Migraine and its variants are common diseases seen in the pediatric population. One of the less well-recognized types of complicated migraine is basilar artery migraine. There is little information on basilar artery migraine concerning its relative frequency in the whole migraine, and no general agreement as to both its sex and age distributions, and locations of headache.
Comparative study of basilar artery migraine and other types of migraine was made with regards to sex distribution, age of onset, locations of headache, and family history of migraine.
This study comprised 72 children with basilar artery migraine (22 boys and 50 girls) and 167 children with other types of migraine (73 boys and 94 girls). There was higher occurrence of basilar artery migraine in female to male than that of other migraine, but this was not significant. Age of onset was not different between the two groups. Locations of headache were not different between the two groups (predominantly at frontal and temporal regions). Anamnesis of orthostatic dysregulation and family history of migraine were not different in frequency between the two groups. The neurological signs and symptoms in children with basilar artery migraine were vertigo (46%), visual changes (33%), diplopia (32%), paresthesia (24%), ataxia (14%), dysarthria (6%), and motor disturbance (4%).
Of 239 children with migraine, 30.1% were diagnosed as having basilar artery migraine. Basilar artery migraine was not so rare as previously considered. It did not occur exclusively in adolescent girls. The location of headache was not restricted to the occipital region.

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The study was designed to quantity the hypotonia of children with Down syndrome using our conventional method of hypotonia measurement.
The subjects with Down syndrome consisted of 84 children (36 boys and 48 girls) who were diagnosed as the regular 21 trisomy type by chromosomal analysis and the normal control subjects consisted of 124 children (65 boys and 59 girls). The age range of Down syndrome group was from 3 months to 7 years of age, and the age range of normal controls was from 1 months to 6 years of age.
The hip flexion-knee extention, ankle dorsiflexion and wrist extension were measured by zero-starting position method. For the evaluation of the scarf sign, the range of motion (ROM) of elbow flexion was measured.
The degree of hypotonia in Down syndrome at hip flexion-knee extension, ankle dorsiflexion, wrist extension and scarf sign testing were significantly larger than those of normal control group. These signs of hypotonia in Down syndrome decreased as the age increased. There was no difference between males and females with Down syndrome.
The correlation coefficients between the scarf sign and wrist dorsiflexion, between hip flexion and ankle dorsiflexion, between wrist extension and ankle dorsiflexion and between scarf sign and hip flexion were 0.71, 0.83, 0.81 and 0.75 respectively. The high correlation between each measurement suggests the nature of generalized hypotonia in Down syndrome.

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Posterior fossa subdural hematoma is infrequent, but potentially treatable in the newborn. For the 19 years from 1958 to 1976, 22 cases of posterior fossa subdural hematoma were discovered among 228 autopsies performed on babies up to 28 days of age suffering from intracranial hemorrhage (9.6%). Three cases were treated surgically, of which 2 cases responded to the treatment. It was already reported by us that these 25 cases (3 of our own treated surgically and 22 untreated and found at autopsy) could be divided into 4 groups according to the hemorrhage site, and there existed some correlation between the site of hematoma and the origin of bleeding.
In this paper, we assessed the operative indications for these lesions, by investigating the relationship of the site of the hematoma to the birth weight, the age of onset of symptoms, and the age at death.
It was show that group II, having the hematoma on the upper surface of the cerebellum, provided the most suitable conditions of the operative procedure. Other desirable conditions were indicated as follows;(1) a birth weight greater than 2, 000 g, (2) a clear lucid interval, (3) no combination of intracranial hemorrhage in other sites, and (4) no severe pulmonary complications.

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Computed tomography (CT) studies were performed on 24 children with bacterial meningitis (1 month-16 years) within 2-4 weeks after the onset of the disease, and the results were correlated to the neurological sequelae after 2-3 years.
CT abnormalities were found in 15 patients less than 2 years of age. Among them 3 patients died at theearly stage of the disease, and the neurological sequelae persisted in 6 patients. The prognosis was better in the patients with normal CT than in those with abnormal CT (P<0.01). Abnormalities consisted of venticular dilatation (6 patients), subdural effusion (7 patients) and brain atrophy (2 patients). Three patinets with ventricular dilatation showed a gradual improvement in follow-up CT and had no neurological sequelae. Subdural effusion disappeared within 6 months after the onset without sequelae in 4 patients with normal intracerebral density in the initial CT. The patients with low or high intracerebral density died early in the course of the disease (3 cases) or survived with severe neurological sequelae (3 cases). The prognosis was worse in the patients with low or high intracerebral density than in those with normal intracerebral density (P<0.05). It was concluded that the late neurological sequelae could be anticipated by the initial CT findings.

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Four cases whose CT scans showed multi-focal or diffuse low density in both cerebral hemisphere were reported and their clinical histories, roentogenographic and electroencephalographic findings were compared
Out of 2 cases with multi-focal low density, one fatal case (Case 1) was diagnosed neuropathologically as ulticystic encephalomalacia (MCEM). He had asphyxia at delivery, convulsion and fever for a few days from the first day. Case 2 was one of twins with a clinical history of intrauterine onset. He had no trouble in delivery but poor feedings in the neonatal period. Two cases showed microcephaly, severe psychomotor retardation and tetraplegia later.
Two cases with diffuse low density had the episode of respiratory arrest in early infancy. In one patient (Case 3), several cavities in CT and PEG at 2 months of age were demonstrated, and changed into diffuse low density of cerebral hemispheres at 8 months. Case 4 showed almost normal CT at the age of 16 days and developed diffuse low density at 5 months. They also showed microcephaly, severe psychomotor retardation and tetraplegia.
EEG showed irregular slow wave activities in 2 cases with multicystic lesions, and almost flat activities in 2 cases with diffuse cystic lesions.
The former was compatible with MCEM and the latter hydranencephaly of postnatal onset described by Friede. It was concluded that diffuse cystic lesion could be classified as an extreme type of MCEM, and both destructive lesions caused by pre-or postnatal hypoxia and circulatory disturbances of different severity. Serial CT scans might demonstrate the onset and progression of these pathological changes. EEG was also useful for monitoring the courses of these diseases.

