CT findings in 43 children with generalized seizures (grand mal seizures) (GM group) and in 50 children with partial seizures (P group), classified according to clinical seizure type, were studied.
1) CT abnormalities were demonstrated in 19%(8/43) in GM group and in 40%(20/50) in P group, including localized CT abnormalities in 9.3%(4/43) and in 34%(17/50) respectively. CT abnormalities were found more frequently in cases with abnormal past histories and/or mental defects (MD) than in those without them.
2) In P group, localized CT abnormalities and generalized brain atrophy were observed in 13 and 7 cases respectively. In 40 cases with focal epileptic discharges (FED) in EEG, localized CT abnormalities were demonstrated in 23%(9/40). The sites of localized CT abnormalities corresponded in 73%(11/15) to the sites of focal suppression in EEG (i. e., slowing, low amplitude and lazy pattern) regardless of FED.
3) The bicaudate cerebro-ventricular index (B-CVI) in 19 cases in the normal control group over 2 years of age was 10.0±1.2 (mean±SD). Ventricular narrowing (VN), with B-CVI less than 8.2 (mean-1.5 SD), was observed in 5, 17 and 27% in control, GM and P groups respectively, indicating more frequently in epileptic children than in normal controls. Seizures were well controlled in 85%(11/13) in VN group and in 70%(26/36) in normal ventricular group. Brain atrophy in CT findings to inspection was ascertained in all cases by measuring B-CVI.

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Adrenocorticotropic hormone (ACTH) or corticosteroids seemed to cause brain atrophy in intants. We studied the atrophy which was caused by these drugs with computed tomography (CT).
1) Nine cases of infantile spasms examined before, during and after ACTH therepy with CT. Brain atrophy on CT was observed immediately after the completion of ACTH therapy. The brain atrophy receded slightly after several months. It was more marked in younger patients, in cases treated by hight doses of ACTH and in cases where brain atrophy had already been obserbed before ACTH therapy.
2) Twenty cases of infantile spasms or Lennox Gastaut syndrome were examined after ACTH therapy with CT. Brain atrophy was observed in twelve cases. Main features of brain atrophy were the enlargement of sylvian fissure and the widening of subarachnoid space at the frontal or temporal region. Mental retardation was observed in eighteen cases.
3) Two cases of nephrotic syndrome were treated with pulse therapy of prednisolone. CT was carried out before and after treatment. Atrophy of cerebrum was observed in these cases.
4) A case of infantile spasms treated with anticonvulsants without ACTH was studied by electroencephalography (EEG) and CT. The abnormal pattern of EEG was markedly corrected, while brain atrophy on CT was not observed after the therapy.
Because of these observations the use of ACTH has to be reconsidered. ACTH should be the drug of second choice for the therapy of infantile spasms and should be used in case other anticonvulsants have no effect. ACTH should be used at lower dosages and for shorter periods of time.

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Serum valproic acid (VPA) levels were studied on children with convulsive seizures, ranging from 2 months to 9 years of age, using gas-liquid chromatographic method. Serum VPA levels were studied hourly on 12 patients before and after oral administration of this drug as syrup. In all cases the drug was administered regularly three times (10 cases), twice (1 case), or once (1 case) a day for over one month. The lowest values (Cmin) of the serum levels were observed just before administration, and the highest values (Cmax) were observed one to three hours, most two hours, after administration. In time-course study done on 6 patients, serum VPA levels (Cmin) were highest (P μg/ml) at 2nd to 4th day, and reached stable concentrations (S μg/ml) in blood at 6th day after administered three times a day. The P/S ratio were lower in younger children.
Cmin and Cmax of serum VPA levels showed a good correlation with the dosage (mg/kg/day) of VPA in cases on this drug alone. There was a negative but good correlation between the percentage of fluctuation and Cmin. The percentage of fluctuation was decreased and constant (20-25%) in cases with Cmin over 100μg/ml. Therapeutic serum VPA levels (Cmin) were 40 μg/ml or more.
The mean ratio of level to dosage was higher in cases with VPA only than in cases with VPA and other antiepileptic drugs.

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A consecutive series of 100 children with neurological diseases or head trauma were examined by means of computerized tomography. A high incidence of bifrontal low density area (BELD) was found in cases below 2 years of age, especially between 6 and 12 months, while BELD was much less common in cases above 2 years of age, though most of them had neurological symptoms and/or signs. Most cases with follow-up CT scanning had increases in width of BELD at the ages of 6 to 12 months. The results suggest that the trend of CT findings related to age in the present study may reflect physiological changes of the space between the skull and the brain with age.
The most characteristic CT findings in 4 cases with chronic subdural hematoma were a crescent-like BELD with a relatively linear contour on the cerebral cortex and a wider BELD than that of dilated subarachnoid space. Other findings included an asymmetry in width of BELD and interhemispheric low density area and less prominent sulci than those in cases with dilated subarachnoid space.

