We investigated the development of the background activity of EEGs in 150 children with epilepsy by means of computerized power spectral analysis, using Fourier transformation by O₁ Twenty-seven cases with idiopathic generalized epilepsy (IGE), 30 cases with idiopathic partial epilepsy (IPE), 60 cases with symptomatic partial epilepsy (SPE), 33 cases with cryptogenic partial epilepsy (CPE) and 48 normal children were studied. The records with paroxysmal activity at 01 were excluded in this study. In normal children, total, delta and theta powers showed a significant inverse correlation with age, but alpha-2 and beta-2 powers showed a significant positive correlation with age. In children with epilepsy, there were significant increases of delta and theta powers and a decrease of alpha-2 power compared with normal children. The development of the EEGs was different among the patients with various types of the epileptic syndrome. It was suggested that age, prognosis and anticovulsants were important factors which influence the EEGs background activity.

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We conducted a retrospective study of 50 patients with West syndrome who had been treated with ACTH. The patients were classified into two groups ; group I, receiving standard dose of ACTH (0.025 mg/kg/day); and group II, receiving low dose of ACTH (0.015 mg/kg/day). The short-term effect of treatment was evaluated. Each group was further divided into two subgroups ; cryptogenic (I-C, II-C) and symptomatic (I-S, II-S).
There was no significant difference in the control rate of clinical seizures, between the two dosage groups of cryptogenic etiology. In the symptomatic cases, a greater effectiveness was achieved in group II-S than I-S. None of the patients with cryptogenic etiology experienced any relapse of infantile spasms. Patients with symptomatic etiology had a higher incidence of relapse. The rate of clinical and EEG seizure control was lower, and the incidence of side effects was higher in group I -S patients. The poor short-term effect of treatment in group I-S patients may be due to a higher incidence of prenatal etiology.

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Five cases with paroxysmal disturbances occurring during sleep were presented. The diagnosis of epilepsy was made in 3 cases and the diagnosis of non- epilepsy was made in 2 cases. The latter 2 cases had been treated as epilepsy. Clinical differences were not apparent on ictal manifestations between the epileptic and non-epileptic paroxysmal sleep disturbances. Seizures on awakening, which were noticed in only the epileptic cases, appeared 1 to 10 months later than sleep seizures. Final diagnosis was based on ictal EEG. Slow wave bursts were followed by a activity in non-epileptic cases. In epileptic cases, EEG revealed frontal dominant localized spike discharges which spread around in 2 cases and fast wave discharges in 1 cases.

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Auditory event-related potentials (ERPs) were recorded from 131 epileptic children of 4 to 20 years of age, and the related factors affecting P300 latencies were evaluated using multiple regression analysis. The examined factors were as follows:(1) age at P300 recording and duration of illness, (2) seizure types and epileptic syndromes, (3) anti-epileptic drugs (AEDs), (4) seizure prognosis, (5) organic brain dysfunctions, (6) x-ray CT and (7) EEG background activity and frequency of paroxysms. ERPs were elicited with the oddball paradigm. Five factors of them had significant correlations with P300 latencies. 1) P300 latencies were gradually shortened with age. 2) P300 latencies were prolonged in the patients before and during AED medication compared with those during the period of off- therapy. 3) Symptomatic partial epilepsy showed obviously prolonged P300 latencies. 4) P300 latencies were shortened with increasing alpha 2 power of EEG. 5) P300 latencies became prolonged with the length of illness.

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Muscle carnitine was measured in 3 severely handicapped children with acute myoglobinuria. Muscle total carnitine levels were 9.6±3.0 nmol/mg non collagen protein (m±SD, control 15.7±2.8, n=19), and free 6.5±3.0 nmol/mg non collagen protein (m±SD, control 12.9±3.7, n=19), muscle free carnitine was significantly low in them. Serum carnitine was measured in 22 severely handicapped children. Serum total carnitine was 48.9±12.3 nmol/ml (m±SD, control 60.3±11.7, n=14), and free 37.7±10.0 nmol/ml (m±SD, control 46.0±12.5, n=14), serum carnitine was also significantly decreased. Secondary muscle and serum carnitine depletion might be one of the potential cause of myoglobinuria in severely handicapped children.