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Our previous work has indicated that the levels of prostaglandinF_2α (PGF_2α) in the cerebrospinal fluid (CSF) were increased during febrile convulsions. The purpose of this study was to investigate the relationshipbetween CSF PGF_2α levels and types of epilepsy or febrile convulsions and to analyze the factors affecting the CSF PGF_2α levels.
CSF PGF_2α was measured by radioimmunoassay. Children with febrile convulsions (31 cases), with epilepsy (32 cases), with meningitis (31 cases), and with non-neurological diseases (20 cases) were tested (total 114 cases).
A 4.5-fold increase of CSF PGF_2α levels (239.3±69.0pg/ml [mean±SE], n=22) was seen in simple febrile convulsion cases and a 2.5 fold increase of CSF PGF_2α levels (130.9±36.3, n=9) in complex febrile convulsion cases, as compared to the levels of CSF PGF_2α (53.5±8.7, n=20) in children with non-neurological diseases. The mean CSF PGF_2α levels (43.0±4.6, n=32) in epilepsy cases was not statistically significantly different from that in non-neurological diseases. There was no statistical difference between CSF PGF_2α levels and types of epilepsy.
When the body temperature was normal, the mean CSF PGF_2α levels had no relation with the age. When the body temperature was between 37.5°C and 40°C, the levels in infants were higher than those in older children and adults. The levels in infants with febrile convulsions were significantly higher than those in infants with non-neurological diseases. The CSF PGF_2α levels in children with meningitis were high. The mean CSF PGF2a levels (118.8±19.1, n=11) in bacterial meningitis cases was not statistically different from that in viral menigitis cases (107.1±19.6, n=27).
The CSF PGF_2α levels in meningitis cases were high on admission and gradually decreased after the therapy. The results from our studies revealed that PGF_2α of the central nervous system was markedly increased in infants with febrile convulsions and in children with cerebral inflammatory disorders.

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Using two-dimensional ultrasonography, we were able to observe normal intracranial structures in newborns and young infants. We used a 5-MHz mechanical sector scanner and occasionally a 3.5-MHz scanner, placing the transducer probe directly over the anterior fontanel.
The 8 standard planes that we proposed in this report were 4 coronal planes (anterior horn, third ventricle, thalamus and posterior horn planes) and 4 sagittal planes (third ventricle, lateral ventricle body, posterior horn and insula planes). In coronal scans, we set the right side of the infant on the right side of the recorded imagittal scans, we set the anterior side of the infant on the rigittal scans, we set the anterior side of the incant on the right side of the recorded image.
This method had many advantages in comparison with CT scanning in the following points. The ultrasonic power was thought to be non-invasive for the neonatal brain. As the unit was portable and the procedure did not require sedation of the patient, we were able to examine premature infants in their incubators, observing the recorded images simultaneously.
On the recorded images of the two-dimensional ultrasonography, the contrast of the ventricle and the brain substance was clear even in premature infants, the basal ganglia and cerebral cortex presented different echo patterns, and the choroid plexus was highly echogenic without contrast enhancement.

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A 12-year-old girl with ataxia-telangiectasia was presented. The main clinical symptom was dystonia, masking cerebellar ataxia since early childhood. Significant deficiency of IgA and impairment of celluar immunity were not observed.α-Fetoprotein levels were significantly raised.
The sensitivity to x-ray of cultured skin fiblasts from our patient was clearly higher compared with that of the cells from normal subjects.
Light and electron microscopical observations on the biopsied sural nerve disclosed decreaed density of total myelinated fibres, a selective loss of large diameter fiber, inculsion bodies in Schwann cell cytoplasma, increased denervated Schwann cell bands and collagen pockets. Those findings in our case were similar to those in Friedreich ataxia. It was concluded that sural nerve biopsy and radiosensitivity test in cultured skinN fibroblasts were of useful diagnostic value especially in atypical cases of ataxia-telangiectasia.

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An autopsy case of Leigh's disease with pyruvate decarboxylase deficiency was reported. An 11-month-old boy with muscle hypotonia and neurologic deteriorations had lactic acidosis, pyruvic acidemia and alaninemia due to deficiency of the pyruvate dehydrogenase complex and pyruvate decarboxylase in platelets and biopsied muscle. At 26 months of age he died of sudden pulmonary aspiration. At autopsy extensive symmetric necrotic lesions were found in the cerebral and cerebellar cortex, basilar nuclei, tegmentum of the midbrain, pons and medulla oblongata and dorsal horns of the cervical spinal cord with proliferation of capillaries and gliosis, and diffuse demyelination in the white matter. These lesions closely resembled those observed in Leigh's disease. The activities of the pyruvate dehydrogenase complex and the pyruvate decarboxylase were markedly decreased as compared to control values in various tissues (liver, kidney, cerebrum and cerebellum) obtained at autopsy. However, the pyruvate carboxylase activity was within normal range.

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[in Japanese]

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[in Japanese]

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