The significance of cerebrospinal fluid (CSF) bilirubin levels on the outcome of neonatal jaundice has been investigated by correlations between kernicterus or brain damage and CSF bilirubin levels (Torii 1974, 1981, Meisels 1984), This study is designed to clarify the significance of CSF bilirubin levels in the longterm prognosis of neonatal hyperbilirubinemia.
Sixteen full-term newborn babies with hyperbilirubinemia (serum bilirubin level 20.0-30.0 mg/dι) who did not receive phototherapy because of low bilirubin in their spinal fluid (usually under 0.5 mg/dι in normal neonates) were followed in this study of growth, intelligence and neurobehavior. The subjects' neurobehavior was compared with that of a matched control group. The physical growth of the children with a history of hyperbilirubinemia was within normal limits except for one girl whose weight was less than-2 SD from the average. Verbal IQ's were lower than performance IQ's in 10 out of 14 children with a history of hyperbilirubinemia. However, no mental retardation was noted. No significant difference was found between the two groups in mean positive scores on the Garfield test. In the Prechtl test, mean score of the finger-touching test was significantly higher in the children with a history of hyperbilirubinemia than in the control group. The soft sign coefficient and the occurrence of attention deficit hyperactivity disorder were not significantly different in the two groups. In summary, a low bilirubin level (<0.5 mg/dι) in the spinal fluid seems to be a good prognostic sign in infants with neonatal bilirubinemia.

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To investigate the functional plasticity of the brain in childhood, the intelligence and language tests, brain CT and dichotic listening test were performed on 34 patients with hemiplegia. All 13 patients with left hemiplegia, 8 to 37 years old, showed a right ear dominance suggesting the lateralization of language in the left hemisphere. Among 21 patients with right hemiplegia, 7 to 22 years old, 16 patients who had suffered from the left hemispheric damage before 6 years and 1 month of age, showed the left ear dominance suggesting the lateralization of language in right hemisphere except 2 patients. On the contrary, 3 patients with right hemiplegia who had suffered from the left hemispheric damage after 6 years and 6 months of age showed a right ear dominance. It was concluded that the critical period for the dislodging of language lateralization in the brain from the damaged left hemisphere to the intact right hemisphere is the first half of 6 years after birth. Five patients out of 8 patients with right hemiplegia showed significantly lower scores in performance than in verbal WISC IQ. The compensation of language function rather than performance function was considered to be characteristic for the left-hemispheric damage in childhood.

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Electrically elicited blink reflex (BR) were analyzed in seven patients with age dependent epileptic encephalopathies (5 patients with infantile spasms and 2 with EIEE).
Four patients with infantile spasms showed prolonged latency of the late BR responses (R2). In 2 patients with EIEE showing suppression burst pattern on EEG, R2 was not detectable.
Rs abnormality in BR might reflect the dysfunction of the brainstem reticular formation in age dependent epileptic encephalopathies.

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We made 10 measurements of Pourcelot's index of resistance (RI) in the anterior cerebral artery (RI-ACA) and basilar artery (RI-BA), and RI ratio (=RI-ACA/RI-BA) in seven cases of hydrocephalus before and after treatment. The mean values of RI-ACA (0.831±0.050, mean ± SD), RI-BA (0.800 ± 0.053) and RI ratio (1.039 ± 0.030) before treatment were significantly higher than in normal infants. Two hours after treatment, the mean RI-ACA (0.654 ± 0.099) was lower than in normal infants, and RI-BA (0.684 ± 0.101) were normalized. Before treatment, the mean value of RI-ACA was higher than that of RI-BA. All RI ratios were higher than 1.00. After treatment, the mean RI-ACA was lower than the mean RI-BA. All RI ratios were normalized and lower than 1.00 (0.957±0.024).

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In order to evaluate pseudohypertrophy of the calves in Duchenne muscular dystrophy (DMD) quantitatively, we measured the circumference of the thigh, knee, calf, and ankle in total 7 patients with DMD whose ages ranged from 3 to 5 years, and 165 healthy boys aged 3 to 6 years.
Simple value of the calf circumference was not useful to evaluate pseudohypertrophy in DMD, because the circumference was variable following to the nutrition (Kaup index) in healthy boys. Various ratios were then tested for the calf circumference in relation to the thigh, knee, or ankle circumference. Of these, the calf/knee circumference ratio was the most informative; the values of 13 out of 14 affected legs were above the mean+2 SD.

