A follow up study was performed on 41 neonates with intracranial hemorrhage confirmed by CT during the 5-year period between 1979-1983. Intraventricular hemorrhage (IVH) was noted in 17 cases and subarachnoid or subdural hemorrhage (SAH/ADH) in 24 cases. Epileptic seizures occurred in 6 of IVH cases (35%); generalised tonic-clonic seizures in 2 cases, partial seizures evolving to secondary generalised seizures in 3 and infantile spasms in one. Nine IVH cases showed abnormal EEG; six showed spikes in the central-parietal area and one had hypsarhythmia. Only one SAH/SDH patient developed recurrent convulsions with an abnormal EEG after discharge from the hospital. It was concluded that SAH/SDH had a good prognosis for epileptic seizures.

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The aim of this study was to analyze the changes seen on cranial computed tomography (CT) of epileptic children, especially in the area of the temporal horn.
The subjects were 242 epileptic children excluding those with encephalitis, brain tumor, neurocutaneous syndromes, degenerative disease, hydrocephalus etc. The control subjects were 195 children without any neurological disease and symptoms. CT scan were taken with a TCT-60A whole body scanner, and 14 check points were evaluated excluding the temporal horn. 195 epileptic children (N-group) and all control children were normal at 14 check points. Next, the areas of the temporal horns and adjoining hemispheres of the epileptic children (N-group) and control children were examined with Muto-Tablet-Desitizer.
The temporal horn ratio ((area of temporal horn/area of ipsilateral hemisphere)×100) was greater in younger children of the control group, and it was higher in epileptic than in control children. Enlargement of the temporal horn was seen in 1% of the controls and in 35% of the 125 epileptic children with normal measurements at 14 points on CT scans (p<0.01). The frequency of enlargement of temporal horns was not variable among different epileptic types. In the epileptic children with normal CT scans except for enlargement of temporal horns behavioral disturbances were 6 boys and 5 had enlarged temporal horns (bilateral 1 case, left side 1 case, right side 3 cases).

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Pyruvate dehydrogenase complex (PDH complex), which is deficient in some patients with congenital lactic acidosis, exists in two interconvertible forms: it is inactive in the phosphorylated form and active in the dephosphorylated form. We measured the native and total activities of PDH complex in biopsied muscles and cultured skin fibroblasts from patients with lactic acidosis and controls using a protein phosphatase with broad specificity. The diagnosis of partial PDH complex deficiency could be established by measuring the native or total activity of PDH complex in cultured skin fibroblasts. However the total activity of PDH complex in biopsied muscles must be determined to confirm the diagnosis of partial PDH complex deficiency, because the native activity of PDH complex in muscle might be extremely changed in various metabolic conditions.

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Serial X-ray CT and MRI were performed on 2 cases of Tay-Sachs disease.
Case 1: a 3-year-10-month-old girl. Her developmental milestones were normal until the age of 6 months. At the age of 10 months, hypotonia and unduly sensitiveness to sounds were noticed. She had cherry red spots in both fundi, and the serum hexosaminidase A activity was low. Significant clinical features during the next 3 years included regression of developmental milestones to the level of one month, and myoclonic and generalised seizures. After 3 years she showed megalencephaly, unstable body temperature, and respiratory disorders.
Case 2: a 2-year-6-month-old boy. His developmental milestones were normal until the age of 5 months. Then he manifested hyperreaction to noises and further developments ceased. He started to deteriorate from the age of 1 year and 2 months, and had no head control at the age of 1 year and 6 months. Cherry red spots were detected and the serum hexsosaminidase A activity was low, too.
X-ray CT at the early stage of cases 1 and 2 showed mild cerebral atrophy and high density areas in bilateral thalami and basal ganglia. At the late stage of Case 1, high density areas appeared in occipital and frontal white matters. MRI in both patients were strikingly similar. In their grey matters, linear light areas were demonstrated on IR images. Their white matters showed extensive dark (long Ti) areas on IR images, and extensive light (long T2) areas on SE images. Bilateral thalami and basal ganglia were light on IR images and dark on SE images. The findings of MRI in our patients seemed to correlate well with pathological and biochemical changes in their brains.

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C-reactive protein (CRP; latex agglutination method), GOT and LDH were determined in CSF of 380 patients admitted to St. Mary's Hospital from 1980 to 1983. CRP was positive in all patients with septic meningitis and negative in all patients with aseptic meningitis. There were significant differences in the value of GOT and LDH between septic and aseptic meningitic patients from 30 days to 1 year of age; both GOT and LDH were significantly higher in septic meningitis (GOT: p<0.01, LDH: p<0.05). It was concluded that those tests were reliable for the diagnosis of septic meningitis in infants.

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It is well known that steroid hormones and ACTH improve acute exacerabations of inflammatory demyelinating diseases. In our study a high dose methylprednisolone therapy (pulse therapy) was performed to two patients with multiple sclerosis (MS) and one patient with acute disseminated encephalomyelitis (ADEM). Clinical symptoms tended to improve within a few days, slow waves in EEG were normalized in several months, ringed enhancement in brain CT disappeared in patients with MS. We observed no major adverse effects.
It was concluded that the pulse therapy was effective and safe for inflammatory demylinating diseases in children.

