The clonazepam (CZP)-induced tonic seizure was reported in a girl with psychomotor seizures (PM). She had been uneventful until the age of 1 year when febrile tonic-clonic seizures began. At 5 years, she had the first attack of unconsciousness with behavioral automatism. During loss of consciousness, the EEG showed high voltage and irregular 4-7 Hz slow bursts in the centro-parieto-occipital region. From 12 years, in addition to phenobarbital, sodium valproate, carbamazepin and phenytoin, CZP was administered daily. Ai 15 years, she experienced tonic seizures; with myoclonia of eyelids, eyes-rolling from side to side, hypertonia of the limbs, increased salivary secretion and irregular respiration in the drowsy state. The seizures, lasting 1to 3 minutes, ceased suddenly with a sigh. The ictal EEG revealed a diffuse fast activity of 10-20Hz and 100-200μv, ending without electrical suppression. CZP was gradually discontinued and the tonic seizures disappeared within 1 week.
Four serial polysomnographical (PSG) recordings were performed. During the period under the CZP therapy, both tonic (% stage REM and % slow wave sleep) and phasic (gross movements, twitch movements and REMs) events of sleep components were severely suppressed. At 1 week and 2 months after withdrawal of CZP, both parameters returned to normal except the abnormality of gross movements and twitch movements.
Our previous PSG studies indicated that the prognosis of epileptic seizures in PM and age-dependent epileptic encephalopathy related to the neural mechanism regulating sleep parameters. It was suggested that the neural mechanism controlling sleep parameters are etiologically important in CZP-induced tonic seizures.

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Although valproic acid (VPA) is efficient in treating epileptic disorders of childhood, severe a dverse effects of VPA have been reported, such as fatal hepatic dysfunction, hyperammonemia, and a Reye-like s y ndrome. Recently the suppression of fatty acid oxidation by VPA was reported in studies of rat liver homogenates and human biopsy specimen. We carried out the ¹³C-palmitate breath test on patients with VPA-t reatment and found low ¹³CO₂ recoveries in the expired air of them.
Methods: Before administration of ¹³C-palmitate, expired air was collected for basal ¹³CO₂ determination.[1-¹³]-palmitate (Kor Incorp, 10 mg/kg) was given orally to the patients who had been fasting for 15-1 6 hours. After administration, breath collection was performed at one hour intervals for the 8 hour period. ¹³CO₂ in the expired air was analyzed by a mass spe ctrometer (Nuclide RMS) equipped with a dualinlet. The ¹³C-abundance in the sample CO₂ was expressed as a permil (%) increase from the base ¹³CO₂/¹²CO₂ o r as cumulative ¹³CO₂% dose/7 hrs. This test was performed on 8 patients treated with VPA and other a n t i c o nvulsants (VPA-treated group) and 4 patients with other anticonvulsants (no VPA-treated group). T h e y were all hospitalized (8 males and 4 females, aged from 9 to 17 yrs), and suffered from epilepsy with o r without other problems such as mental retardation and cerebral palsy. They had no evidence of liver d y s f u ction when e x a m i n e d. Results: ¹³CO₂ production in no VPA-treated group revealed a maximum 2 to 3 hours after administration and then declined. VPA-treated group revealed that ¹³CO₂ production stayed at maximum for 2 to 3 hours and then showed a slow decline. The cumulative ¹³CO₂ recovery of no VPA-treated group was 14.4±2.3%dose/7 hours, whereas that of VPA-treated group, 6.4±4.1%(P<0.01). We interpret these results to suggest that VPA interferes with the fatty acid metabolism in vivo and 13C-fatty acid breath test may be useful to detect its adverse effects

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Clinicopathological examinations on 15 cases of the subependymal germinolysis (SEG) revealed that there were no specific clinical signs in any cases, but pathologically congenital anomalies in 10 cases including congenital heart diseases and 3 with cytomegalic inclusion body diseases. In the central nervous system there were migration disorders in 6 cases such as neuronal heterotopia, polymicrogyria and cerebellar dysplasia. Immunohistochemical stains of neuron specific enolase showed positive cells in the wall of the SEG in a case. Therefore, it was concluded that the SEG occured at the early fetal period up to 34 gestational weeks because the migration of neuroblasts to the olivary nucleus was observed before the 3rd month of gestation. When SEG is diagnosed by cranial echography or CT, it is necessary to consider an association of anomalies as well as intrauterine complications of various viruses, chemical substances, radiation or hypoxia as causal agents.

