provided us information of the existence of transfer and interference phenomena in the limblic system, especially amygdaloid kindled rats.
The present study, however, designed as an investigation in order to know whether these phenomenon in the neocortical kindling are similar in the limbic cortex or not.
Biopolar stimulating and recording electrodes were implanted, bilaterally, into the anterior frontal cortex, and methods of experiments for the development of kindling were adopted and employed as similar as possible to those for amygdaloid kindling.
As results, positive transfer was observed in one group, and negative transfer, or inhibitory event at the contralateral homologenous cortex, was observed in the other group. When retest of primary site kindling was done, it was further discovered that post-transfer interference was observed in the former group, and was not observed in the latter group.
It seemed to be suggested that frontal cortical kindled epileptogenesis might involve the development of activity in inhibitory system, whereas in excitatory system, too.
Therefore, the authors have concluded that neocortical kindling will differ from limbic kindling in their transfer.

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