In evaluating severe myoclonic epilepsy in infancy (SME), high voltage slow wavegrand mal syndrome (HVSW-GM), and the peripheral group of the former two syndromes, it seems important to take the viewpoint that the grand mal seizure is the common and principal clinical symptom of all three clinical entities.
To investigate general characteristics including pathophysiological factors of these clinical entities, the following clinical criteria common to both SME and HVSW-GM were established: grand mal with onset before the age of one year as the principal seizure type; difficulty in determining the type of epilepsy whether partial or generalized; some degree of mental and motor dysfunction appearing after the seizure onset; difficult detection of epileptic discharges in EEG in the initial stage; and, therapeutic resistance at the commencement of therapy.
Of twenty-two patients meeting all the clinical criteria, twelve patients had been diagnosed as having SME, six as having HVSW-GM, and four as having symptoms peripheral to these syndromes (P).
The percentage of cases with status epilepticus for the SME group was markedly high and that of the obtunded state showed a similar tendency, but neither HVSWGM nor P showed such tendencies. Adding that, each percentae of cases with severe mental dysfunction, abnormal brain CT findings, and poor prognosis for the three groups seemed to increase in the order of HVSW-GM, P and SME.
According to these results, it seems reasonable to regard cases with P as intermediate between cases with SME and HVSW-GM. It also seems that grand mal in combination with other seizure types complicates pathophysiological conditions, and, especially in combination with myoclonic seizures, increases the severity of epilepsy. It is suggested that the three clinical entities, SME, HVSW-GM and P, share certain characteristics and may in fact be more productively conceptualized as representing portions of a continuum.
In conclusion, it is suggested that there moy be a common pathophysiological basis for all three clinical entities and therefore, they may belong to the same clinical entity, “infantile refractory grand mal syndrome”.

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Eighty-seven mentaly retarded patients were studied over a period of three years. The incidence of bone atrophy in these patients and the therapeutic effects of active vitamin D₃ (VD₃) upon it were investigated using microdensitometry (MD) and multiple X-ray photodensitometry (MD/MS) methods.
Sixty-two of the 87 patients had been treated with antiepileptic drugs before starting the study, and medication was continued during the study. The first MD test was carreid out in 1986 and abnormal findings were found in 30 patients (20 of these had been taking antiepileptic drugs, 10 had not). After the first test, VD₃ was given to the 30 patients for the three year period but it was not given to the remaining 57 patients (42 of these received antiepileptic drugs and 15 did not). The incidence of abnormal findings gradually decreased in the 30 patients, while it increased in the 57 patients who were not given VD₃ during the three year period. An improvement in bone atrophy was mainly due to an increase in cortical bone mineral density, and, moreover, this increase was pronounced in the patients who had been given antiepileptics. The therapeutic effects of VD₃ did not decrease during the period, and no side effects were detected in the patients who were given it.

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Various evoked potentials and sensory nerve conduction velocity were recorded in patients with long term antiepileptic drugs. Preliminary investigation indicated that there was no significant difference in auditory brainstem evoked potentials (ABP) and pattern reversal visual evoked potentials (P-VEP) between 39 patients and 20 normal controls. N₁₈ and N₃₄ of short latency somatosensory evoked potentials (SSEP), sensory nerve conduction velocity (SCV) and early and late components of blink reflex (BR) in 66 patients revealed significant delay as compared to 20 normal controls.
Furthermore there was significant positive correlation between serum phenobarbital and late components of BR and N₁₃, N₁₈, and N₃₄-N₁₈ of SSEP. N₁₃-N₉ of SSEP was also positively correlated with serum phenytoin level. N egative correlation was found between PB and SCV. The above findings indicated that AEDs would affect a function of impuls conducting system in widespread areas of central as well as peripheral nervous system.

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We investigated the effects of sodium valproate (VPA) and phenytoin (PHT) on cognitive functioning utilizing event related potentials (ERPs), particularly N1 and P3 components.
For each VPA and PHT group, twelve normal volunteers were subjected to this study. Each subject participated in two experimental sessions and was administered either real drug (VPA: 800mg/day, PHT: 200mg/day) or placebo for 1 week in a double-blind design before each session. For the ERPs measurement, we used the oddball task and to investigate the effect of taks difficulty, 3 types of stimulus conditions were employed. For the psychological test we used Wechsler Memory Scale-Revised (WMS-R) on each session.
No significant effect of VPA was found on performance, correct reaction time, amplitude and latency of N1·EP3 and indexes of WMS-R. PHT of high serum level (mean serum level: 6.1μg/ml, 5 subjects) significantly reduced N1 amplitude in a specific type of stimulus condition.
It may be concluded that 1) no significant effect of VPA on cognitive functioning was found in the present study, 2) PHT has negative effects on attentional functioning.

