This study was undertaken to investigate the effects of early undernutrition on brain develop-ment using autoradiographic technique.
Newborn mice were divided into the under-nourished group and the control group. The dams, provided with an unresticted ration and water, nursed the litter until weaning. Thereafter the weanlings from both groups were provided with an unlimited balanced ration until the 60th day.
At 20th day, the undernourished revealed a 50 % reduction in body weight and a 18% in brain weight compared with the control. Despite of nutritional rehabilitation until 60 days after birth, the undernourished was not completely recovered in both body and brain weights.
Entire size of the cerebellum of the undernourished was smaller than that of the control and the external granular layer (EGL) was disappea-red later. Labeling index counted at the external matrix layer in the EGL of 5- and 10-day-old mice did not show the significant difference between the undernourished and the control.
However after 15 days of age, the labeling in-dex of the undernourished showed significantly higher value than that of the control. Generation time obtained on the external matrix cell at the EGL of 10-day-old mouse was about 2.5 hours longer in the undernourished. Hence it may be concluded that the undernutrition during early postnatal life may cause reduction of the granule cell number in the cerebellum subsequent to the prolongation in the generation time of the exter-nal matrix cell in the EGL.

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The activity of α-L-iduronidase was determined in leucocytes, and cultured skin fibroblasts from the homozygotes and heterozygotes of the Hurler syndrome or the Hunter syndrome.
It was found that the determination of α-L-iduronidase was a useful method for the differen-tial diagnosis of the Hurler syndrome from the Hunter syndrome and for the diagnosis of hete-rozygotes of the Hurler syndrome which had been difficult so far. Normal cultured amniotic fluid cells revealed a lower but significant activity of α-L-iduronidase. This suggests a rapid and useful procedure of prenatal diagnosis of the Hurler syndrome.

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The average maximal blood flow (A/L) and late cardiac diastolic blood flow (d) of right and left internal carotid arteries were measured on the patients with moya-moya disease by the Doppler Ultra-sound method percutaneously. All patients has been diagnosed as moya-moya disease previously by the cerebral angiography. They were two males and three females and their ages were between 6 years 10 months and 9 years 6 months, averaging 8 years a month. The blood flows were measured at the time from 6 months to 4 years 6 months after the diagnosis by cerebral angiography, and all patients did not show any significant neurological abnormality. The blood flow of thirteen epileptic patients with grandmal episodes were measured as control and their average age was 8 years 3 months. Comparing with the control, low values of both A/L and d were shown in the moya-moya patients. But d value was more useful for the diagnosis of the moya-moya disease than A/L value. The control children showed higher values of A/L than that of healthy adults.

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In order to investigate the in vivo effects of anticonvulsants on the immune system, the pro-portion of T and B-cells in the circulating lym-phocytes and serm immunoglobulin levels were determined in 36 severely handicapped children on anticonvulsants for more than 6 years, and were compared with those of 28 patients who were not treated by anticonvulsants.
Percentages and the number of T-cells were significantly reduced in the treated group as compared with the control group. The number of B-cells was not changed. The low lymphocyte counts observed in the treated group were con-tributed by the reduction of T-cells. There was no significant correlation between the percentage of T-cells and the duration of anticonvulsant therapy.
There was no difference in the number of gra-nulocytes and serum immunoglobulin levels bet-ween the two groups.