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“Shuffling babies”(SB), who show propulsion in a sitting position as a form of prewalking locomotion, are well known as a normal variation of motor development. However, there are only a few epidemiological studies of SB. We report here on a regional study of SB in Fukuoka City using the opportunity of health check-up at 18 months of age.
The subjects were 1, 663 18-month-old children who lived in Chuo-Ku, Fukuoka City and had been born in the period between August 1983 and July 1984. We picked up SB according to the motor development questionnaire distributed just before health check-up at 18 months of age. SB were examined at Fukuoka Chuo Public Health Center where neurological findings, Enjoji development screening test and detailed history were taken into account.
Forty-five SB, 21 males and 24 females, were found in 1, 470 recovered questionnaires, so that the incidence of SB was 3.1%. A family history of shuffling was seen in 38% of SB. SB were divided into 2 groups according to the type of motor development. Group 1 (the core group of SB) included 12 babies whose motor development in a prone position and bearing weight on the lower limbs were delayed. Group 2 (the fringe group of SB), which showed normal motor development in a prone position and/or bore weight on the lower limbs by 10 months of age, comprised 33 babies. Comparing the two groups, the duration of shuffling was shorter and walking without support was achieved significantly earlier in group 2 (the age of the first walking was 15.0±1.9 months in group 1 and 12.5±1.9 months in group 2). Hypotonia was more frequently seen in group 1. Twenty-three SB who visited Fukuoka Children's Hospital almost all belonged to group 1 and they showed more delayed walking (18.8±3.3 months). Therefore, it was evident that the motor development of SB showed a wide spectrum. Finally, we speculated on the possibility of environmental factors in the occurrence of SB, because SB occurred more frequently in those born in or around summer time (from May to September).

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9-year-old boy with adrenoleukodystrophy (ALD) was diagnosed as having alpha-coma because of his alpha-like pattern in EEG and his comatose state due to phenobarbital intoxication. These alpha-wave were considered to be abnormally generated rhythms, because the normal alpha-rhythms had been absent in the previous EEG of this ALD patient. The spectrum analysis of alpha-waves showed abnormally high coherence values in the frontal, central, and midtemporal areas in relation to the ipsilateral occipital area of this patient as compared with those of normal children.
His auditory evoked brain stem response and the electrically elicited blink reflex showed the almost normal latencies. The short somatosensory evoked potentials revealed the prolonged latency and the low ampli-tude of N₂₀. In the visual evoked potentials, the normal waves of AT was not found.
These electrophysiological examination suggest that the abnormal alpha waves are delivered from a single lesion and projected diffusely to the scalp surface in our patient who showed dysfunction of the cerebral white matter pathways and the almost normal function of the brain stem.

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Two learning disabled boys who had normal intelligence quotients in Wechsler intelligence scale for children were reported. They showed severe deficits in reading and writing skills without dyscalculia. Neuropsychologically, deficits were suggested to be in auditory-linguistic skills in Case 1 and in connection of visuo-audiotory perceptions in Case 2. The right hemisphere was language dominant in Case 1 and the left in Case 2, determined by their handedness and the dichotic listening test.
Both cases showed poor formation in late components of visual evoked potential in their language dominant hemispheres. These suggest that our two cases may cause from some dysfunctions in the language dominant hemisphere.

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Hereditary progressive dystonia (HPD), first reported by Segawa et al. in 1971, represents a disease manifesting dystonic posture and movement with marked diurnal fluctuation. In this report, we described a sporadic case of HPD treated effectively with L-DOPA, and investigated the alterations of catecholamines (CA), serotonin, and their metabolites in plasma, urine and CSF.
The patient was a 9 year-old girl. On admission, she had an abnormal posture and gait disturbance caused by dystonia. Her symptoms were mild in the early morning and worsened gradually toward the evening. We suspected HPD and examined the metabolic changes of CA before and after L-DOPA therapy.
Prior to therapy, although the CSF dopamine (DA) level decreased to one third in the evening compared with that in the early morning, both were within normal values. The CSF HVA levels were significantly low both in the early morning and in the evening. After therapy, the CSF DA level increased remarkably. Similarly, the CSF HVA and DOPAC increased, but the CSF 5-HIAA decreased.
It was concluded that the etiology of HPD and its diurnal fluctuation were not always attributable to the quantitative decrease of DA in the central nervous system.

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Heterotopic gray matter was found by magnetic resonance imaging on a 3-year-old girl with left hemiparesis and atonic seizures. In the inversion recovery sequence, a large area of decreased signal intensity was noted in the right centrum semiovale and the differentiation of right basal ganglia was not clear.
We speculated that the brain malformation of our patient occured at ten weeks of conceptional age.

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Cerebrovascular “moyamoya” disease has been well known to occur among the Japanese, and over 1, 500 cases are registered in Japanese research committee up to the present. According to these large amounts of data, the involuntary movements are listed at the bottom as the initial symptoms. The incidence of 2.6% is not small considering its large background numbers. However, the details of involuntary movements have not been well documented.
We experienced an 8-year-old boy of “moyamoya” disease who presented with choreo-athetotic involuntary movements without vascular episodes. He was admitted to our service for the evaluation of involuntary movements that occurred 7 weeks before, after catching a common cold. His family history was unremarkable and his past history showed atopic constitution and several attacks of bronchial asthma. The neurological examination on admission revealed mildly low intellectual achievements, slight generalized hypotonia and generalized involuntary movements which were choreo-athetotic in nature and seen over face, trunk and all extremities. CT scan and MRI showed ischemic lesions over bilateral Corpora striata and their surrounding structures. The angiographical findings were consistent with “moyamoya” disease.
These clinical and radiological findings suggested the ischemic lesions on bilateral Corpora striata and their surroundings, especially at the anterior limb of Corpora striata, which could be responsible for the occurrence of involuntary movements in this case.
We would like topropose that it is necessary to consider apossibility of “moyamoya” disease in the differential diagnosis of childhood involuntary movements.

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[in Japanese]

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