Ten children (nine males and one female) from 11 months to 5 years of age were diagnosed as having basal ganglionic infarction by computed tomography (CT).
In all patients presented hemiparesis progressed either rapidly or gradually. Other neurological findings included facial palsy, dysarthria, aphasia, tremor and mild consciousness disturbance. The lesions were classified by the findings of CT. The most frequent lesion was the putamino-capsulo-caudate type which involved the putamen, the head of the caudate nucleus, and the anterior limb of the internal capsule. Lesions involving the posterior limb were found in only one patient, who had a history of repeated transient ischemic attack.
The severity of hemiparesis and prognosis were not correlated with the location and the size of the lesions, but the prognosis was correlated with the mode of the onset. Two patients who did not have good prognosis showed abnormal findings on cerebral angiography and had a gradual onset.
Although seven of the 10 patients had clear-cut histories of mild head trauma prior to the episode of hemiparesis, the trauma occurred one month before the onset in two patients and previous fever suggesting a history of infection was found in four patients. There were two patients with narrowing of the middle cerebral artery and one with congenital heart disease. The etiology was unknown in the other cases.

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Mice injected with monosodium L-glutamate (MSG) in the neonatal period become obese in adulthood and have a short body length, hypogonadism and microcephalus. These MSG- treated mice may be a model of symptomatic obesity with brain damage such as the case of Laurence-Moon-Biedl syndrome. This study was undertaken to investigate the dendritic development of pyramidal cells in the cerebral cortex of MSGtreated mice.
Male Jcl; ICR strain mice were subcutaneously injected with MSG daily for the first five days of life. MSG-treated mice, as well as control mice, were sacrificed at 30, 60, 90 and 120 days, and their brains were then processed by the Golgi-Cox technique. In coronal sections, dendritic development of pyramidal cells in layer 5 in the parietal region (area 1) was assessed quantitatively according to Eayrs' method. The dendrites of MSG-treated mice were shorter than those of controls, and the density of dendrites in MSG-treated mice was less than that in controls in the distal zone from the perikarya. Although the dendrites of MSG-treated mice showed the “catch up” phenomenon especially in the proximal zone, the recovery was not enough as a whole.
These results suggest that mentally retarded patients with symptomatic obesity may have similar developmental aberration of dendrites in their brains.

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One hundred and forty-seven asphyxiated full-term neonates admitted over an about 4-year period (April 1977-December 1980) were followed up for 3 to 6 years. Ten (6.8%) died in the neonatal period. Sixteen (10.9%) developed neurological handicaps, 5 of which died in infancy. A poor prognosis was found to be significantly correlated with the presence of neonatal seizures, especially intractable ones, and also with an increased base deficit. Outborn patients had worse prognosis than inborn patients. Obstetrical care based on fetal heart rate monitoring and pediatric care involving proper resuscitation after birth would be necessary to decrease the incidences of asphyxiated neanates and handicapped children.

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Recently, the blink reflex (BR) has proved to be useful for evaluation of the brain stem function and has been investigated in different neurological diseases. We investigated electrically elicited BR in 30 handicapped children, aged 7 months to 13 years, and studied the relationship between the BR pattern and the clinical findings. Twenty of the 30 cases exhibited idiocy could not rotate their trunks by themselves.
The BR was classified into 9 types; A) no response (10), B) R 1 on one side only (1), C) absence of bilateral R 2 (1), D) depressed R 1 and R 2 on one side (1), E) depressed bilateral R 2 (9), F) delayed R 1 and R 2 latency (3), G) delayed R 2 latency (2), H) delayed R 1 latency (2) and I) normal response (10). Each case of showing types A-D, which suggested a greater supression of brain stem function, had respiratory disturbance and dysphagia. Out of the 9 showing type E, whose motor and mental disturbances were severe, 7 had stridor and 2 dysphagia. Out of the 17 showing type F-I, 12 were idiocy and 7 had severe motor disturbance, but only one had stridor and none had swallowing problems. This may indicate that in some cases of severe handicapped children without dysphagia, respiratory disturbance or stridor, there are a normal brain stem function and normal BR.
We conclude that BR is a simple and objective neurophysiological test for evaluating the brain stem function related to neural organization of respiration and deglutition in children with neurological disorders.

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The auditory brain-stem response audiometry (ABR) was recorded in children with a delay in development of speech and language. One hundred and twenty-three children (105 males, 18 females), who had no response to mother's calling at 1 year, no 2-word phrases at 2 years, or no simple sentences at 3 years of age, were examined. Their ages ranged from 1.1 to 7.5 years with a mean of 3.7 years. Click auditory stimuli (4, 000 Hz, 20 stimuli per second, 95-35 dBHL) applied to the subject's ears were used. From the response average trace, the latency period of wave I, III, V, I-V were measured. The children with delayed speech development in this study were divided into 6 groups: mental retardation (55 cases, 44.7%), autism (31 cases, 25.2%), minimal brain dysfunction (11 cases, 9.0%), sensorineural hearing loss (10 cases, 8.1%), cerebral palsy (8 cases, 6.5%) and idiopathic speech retardation (8 cases, 6.5%). Eight children had no responses in the ABR, and 2 children had high threshold (55 and 65 dBHL) of wave V. These 10 children were diagnosed as hearing loss by behavioral audiometry. There were no differences in the ABR pattern between our subjects other than hearing loss and the normal control. ABR was a useful screening method for detection of hearing loss in young children with delayed speech development.