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In patients with infantile spasms, ACTH therapy was carried out according to Fukuyama's method which is the generally accepted method in Japan. The long term prognosis in these patients was then examined. The patients whose course could be observed for 5-24 years (11 years on the average) after the therapy were divided into the cryptogenic (C) group and the symptomatic (S) group. On the basis of the initial therapy, they were further divided into C₁, S₁, C₂ and S₂. In the former two groups ACTH-Z was consecutively administered at a daily dose of 0.25mg (10 units) to 0.5 mg (20 units) for more than 14 days and in the latter two groups the drug was given in the same way as the former groups at smaller daily doses for less than 14 days, or other anticonvulsant drugs were given. C₁, C₂, S₁ and S₂ consisted of 13, 9, 4 and 12 patients, respectively. At present 8% of C₁, 11% of C₂, 50% of S1 and 58% of S₂ have seizures, and 69% of C₁, 44% of C₂, none of S1 and 8% of S₂ are in or have graduated from normal school. The prognosis of seizures and educational abilities of C₁ patients were favorable and approximately the same as those following longterm therapy by massive administration of ACTH in western countries. The difference in prognosis between the patients in the Ci group and those following lower doses and shorter duration of ACTH therapy (a part of C₂ patients) should be studied further.

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Event-related potentials (ERPs; P₃₀₀) were recorded from 22 neurologically normal children of 4 to 15 years of age. Stimulation was performed by an “odd-ball” paradigm. A run consisted of sequences of 400 tone bursts in which 85% were 2, 000Hz (nontarget tone) and 15% were 1, 000Hz (target tone). The subjects were instructed to listen to the rare tones (“Listen”), to count the number of rare tones (“Count”) and to press a key when a rare tone was detected (“Key-Press”). Auditory P₃₀₀ recordings were made at Cz and averaged separately for rare and frequent tones. In this study, differences of the wave form between target and nontarget ERP and age-related changes of P₃₀₀ latency and amplitude were analyzed. A prominent P₃₀₀ component for rare tone was clearly identified by visual inspection in the majority of children except two of 6 years of age. The relationship between age and P300 latency was highly linear with a negative slope (r=-0.72, P<0.01). Latency of P₃₀₀ decreased with aging and reached adult values after the age of ten years. There was no statistical correlation between the age and the P₃₀₀ amplitude. In the younger group (4-9 years), an obvious P300 peak was elicited by the task “Count”(70%) or “Key-Press”(75%), and less often by the task “Listen”(42.9%). P₃₀₀ is useful measure for the evaluation of the cognitive function in children over the age of 4 as well as in adults.

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Eight patients with age-dependent epileptic encephalopathy (ADEE) three patients with West synfirst records, sleep-stage-dependent distributions of BMs were markedly disturbed and the numbers of BMs obtained two to five times for each patient when their fits and hyperkinesias were aggrevated. Rapid eye movements (REMs) during the stage REM (SREM) and body movements during sleep (BMs)[gross movements (GMs) and twitch movements (TMs)] were studied. GMs were the movements including the trunk lasting two seconds or more and TMs were those localized in one muscle lasting 0.5 seconds or less.
In most records, the length of SREM was preserved, while that of the slow wave sleep (SWS) decreased. The rhythms of SREM and SWS with sleep progression were disturbed in all records. REMs showeddecrease in number more in LS than in WS, but showed no characteristic change with increasing age. In their first records, sleep-stage-dependent distributions of BMs were markedly disturbed and the numbers of BMs were suppressed. The former tended to be normalized and the latter increased in the records thereafter. The increase of the numbers of BMs was prominent in TMs during SREM around the ages of two in WS and nine in LS.
BMs in normal subjects are known to decrease with age. It is thought that BMs reflect brain catecholaminergic (CA) activities, and that, during the process of the kindling model, the CA system gains supersensitivities, which lead to psychotic symptoms or behavioral hypersensitivities. Our results suggest that CA systems are primarily affected in ADEE and this interference results in abnormal reactions of CA systems in later with the occurrence of intractable fits and hyperkinesias in ADEE.