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Auditory brain stem responses (ABR) were examined on 121 individuals with normal hearing in special reference to the developmental changes. The following nine age groups were tested: 8 neonates, 10 two-month-olds, 10 four-month-olds, 10 six-month-olds, 15 one-year-olds, 17 two-year-olds, 18 three-year-olds, 18 eight-yearolds and 15 adults.
Under our test conditions wave components of ABR were well differentiated even in neonates. Although there were considerable developmental changes in the latency and the amplitude of each ABR component, the fundamental characteristics of the wave patterns were retained withn the same individual. This was found in 7 subjects who were studied longitudinally from birth to 2 years of age. Also a remarkable resemblance was observed between the wave patterns of a pair of monozygotic twins.
Mean values for the peak latency and the amplitude of each ABR component were obtained for all age groups. The peak latencies of all components showed a steady decrease with age toward the adults. Although the decrease was more remarkable with the slower component, the peak latency of the faster c9mponent reached the adult value more rapidly. Thus, the latency of wave I reached that of the adult at 2 months of age, wave III at 2 years and wave V at 3 years of age.
The amplitude of each ABR component showed larger individual variability than the peak latency. Until 3 years of age the amplitude of wave III was the largest of all waves, but thereafter wave V became the largest.
The application of ABR to objective audiometry was examined and discussed. The delay of wave V peak latency was considered to be the most suitable index for that purpose. Our results showed that 0.7 msec prolongation of wave V latency at 85 dB corresponded to 30 dB hearing loss.

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One hundred and fifty one patients (64 with epilepsy, 21 with febrile convulsion, 26 with myasthenia gravis, 10 with cerebral palsy and others) were vaccinated against measles, varicella, rubella, mumps, influenza, diphtheria, tetanus, pertussis and Japanese B encephalitis. The vaccines were inoculated 510 times and the side effects were observed 56 times (11.0%). Fever was observed in 47 patients, 6 revealed convulsion and 3 showed aggravation of the underlying disease. However, these side effects disappeared within a few days. EEG patterns did not change significantly before and after vaccination in epileptic patients. Severe complications were not experienced in any of the cases studied. The seroconversion rates were 83% in 100 varicella vaccine recipients, 95.7% in 69 measles vaccine recipients, 93.4% in 106 rubella vaccine recipients and 99.0% in 97 mumps vaccine recipients. These results were similar to those in healthy children.

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The clinical significance of CT was investigated in group of 25 patients with non-progressive ataxic syndromes. The age of the patients ranged from 3 to 19 (11 males and 14 females). All patients were evaluated from the points of the neurological findings and their motor developmental history, and also examined radiologically with CT.
Six view points about the hemisphere and vermis of the cerebellum in CT were graded from 0 through 3; 0-normal, 1-mild, 2-moderate, 3-severe. The degree of cerebellar atrophy was expressed as the total score the score of cerebellar atrophy, SCA11 cases whose total scores were above seven had distinct abnormalities in the vermis, and markedly retarded in their motor development. Although the cases without any abnormal findings in the vermis were below seven in SCA, three cases in the group had severely retarded milestones (24 months were needed for them to sit up and crawl). No close correlation was found between SCA and the motor developmental retardation.
Thirteen cases, over 5 years, were classified into dysequilibrium syndrome (DES) and congentital cerebellar ataxia (CCA) according to Hagberg's criteria. Six cases who had abnormalities in the vermis were diagnosed as DES, while seven cases who had no abnormalities in the vermis consisted of four cases of DES and three cases of CCA. Therefore, in the cases of DES diagnosed on the clinical findings, there were two different types DES with or without abnormalities in the vermis. There were no differences of IQ and motor development between these two types.
In summary, CT allowed the diagnosis of DES with abnormal findings in the vermis, but when the vermis has no abnormalities it may be difficult to distinguish DES from CCA. There was no correlation between SCA and motor developmental retardation.