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Local cerebral glucose utilization (LCGU) during the postictal phase of amygdaloid kindled seizures was studied with the quantitative autoradiographic [¹⁴C] deoxyglucose (DG) method in unanesthetized rats. In the partially kindled (PK, class 1-2 seizure) rats, DG was injected 30s after termination of a convulsive seizure. In the fully kindled (FK, class 5 seizure) rats, DG injection was made either just after (FK-II) or 30s after (FK-I) seizure termination. LCGU of the neocrotex deceased in the kindled animals as compared to the control and the extent of the decrement was most prominent in FK-II. In the hippocampus, on the contrary, LCGU increased in FK-II and rather decreased in PK and FK-I as compared to the control. Increased glucose utilization of the hippocampus, which had been already shown before and during the kindled seizure, was also observed in the early phase of the postictal period. The results suggest that the hippocampus is one of the key structures in not only initiating and maintaining, but also terminating kindled seizures.

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Methionine enkephalin-like immunoreactivity (ME-LI) in the brain of El mice (seizure-susceptible strain) was measured by radioimmunoassay (RIA) to elucidate the relation between seizures and the opioid system. The concentration of ME-LI in 25-day-old El mice that had no seizures was significantly decreased in the hippocampus. At the age of 50 days when El mice displayed abortive seizures, the levels of ME-LI in both El (+) and nonstimulated El (o) mice were also significantly reduced in the hippocampus and septal area. It was further shown that the ME-LI concentration in both 150-day-old adult El (+) during interictal periods and El (o) mice were markedly decreased in the cerebral cotex, septal area, and striatum, as compared with the corresponding regions in ddY mice (seizure-nonsusceptible strain: the mother strain of El). The decrease of ME-LI in the El mouse brain was generally compatible with our previous findings concerning the up-regulation of opioid delta receptors in this species. These results suggest that the reduction of ME-LI in the El mouse brain is not due to convulsions, but could be associated with the pathogenesis of seizure diathesis and seizure manifestations in the El mouse.

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A clinico-electrographic correlative study was carried out to explore whether ictal involvement of bilateral temporal lobes is indispensable for complex partial seizure (CPS). The subjects consisted of 123 CPSs of 24 patients documented by chronic intracranial EEG recordings which were performed as a presurgical evaluation of intractable temporal lobe epilepsy.
In the majority of CPSs (89 CPSs, 72%), selfsustained rhythmic discharge involved bilateral temporal lobes; however, in the remaining 34 CPSs (28%), it remained unilateral to the originating side throughout the CPSs. Although bitemporal ictal involvement was not necessary for impairment of consciousness, both mesial (amygdala and hippocampus) and lateral (cortex) temporal lobe structures were consistently involved.
In comparison with the CPSs with bilateralized discharges, those with unilateral ones were characterized as follows: 1) responsiveness to external stimuli was partially retained, 2) postictal recovery of consciousness was more prompt and 3) motionless staring characterized by total unresponsiveness was never observed.
Based on these findings, the mechanisms underlying impairment of consciousness of CPS was discussed in association with a functional linkage between temporal lobe and brain stem.

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The subjects were 25 children with synchronous spike or spike and wave in the occipital and frontopolar areas (O, Fp spike). The average age at the time of investigation was 10 years 5 months.
We recognized 19 cases of epilepsy, 4 cases of febrile convulsions and 2 cases without seizures. Regarding the seizure type, 18 of 19 epileptic patients evolved to secondarily generalized seizures. Three patients had visual aura. Brain CT showed abnormal findings in 3 patients. Of all the patients, 28.0% indicated a developmental or intelligence quotient under 80. The O, Fp spike developed between 8 and 12 years in 68.0%. In 16 patients, the O, Fp spike was found bilaterally at the occipital area, and unilaterally at the frontopolar area. Three-year remission rate was 43.8% in 16 patients followed for the past 3 years or more.
We could not determine a primary focus of O, Fp spike, but presume that O, Fp spike spread through the long association fiber (occipitofrontal lasciculus) running longitudinally in the ipsilateral hemisphere.

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In contrast to “spike-wave stupor”, complex partial status epilepticus (CPSE) has rarely been explored from electroencephalographic and clinical stand-point of view.
Generally speaking, CPSE is devided into two groups of continuous type and discontinuous type.
Little attention has been paid to the relationships between these two conditions.
We report a 32-year-old female patient with CPSE, in which she showed clinical symptoms of both discontinuous and continuous types.
At the age of 15 years, she started to have a generalized convulsion which occurred once a year thereafter. Since 24 year old, complex partial seizures with tonic staring or fugue has begun several times per month. It followed macropsia, metamorphopsia, macroacousia, verbal auditory hallucination, musical hallucination, visual hallucination, anxiety and fear etc.
At the time of initial clinical evalution, fugue and automatism followed suddenly verbally auditory hallucination in a medical examination.
Ictal EEG during these episode showed discontinuous left dominant δ, θ activity. On the other hand, she showed continuous and discontinuous clinical symptoms during CPSE.

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