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Children were studied on distribution of the severely mentally and physically disabled aged 6 to 15 years in Gunma Prefecture as of September 1, 1991. They were all bedridden or able to sit, but could not stand or walk, even with support. Today, all the Japanese should attend elementary school as well as junior high school. Even though they are severely disabled, they are all registered by the regional Board of Education, where we could get exact data on the disabled children throughout Gunma Prefecture. We collaborated with the regional Board of Education, the Child Consultation Center of Gunma Prefecture and 6 hospitals for the disabled.
A total of 129 cases were found. The population of schoolboys and schoolgirls aged 6 to 15 years of age was 236, 000, most of whose gurdians were residents of this prefecture, and the prevalence rate was 0.55/1000. Among 129 cases, 53 cases (41.1%) were institutionalized and 76 cases (58.9%) were cared for at home. Fifty-nine cases (45.7%) were males and 70 cases (54.3%) were females. Thirty-four cases were aged 6 to 7 years (26.4%) and older cases declined probably due to premature deaths. Ninety-four of the 129 cases (72.9%) were bedridden and mentally retarded with IQs 20 or less.

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Measurements such as intracranial pressure-volume index (PVI), out flow resistance (Ro), and absorption pressure (Abs) of the cerebrospinal fluid were utilized for intracranial bio-mechanical analyses of four infants who suffered from hydrocephalus prior to insertion of a ventriculoperitoneal (V-P) shunt and 4 infants suffering from malfunction of a previously inserted V-P shunt. Changes in parameters immediately before and 14 days after insertion of a V-P shunt and before and after revision of a malfunctioning shunt were compared. Further, to clarify the clinical significance, in infantile hydrocephalus, of changes in bio-mechanical parameters, measurement results also were compared with the outcome of V-P shunts by assessing changeg in pre-and post- operative reduction in size of the frontal horn of the lateral ventricles (frontal horn index ; FHI), and also by Watanabe's method of neurological evaluation.
Among 7 infants whose Ro and Abs prior to shunting were abnormally high when compared with normal values reported by other investigators, values such as FHI, Ro and Abs tended to return, after the V-P shunt insertion, toward normal values in association with marked improvement of neurological status. The results appear to indicate that high Ro and Abs values recorded prior to surgery are factors predicting good functional outcome of a hydrocephalic infant after V-P shunt insertion. It should be noted, however, that in 3 out of the 7 cases, both preoperative and postoperative PVI values were found to be higher than the predicted PVI values reported by Shapiro. We speculate that, in such cases, intracranial compliance may have under-gone an irreversible change due to a degenerative change in the brain substance secondary to progressing hydrocephalus. Based on literary investigation in regard to this matter, we gained the impression that there exists an indication for a V-P shunt in these cases, although further investigation is required.

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We reported a girl of Down syndrome with both moyamoya disease and spinal cord compression due to atlanto- axial instability. At the age of 3 years, she presented with muscle weakness of gradual onset, and could not walk without support. On admission, at the age of 6, she presented with spastic paraparesis with predominance on the right side. To explain her neurological manifestations, we performed cranial MRI first, and found multiple infarctions with predominance on the right hemisphere, the occlusive changes of internal carotid and middle cerebral arteries, and signal void flow signs in basal ganglia, which suggested moyamoya disease. Although diagnosis of moyamoya disease was confirmed by cranial angiography, discrepancy between clinical manifestations (right- side dominant spasticity) and MRI findings (right- side dominant infarctions) could not be explained. Thereafter, we performed cervical MRI, which showed severely compressed lesions with predominance on the right side, corresponding to her clinical manifestations. It is essential to perform not only cranial but also cervical MRI for a patient with Down syndrome presenting neurological complications.