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1. Changes in the platelet count before and after sodium valproate administration were studied in 90 epileptic children. A decrease of more than 20% in the platelet counts as compared to those prior to the administration was found in 63 cases. An abnormal decrease to below 150, 000 was seen in only three cases.
2. The platelet count decrease started four months after the commencement of the administration in the case of the group given VPA alone (Group I), whereas it started one year and four months after the commencement of the administration in the case of the group to which VPA was given in conjunction with other drugs (Group II). In most cases, a decrease of about 30% in the count continued. Although no substantial difference was found between Group I and Group II in the decrease of the platelet count, the degree was slightly higher in Group I.
3. No particular relationship between the decrease in the platelet count and sex, age, form of the drug or the dosage was found, except that the decrease was slightly greater among females in Group I and the commencement of the decrease was earlier among the Group I cases who were given tablets.
4. In one case, petechiae was observed clinically.

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Treatment with sodium valproate (VPA) alone was undertaken in children with epilepsy of the convulsive type, in particular, tonic-clonic seizures, and the comparative efficacy of VPA in primary versus secondary generalized epilepsies in terms of seizure control and electroencephalographic improvement was studied.
1. Complete seizure control was achieved in 23 out of 33 primary (70%), while in 5 out of 15 secondary cases (33.3%).
2. Between the effects and the time lag of starting medication after the onset, no significant relation was found.
3. The effectiveness of VPA on paroxysmal EEG abnormalities was found to be marked in 8 and moderate in 4 among 17 cases of the primary group. Among 13 cases of the secondary group, marked and moderate improvements were noticed only in three and one, respectively, while in other nine (69.2%) EEG did not changed at all or even worsened. However, the difference between the two groups was not significant.
4. Electroencephalographically, the effect of VPA most prominently manifested against diffuse paroxysmal discharges, while it was poor against unilateral focal paroxysmal discharges.
5. In a few cases, a complete control of seizures was attained with serum VPA concentration of less than 40 μg/ml.

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The patient was a 6 1/2-year-old girl. Her 9-year-old sister showed almost identical symptoms and was diagnosed as lipid storage myopathy in this hospital on the basis of muscle biopsy. Her parents were non-consanguineous. This patient had been floppy with delayed motor development (walking alone at 1 year and 8 months). Since the age of 4 years, myopathic face including puffy eyelid and open mouth, climbing up herself, and poor activity were present. She could not walk for a long time. On admission, physical examination revealed a slender girl of normal height with muscle weakness and hypotonia. Her activity was markedly poor and deep tendon reflexes were weak. She had normal intelligence.
Laboratory data were as follows; serum CPK 4 U/ml (normal range 0-12), aldolase 3 mU/ml (0-6), lactate 11.2-22 mg/dl (4-16), pyruvate 0.98-0.57 mg/dl (0.3-0.9), EMG myogenic pattern. NCV (ulnar nerve), EEG, optic fundi, audiometry, ECG, amino acids in urine and serum were all within normal limits.
Intravenous glucose loading test (2 g/kg) revealed an elevation of lactate from 22 to 38 mg/dl, pyruvate from 0.54 to 1.25 mg/dl, blood glucose from 75 to 440 mg/dl. Mild exercise (slowly up and down stairs) provoked the following changes; blood lactate from 12 to 68 mg/dl, pyruvate from 0.9 to 0.8 mg/dl, arterial blood pH from 7.373 to 7.262, BE from-2.1 to-12.
Muscle biopsy (quadriceps muscle) revealed variation in fiber size on light microscopy. Multiple vacuoles were found in muscle fibers in HE stain. Modified Gomori's trichrome stain showed some ragged-red fibers. Electron microscopy showed many lipid droplets between myofibrils and some abnormal mitochondria.
Carnitine, carnitine palmityl transferase in biopsied muscle, and plasma carntitine determined by Dr. A. G. Engel were within normal limits. Pyruvate dehydrogenase complex activity in muscle assayed by Dr. H. Maesaka was 7.8 pMol/min/g. protein (normal control 14.6±1.12). This patient's abnormality was suspected due to disorder of pyruvate dehydrogenase complex in muscle.

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A 3-year-old girl was admitted to our clinic because of an ocular hypertelorism due to subcutaneous lipoma at the midline of forehead which is possibly communicating with the corpus callosum. No seizure and no neurological abnormalities were noted. Her mental and motor developments has been kept within normal range up to the present. The plain skull films showed an egg-sized radiolucent area which was surrounded by the curvilinear calcification in the midline of the forehead frontal bones. Angiograms revealed bilaterally dysplastic pericallosal arteries that were irregularly dilated. CT scan showed an extremely low density area which was enclosed by the characteristic high density area of the calcified lipomatous capsule. The neuroradiological characteristics of the lipoma of the corpus callosum were described.

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