We treated five severely handicapped persons who had uncontrolled seizures with immunoglobulin (Ig). We examined its efficacy on seizures and immunity. Ig with the dose of 100-300mg/kg body weight was administered intravenously at intervals of one or two weeks. Seizures decreased in one, did not change in two, and increased in two cases. There was no improvement in electroencephalogram abnormalities. The serum IgG and IgA increased whereas IgM decreased. Ig production stimulated by pokeweed mitogen decreased parallel with a decrease of Leu 10 positive lymphocytes. No change was observed in natural killer and killer T cell activities. The mechanisms of Ig effect on seizures are not yet known.
It was speculated that Ig might act through suppression of antibody synthesis on the epileptogenic focus.

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Seventy-seven full-term newborns admitted during the period from 1979 to 1983 were subjected to polygraphic recordings (EEG, EOG, ECG and respiration) of 2.5-3.5hr duration in the first week because of suspicion of CNS abnormality. Their conceptional age ranged from 38w to 42w and birth weight from 1, 550g to 4, 450g. Forty-eight had anoxic encephalopathy (28 of them developed neonatal convulsion), 2 CNS infection, 1 bacteremia, 15 intracranial bleeding or neonatal seizure of unknown etiology and 11 CNS abnormality of prenatal origin.
The classification of the background EEG was performed by visual inspection of whole polygraphic records; maximally, markedly, moderately, mildly and minimally depressed, normal and other abnormalities, according to the distribution of EEG patterns.
Twenty-four cases died in the neonatal period, 28 developed normally and 23 became neurologically handicapped.
The clinical outcome of the subjects in relation to the neonatal background EEG were evaluated.
Eighteen out of 22 neonates who showed normal or minimally depressed background EEG developed normally and the other four became mentally retarded. Ten out of 25 patients who showed markedly or maximally depressed background EEG died in the neonatal period and all the survivors were handicapped neurologically including 7 epileptics.
In the perinatal hypoxic group, the neonatal convulsion group and the neonatal convulsion caused by perinatal hypoxia group, the patients whose background EEG was normal or minimally depressed did not develop cerebral palsy or epilepsy, whereas those with markedly or maximally depressed background EEG were associated with neurological sequelae. The more abnormal the neonatal background EEG was, the more frequently neurological sequelae developed later.
Serial EEG recording was found to be useful to prognosticate the clinical outcome.

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The present study was conducted to examine the visual recall memory and the visual information processing of 28 epileptic children whose ages ranged between 8 and 12 years and who had an IQ between 90 and 130. Age-and IQ-matched healthy children were served as controls. A task of recalling 12 pictures was given ten times repeatedly to each subject. The recall score (the number of words correctly recalled) and the clustering score (the number of pairs of conceptually similar words recalled) were employed for evaluating the subject's recall memory and the category clustering. The epileptic group showed a lower level in both scores than the normal group.
Then the effect of anti-epileptic therapy was examined on the recall memory and the category clustering of patients. The groups of patients with the onset of seizures under 4 years of age, with generalized seizure type, receiving therapy with multiple drugs, receiving phenobarbital, and with a good seizure control, showed lower levels of both scores than the other epileptic groups.

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This study was undertaken to investigate the effect of monosodium L-glutamate (MSG) on brain de-velopment with special reference to the cell proliferation at the external granular layer (EGL) of the cerebel-lum using autoradiographic technique.
Male Jcl: ICR strain mice were subcutaneously injected with MSG, 2mg/g of body weight daily for the first five days of life. Cerebral and cerebellar weights of MSG-treated mice were compared with those of control mice at 10, 20, 30, 60, 90 and 120 days of life. At 20 days of life, the cerebral and cerebellar weights of MSG-treated mice were significantly lower than those of control mice.
Cell cycle studies on the external matrix cells of EGL of MSG-treated and control mice were carried out at 1 day and 7 days by means of 31-I-thymidine autoradiography. At 1 day, the generation time of MSG-treated mice was about 17.0 hours, whereas that of control mice was about 15.1 hours. This prolongation of the generation time of MSG-treated mice was caused mainly by a delay of Gi-phase and partly by that of S-phase. At 7 days, the generation time of MSG-treated mice and control mice was 17.5 hours and 16.6 hours, respctively. The prolongation of the generation time in MSG-treated mice was caused by a delay of G1-phase.
Mitotic index (MI) and labeling index (LI) were calculated at EGL of 1-, 2-, 3-, 4-, 5-, 7-, 10-, 15-, and 20-day-old mice in both groups. MI and LI of MSG-treated mice were significantly smaller than those of control mice during the first 7-10 days of life. After 10-15 days of life MI and LI of MSG-treated mice be-came significantly higher than those of control mice. EGL in MSG-treated mice still remained at 20 days of life and disappeared later than that in control mice. These facts indicated that MSG treatment to neonatal mice reduced the mitotic activity and DNA synthesis and suppressed the cell proliferation at EGL for the first several days with subsequent catch-up in MSG-treated mice. In addition, it was suggested that MSG had a direct effect on the cell proliferation in EGL.