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It is really important to evaluate the sustained attention of children with epilepsy or febrile convulsions, because their behavioral problems might be connected with an attention deficit and the influence of anticonvulsants on the attention has not been fully elucidated. A test for sustained attention, a new modified procedure called the Continuous Performance Test (CPT), was developed.
The subjects consisted of 34 children with afebrile or febrile seizures taking no drugs, 28 taking some anticonvulsants and 30 healthy control children. All subjects were confirmed to be of normal intelligence by Binet tests.
The two epileptic groups showed inferior CPT scores compared with the controls, and there was no difference in the CPT scores between the epileptic groups. The CPT scores of children with febrile seizures were not better than those of children with afebrile seizures. There was no difference either in CPT scores between the epileptic children on phenobarbital and those on other anticonvulsants.
The results of this study suggest that the majority of epileptic children suffer from a subtle attention deficit irrespective of whether they have febrile or afebrile seizures, and irrespective of whether anticonvulsants including phenobarbital are given or not.

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A patient with late infantile type of neuronalce roid-lipofuscinosis was followed by CT and electrophysiological examinations for 7 years. CT showed that brain atrophy started in the cerebellum and the brain stem and progressed to the cerebral cortex later. In EEG, diffuse and high voltage slow waves seen at the first stage were decreased in the voltage, and multiple focal spikes appeared. They disappeared at the terminal stage. Giant evoked potentials in the visual evoked response and the somatosensory evoked potential were present initially, but they were decreased later and finally there was no response. The origin of myoclonus frequently observed at the terminal stage was studied by myoclonus related cortical potentials. The first appearance of myoclonus in the sternocleidomastoid muscle suggested that the myoclonus might have originated from a lesion near the accesory nucleus in the brain stem.

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Alternating hemiplegia in infants (AHI) is characterized by the onset before 18 months of age, repeated attacks of hemiplegia involving both sides of the body, ocular motor abnormalities, autonomic disturbances and mental or neurological abnormalities.
We reported a case of 19-month-old Japanese boy with typical AHI. The boy showed characteristic features of AHI at 9 months and received phenobarbital, valproic acid and carbamazepine, which were ineffective. His hemiplegic attacks exhibited many phenomena, such as tonic spells, dystonic posturing, ocular motor abnormalities and autonomic disturbances. After treatment with clonazepam (1mg/day), the attacks dis-appeared completely for three months and recurred less frequently.
A perusal of the relevant literature on AHI gives interesting information. A family history of migraine was present in 42% of the reported cases. Hemiplegic attacksdis appeared during sleep and reappeared after waking up. A majority of the older cases exhibited extrapyramidal signs.

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There are some reports of familial neonatal hydrocephalus. It is well known that X-linked hydrocephalus is one of the genetic forms of congenital hydrocephalus. A small number of cases have other genetic forms and are not well known about their clinical features and pathogenesis.
In this report, we described two cases of congenital hydrocephalus involving both sexes. Case 1 was a female baby who was born by cesarean section after 40 weeks of normal gestation to a primiparous mother. Birth weight was 4, 300g and head measured 43cm. Hydrocephalus was diagnosed by CT scan, which revealed a marked enlargement of the lateral ventricles of both side and the third ventricle. Fourth ventricle was of normal size. Chromosomal study was performed on the second day after birth which revealed no abnormality. Her development has been within normal limits.
Case 2 was a male baby who was the younger brother of Case 1. He was born after 38 weeks' gestation also by cesarean section. Birth weight was 3, 300g and head circumference was 39 cm. CT scan revealed the same findings as in Case 1 which confirmed the diagnosis of hydrocephalus. Chromosomal study was normal. Ventriculo-peritoneal shunt was done and his development was excellent. He was one of dizygous twins, and the other baby was normal. Survey of the family revealed no family history of hydrocephalus except for Case 1.
Until now, only 6 families have been reported with familial uncomplicated congenital hydrocephalus including female patients. We discussed the clinical feature and pathogenesis of this genetic form of hydrocephalus.

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