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The maturational changes of sleep cycle characteristics and EEG patterns were studied during different sleep states in preterm infants of various conceptional ages. The following criteria were used to differentiate sleep states.
Active sleep: rapid eye movements, irregular respiration and presence of various patterns of body movements.
Quiet sleep: absence of eye and body movements and regular respiration. Sleep states which could not be classified as either active or quiet sleep were regarded as indeterminate sleep. One minute smoothing time was used for the determination of sleep state. After 36 weeks of conceptional age, sleep cycle was better defined.
The amount of quiet sleep increased with the conceptional age. Indeterminate sleep decreased with maturation. The percentage of time the infants spent in active sleep decreased as the infants approach term. The duration of quiet sleep was approximately 20 minutes after 36 weeks of conceptional age.
Quiet sleep was divided mathematically into 3 equal parts. The distribution of EEG patterns in each part of quiet sleep was calculated at different conceptional ages. Before 36 weeks, EEG consisted of tracé alternant and a pattern of continous low voltage activity in all three parts of quiet sleep. After 36 weeks, high voltage and slow wave pattern. began to occur in the first third of quiet sleep and increased with conceptional age.
EEG consisted mainly of tracé alternant in the 2nd and 3rd part of quiet sleep. Active sleep also was not a constant stage. There were more slow waves with higher amplitude in the EEG after 36 weeks than before 36 weeks. After 36 weeks conceptional age, the transitional stage from active to quiet sleep continued about 2 minutes and the EEG in this stage consisted mainly of high voltage and slow wave pattern.
The duration of transitional stage after quiet sleep to the following active sleep was approximately 3 minutes. The EEG in this stage was almost of low voltage and irregular wave pattern.
In summary, high voltage and slow wave pattern appeared in quiet sleep and in the transitional stage from active to quiet sleep, at about 36 weeks of conceptional age and it gradually increased with the conceptinal age. This pattern predominated in the first part of quiet sleep.

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1. Cerebellar pathology of a case with classical Tay-Sachs disease was reported. The clinical signs and symptoms appeared at 8 months of age, and the patient died at 3 years.
He was on diphenylhydantoin (DPH) for 2 years and 2 months, which might have contributed to the development of cerebellar degenerative lesions. Chronic DPH intoxication was reviewed with regard to the contributing factors, the clinical aspects, and the diagnosis, as compared to our present case.
2. The common features Of cerebellar pathology between Tay-Sachs disease and chronic DPH intoxication were 1) increase in consistency due to gummy state, 2) wide-spread and diffuse loss of Purkinje cells, and 3) presence of membranous cytoplasmic bodies ultrastructurally.
It was assumed that the pathological changes similar to those in the systemic CNS degenerative diseases might be caused by long-term administration of DPH.
3. It was emphasized that chronic DPH intoxication should be considered on every case under long-term DPH therapy, as it could not be manifested because of the predominant signs and symptoms of the underlying disease.

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We have studied 45 patients having syncopal attacks with loss of consciousness, abnormal EEG, episodes of transient paleness, cyanosis, headache, nausea and sleep after the attack. These patients were from 6 months to eleven years of age. Fifty-six per cent of the patients developed the symptoms by 5 years of age. The history of convulsive disorders was proved in 23%, and the possible causes of seizures such as perinatal abnormalities were recognized in 42%.
The interictal EEGs were abnormal in all patients, showing dysrhythmia or asymmetry (67%), and generalized spike and wave complexes or spikes (96%) which were precipitated by hyper-ventilation in a third of the patients.
The attacks were controlled in 77% of patients by administration of antiepileptic drugs such as phenobarbital and diphenylhydantoin.
The form of attacks and abnormal EEG suggest that this disorder is epileptic and is caused by the subcortical disturbance, especially of the reticular formation system of the brainstem.

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Serum DPA levels were studied on 41 epileptic patients and CSF-DPA levels were also studied on 3 cases.
The results obtained are as follows;
1) The half-life of DPA in serum varied from 18 to 30 hours with a mean of about 24 hours and 4-5 days were required until 95% of the maintenance serum levels was attained.
2) Therapeutic levels were between 65 and 150 μg/ml and an apparent adverse effect of DPA was not observed even in one case with high serum concentration of 160 μg/ml.
3) The level/dose ratio for DPA was about 4.3 in adults and was lower in children than in adults.
4) Diphenylhydantoin and barbiturates decreased the level/dose ratio of DPA, but carbamazepine did not affect it.
5) The concentration in the cerebrospinal fluid for DPA was about 12% of the serum concentration.

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Two cases of “diencephalic cyst”, first presented under this special name by Brocklehurst in 1973, were reported with special reference to its pathogenesis. Our cases, both males, showed abnormally large head, widely open fontanel and ocular hypertelorism. Pneumoencephalograms and cerebral angiograms showed similar abnormalities with those reported by Brocklehurst.According to our histological studies on the developing brain, the wall at the roof of the third ventricle, which was composed of thin membrane, was consi dincreased CSF pressure.
Hence, we concluded that increasing bulging at the roof of the third ventricle might have caused cystic dilatation of the diencephalon and agenesis of the corpus callosum.

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