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We report an 8-month-old infant with tonic spasms that started at the age of 38 days and occurred in clusters. He had no myoclonic seizures, or erratic myoclonus. Repeated simultaneous video-EEG monitoring revealed abrupt flexion of the neck and extremities associated with high voltage slow waves preceded by multiple spikes and/or sharps, which resembled spasms seen in West syndrome. Interictal EEGs, before and after the onset of epilepsy, showed suppression- burst. The bursts consisted of irregular high voltage slow waves mixed with poorly developed spike before the onset of epilepsy. On the other hand, duration of suppression phase ranged from 1 to 27 seconds, and burst-burst interval from 2 to 30 seconds after the onset of epilepsy. He was considered as having an atypical early infantile epileptic encephalopathy with suppression burst.

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We present the brain autopsy and MRI findings in a four-year-old boy who was born at 23 weeks of gestational age and 700 gram of birthweight and experienced an episode of cardiac arrest at 10 months of age. Neuropathological study showed the findings compatible with cardiac arrest syndrome, the gliosis or neuronal loss of extensive cerebral lesions including the cerebral white matter and cortex, basal ganglia, cerebellum and hippocampus, and large ulegyric formation in the bilateral parietal and occipital lobes, as well as the periventricular leukomalacia, which characterize hypoxic-ischemic insult in the brain of preterm infant. MRI performed eight months before his death demonstrated prominent atrophy and periventricular and parenchymal hyperintensity of the bilateral occipital lobes consistent with the above pathological findings.

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We reported an infant case of Guillain- Barré syndrome treated with high-dose gammaglobulin. Gammaglobulin was given at a dose of 1 g/kg/day for 2 cosecutive days. Dyspnea disappeared six hours after the start of the infusion. The patient was able to sit 3 days and walk alone 25 days after the end of therapy. Circulating immune complexes were 48μg/ml before the therapy, 95μg/ml and 39μg/ml 10 days and 37 days, respectively, after the therapy.

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A 14-month-old girl with spontaneous spinal epidural hematoma and complete postoperative recovery was described. MRI proved to be a sensitive tool in identifying the nature and extent of this lesion. A review of literature was made with particular emphasis on the pediatric age group.

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Two cases with secondary generalized epilepsy whose epileptic attacks disappeared abruptly were reported. Permanent improvement was observed in case 1 with residual state of West syndrome, aged 4 years, after viral pneumonia, both epileptic attacks and epileptic discharges on EEG disappeared more than 3 years. Transient improvement was observed in case 2 with Lennox- Gastaut syndrome, aged 6 years, after an episode with insomnia, poor appetite and confusion for three days. After this episode epileptic attacks disappeared for 9 months and epileptic discharges on EEG were also markedly decreased. However, epileptic attacks relapsed following the reduction of phenytoin after 9 months. An effect resembling ketogenic diet therapy and a change of pharmacokinetics of antiepileptic drugs may be involved in the transient improvement of case 2. However, any effects involving an immunological effect and secretions of adrenocorticohormone following after stress may be not involved in the permanent improvement of case 1.

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Two asthmatic children who developed status epilepticus during theophylline treatment followed by semicoma were reported. They suffered from severe neurological sequelae. A two- year- old male received oral maintenance theophylline therapy and a four-year-old male received intravenous theophylline therapy at the time of seizures. Theophylline blood levels measured several hours after the onset of seizures were within or below the therapeutic level 12.7 and 8.8μg/ml. They had febrile convulsions prior to the onset of episode and one of them was mildly delayed in psychomotor development. Brain CT scans showed diffuse cortical low- density in the acute period. Follow up CT scans revealed progressive cortical low- density in the subacute period and subsequently reached to the peak in the 10th day and 19th day of illness respectively. We consider that the progressive and long-lasting severe cortical edema on brain CT scan is characteristic of theophylline-associated encephalopathy.

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Female identical twins with atypical absence in waking state and partial seizures in sleep state were reported. During atypical absence the EEGs of both cases showed bursts in bilateral parietal and temporal regions during waking state and almost continuous diffuse spike-waves during sleep. Phenytoin was effective to discontinue the seizures in both cases. These findings were compatible to atypical benign partial epilepsy first reported by Aicardi et al. A possibility of genetic cause of this disorder may be considered.

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[in Japanese]

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[in Japanese]

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[in Japanese]

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