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Amino acid concentrations in the serum and urine of 118 neuropediatric outpatient clinic children aged from 3 months to 22 years were measured by ion exchange column chromatography. The subjects were divided into three groups: 53 epileptic patients taking VPA (sodium valproate) alone or VPA and other anticonvulsive drugs (group 1), 38 epileptic patients on anticonvulsive drugs other than VPA (PB, CBZ etc.) (group 2) and 27 patients who had no metabolic disorders and were taking no anticonvulsive drugs (group 3 as controls).
In serum, glycine, proline, lysine, serine, tyrosine, threonine, valine, leucine, and isoleucine were elevated more than 2 S. D. above normal values in group 1, and statistically significant differences were found between group 1 and group 2 except in the values for lysine and threonine. In urine, only gycine was elevated in group 1 and a statistically significant difference was found between group 1 and group 2 and 3.
These results indicate that VPA administration causes the elevation not only of glycine, as previously reported, but also of many other serum amino acids. Further examination of this problem is necessary.

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A case of pituitary dwarfism associated with morning glory syndrome and transsphenoidal encephalocele was reported. A 4-year-old boy visited our hospital because of dwarfism. He had been diagnosed as morning glory syndrome at 6 month of age. He was 90.2 cm tall (-3.5 SD) on admission. The hypothalamic-pituitary function was studied and growth hormone deficiency was revealed. Radiological studies showed transsphenoidal encephalocele extending into the epipharynx. It was concluded that pituitary dwarfism of this patient had been caused by transsphenoidal encephalocele.

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Arthrogryposis multiplex congenita (AMC) is a disorder characterized by rigidity and clubbing of more than two joints at birth. The authors presented a case of severe AMC associated with pseudohypoaldosteronism.
This male infant was born at 41 weeks' gestation. He presented flexional contractures of the elbows, hips, ankles and fingers, with ulnar deviations at the wrists, straight knees, and adducted and internally rotated shoulders. He also showed typical smooth skin, sausage-like limbs and a lack of skin creases.
A biopsy of the biceps femoris muscle (paralytic side) showed marked group atrophy, whereas one of the quadriceps femoris muscle (non-paralytic side) showed an almost normal structure. Brain CT revealed marked cortical atrophy with enlargement of the lateral ventricles. Laboratory data were normal on admission. When he started vomiting frequently at 4 months of age, hyponatremia and hyperkalemia were seen in spite of the high level of aldosterone. And there was sodium loss from his salivary glands, so he was diagnosed as having pseudohypoaldosteronism. He improved with oral sodium intake, but he died at 9 months of age because of heart failure.
Autopsy examination showed marked atrophy of muscles, segmental atrophy of anterior horn cells and scattered gliosis in the white matter of the parietal lobe. Both adrenal glands showed marked hypoplasia, which comprised ill-defined three zones (left, 1.0g; right, 1.5g).
We suggested that different grades of muscle atrophy were due to the segmental atrophy of the anterior horn cells, which resulted in joint contractures. Furthermore, scattered changes of the parietal lobe might have been associated with the atrophy in the anterior horn cells.

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Although viral encephalitis is known as one of the causes of infantile spasms, no cases of infantile spasms due to herpes simplex type 1 virus encephalitis (HSE) has appeared in the literature. A case, a 1-year-4-month-old boy, with infantile spasms due to herpes simplex type 1 virus encephalitis (HSE) was reported. He was admitted to St. Mary's Hospital beCause of generalized convulsion, fever and disturbance of consciousness. The herpes simplex virus antibody titer was measured both in the acute stage and at eighteen days of illness, and he was finally diagnosed as having HSE.
Electroencephalography showed typical periodic sharp waves and CT revealed marked bilateral low density areas in the temporal lobes. Typical streak linear enhancement was seen after the injection of contrast media. In spite of the early administration of acyclovir, he developed severe neurological sequelae including mental retardation, double hemiplegia and epilepsy. Combination therapy with carbamazepine and nitrazepam had been administered, however, the convulsions were not controlled at 1 year and 4 months of age and finally he developed infantile spasms.
CT showed diffuse and bilateral brain lesion, cortical and deep central (thalamus and brain stem). A possibility was suggested that infantile spasms might follow HSE in infancy with these cerebral lesion.

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A case of multiple subdural empyema complicated by intracranial mycotic aneurysm is reported. The patient was a 13-year-old boy and had a past history of nasosinusitis. He sufferd from fever, headache and hemiplegia of the right side. A CT scan revealed multiple subdural empyema in the left cerebral longitudinal fissure and the anterio-temporal area. The effect of contrast enhancement was revealed at the subdural empyema. Subdural empyema was treated with massive antibiotics, and neurological symptoms and multiple subdural empyema was disappeared completely. But left carotid angiography demonstrated a 7×5×5 mm sized fusiform aneurysm at the peripheral branch of the left anterior cerebral artery. Repeated angiography showed the aneurysm of the same size. The operation of the aneurysm was carried out. The patient was discharged without neurological and mental deficit. The aneurysm was suspected to be mycotic of extravascular origin. This complication in subdural empyema has rarely been reported.

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We described a 5 year-old boy manifesting psychiatric symptoms which were prolonged during the course of encephalitis.
The patient developed psychiatric disturbances such as night delirium and mutism. During the course, consciousness remained clear without any abnormal neurological findings. The CSF examination showed mild pleocytosis and the EEG showed diffuse low voltage fast activity.
About 3 months later from onset, he developed first seizure. Subsequent CSF examination showed normal but the EFG showed diffuse high voltage slow activity. Then he was thought to be suffering from subacute encephalitis.
It could not be easy to diagnose such atypical encephalitis promptly at the early stage, because it must be distinguished from other types of psychiatric disorders; namely psychogenic reaction and functional psychosis, Thus, it was suggested that repeated examination of CSF and EEG may be helpful for the differential diagnosis.

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[in Japanese]

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[in Japanese]

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