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In order to clarify the relationship between brain damage and skull asymmetry or supine head position preference, we classified CT findings of 330 cases with cerebral palsy or risk of motor disturbance into 6 groups according to skull shape. Those were severe (I, n=37) and mild (II, n=114) grades in the right occipital flatness, severe (III, n=34) and mild (IV, n=58) grades in the left occipital flatness, long skull with temporal flatness (V, n=33) and symmetric round skull (control, n=54).
It was considered that the asymmetry of cortical atrophy in appearance was formed physicaly by skull asymmetry but that the asymmetric dilatation in appearance of lateral ventricle was related to the asymmetry of brain damage. The severity and the asymmetry of brain damage were tend to increase the grade of skull asymmetry. The incidence of cases with the right occipital flatness was 1.6 times more frequently than the left sided. The incidence of cases whose left (lateral) ventricle was larger than the right was 4.1 times more than the cases whose right ventricle was larger than the left. The cases with occipital flatness in the contralateral side of the larger lateral ventricle were found more than the cases with occipital flatness in the ipsilateral side of the larger ventricle, that is to say, the direction of supine head position preference during early infant was suspected to be the more severely disturbed side of body.
These results suggest that the supine head position preference to the right in newborn babies and infants with scoliosis or cerebral palsy might be the result of transient or permanent asymmetric (left>right) brain dysfunction.

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Twelve cases of atlanto-axial subluxation (AAS) with Down's syndrome and one case of occipito-atlantal dislocation (OAD) were described. All the patients developed neurological symptoms. The diagnosis was obtained by extension and flexion cervical spine X-ray, myelography and CT myelography. Out of 12 patients with AAS, 11 had an ossiculum terminale, and 4 had basilar invagination. All patients had myelography, which revealed stenosis of the spinal canal at C1 level with cord compression in the flexion position. Patients diagnosed in the early period and receiving solid C1-2 posterior fusion in the reduced position showed favorable results.

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We investigated 6 patients of central nervous system disorders who showed low body temperature chronically. Their etiologies were varied. Hypothalamic dysfunction was suggested by the endocrinological examination, and clinically brainstem dysfunction was suspected in all cases. Five cases of them were accompanied with circadian rhythm disturbances of body temperature and sleep-wakefulness. This fact suggests that the close relation of thermoregulation and its rhythm mechanism, and also that the pacemakers of sleepwakefulness and body temperature rhythms are situated closely.

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An 8-year-old boy of epidermolysis bullosa hereditaria (autosomal recessive dystrophic type) was re-ported, who had demonstrated trunkal ataxia and coarse tremor of upper extremities on extension for a half year prior to his death.
Slight ventricular dilatation, widening of the sulci and maldevelopment of the white matter were noticed already at 6 years of age by CT scan.
Neuropathologically, the following changes were found; 1) degeneration of the posterior columns, the spinocerebellar tract and the corticospinal tract, 2) neuronal swelling of the anterior horn cells, the nuclei of the brainstem and the dentate nuclei of the cerebellum. These pathological findings were compatible with those B complex deficiency. Therefore the supplementary therapy of vitamin B complex is recommended on this disease.

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We reported an 11-year-old boy with multiple sclerosis who relapsed whenever he suffered from upper respiratory infection. He gained transient remission with peroral steroid therapy for 6 months after his initial onset. But, since his first relapse, he was unable to support his posture for 20 months because of his severe trunk ataxia. During that period, he was treated with a high-dose of methylprednisolone (pulse therapy) in exacerbation and gained remission. Subsequently, even though he had not neurological disorders, we treated him with pulse therapy, whenever he seemed to be in aggravation with high levels of CSF IgG and myelin basic protein (MBP). Then he maintained remission and became quite normal. A pulse therapy according to the CSF findings was beneficial in the management for acute multiple sclerosis relapse.

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Epidural hematoma is rare in newborn infants. Only 12 prior cases have been reported in English or Japanese writings. Nine cases of those were treated surgically. We reported two cases of newborn infants with an epidural hematoma which was detected by ultrasonic sector scan below a cephalhematoma with linear skull fracture immediately, after birth. In both cases, the epidural hematoma disappeared after the aspiration of cephalhematoma using a syringe. The patients have no abnormal neurological signs at present one year after birth.
It was recommended to perform aspiration of cephalhematoma in cases of possible communication between epidural hematoma and cephalhematoma through skull fracture was suspected. Furthermore, ultrasonic sector scan is a very useful image system in the diagnosis and follow-up of epidural hematoma as well as other intracranial hemorrhages.

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[in Japanese]

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[in Japanese]

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[in Japanese]

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