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A total of 21 neonates with intraventricular hemorrhage (IVH) were studied; 12 (57.1%) survived, and 7 (58.3%) of these 12 cases suffered from hydrocephalus. Hydrocephalus occurred only in patients with IVH of grades III and IV on Papile's classification, and its occurrence rate was 87.5% as the sum total for thetwo groups. Destructive hydrocephalus (DHC) occurred in 5 cases of grade III and grade IV, and its occurrence rate was 62.5% as the sum total for the two groups.
It could be conjectured that the prognosis of the IVH depends upon the extent of the primary hemorrhagic lesions. However, in cases with DHC, the resistance of the cerebral parenchyma to the hydrodynamic force is decreased, so that secondary cerebral destruction yielding rapid and intense ventricular dilatation, which is reponsible for the development of neurological disorders, might occur more easily than in cases with an intact brain. Accordingly, attention should be paid to the primary cerebral lesions occurring in cases with IVH of grades III and IV, and to the secondary parenchymal destruction that may arise in association with hydrocephalus.
It is essential that a shunt operation should be performed as soon as possible after the diagnosis of DHC has been established so as to allow repair of the primary lesion and to minimize the secondary brain damage.

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Facilitation and recovery of the H-reflex were studied in 63 children with cerebral palsy, and the results were compared with those obtained for 60 normal children. H waves were recorded from the gastrocnemius with surface electrodes, after applying paired stimulations to the tibial nerve in the midpopliteal crease.
In normal children, both the facilitation and the recovery showed a tendency to decrease with their de-velopment, while those for children with cerebral palsy were more marked than normal children in all age groups.
The present study indicated that the excitability of spinal alpha motoneurons and the subliminal fringe in the motoneuron pool might change due to the maturation of and the damage to the central nervous system in children. We consider that H-reflex examination provides useful information for the supraspinal control to the spinal mechanism to regulate muscle contraction in children as well as in adults.

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The mechanism of periodic lateralized epileptiform discharges (PLEDs) still remains unclear, although it has been the subject of a number of theories. We investigated the relationship between the level consciousness and PLEDs in order to clarify both the clinical significance and the mechanism of PLEDs.
We studied two neonates and two infants with acute organic lesions of the brain (cerebral infarction, meningoencephalitis, cerebral hemorrhage, acute infantile hemiplegia), of whom all showing PLEDs in EEG. In each case, we analyzed sequential EEG records and the level of consciousness, and measured the periodicity, voltage and duration of PLEDs by a computer controlled digitizer.
In each case, the periodicity and voltage of the discharges were related to the level of consciousness. The appearance rate of PLEDs was relatively high at the level of consciousness from III-100 to 111-200. The inverse correlation between the frequency (1/periodicity) and the voltage of PLEDs was significant in 53% of the total records of PLEDs; it was also frequently observed at the level of consciousness between III-100 and III-200.
From above findings we concluded that an appropriate degree of damage to produce PLEDs is required in both the cerebral cortex and subcortical white matter corresponding to the level of consciousness from III-100 to 111-200.

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The clinical effect of mechanical ventilatory assistance with tracheotomy in respiratory failure of terminal phase muscular dystrophy was studied. The subjects were 6 Duchenne muscular dystrophy cases and 1 Ullrich type congenital muscular dystrophy case. Duration of the longest survival case was 4 years and 5 months. General physical conditions, complications, ADL and muscular atrophy were examined. By ventiratory assistance respiratory failure improved, and the physical condition stabilized and took good progress. Arterial hemorrhage which is lethal complication was observed in 2 cases. Mechanical ventilatory assistance with tracheotomy is an effective symptomatic therapy for the improvement of respiratory failure that can be applied when life prolongation is wished for by the patients or their families.

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We reported an additional case of Arima'syndrome with slowly progressive renal insufficiency and epilepsy. The patient is a 20-year-old man whose parents are consanguineous. He had a history of mild asphyxia at birth, and unexplained tachypnea developed during the neonatal period. But it disappeared later, and he have had no respiratory problem since then. Physical examination on admission at the age of 19 years revealed bilateral blephaloptosis, narrow palate, searching nystagmoid movement, absence of light reflex, muscle hypotonia and wasting of extremities. Funduscopic study showed optic hypoplasia, choroid coloboma and narrowing of vessels.
Head CT scan showed agenesis of cerebellar vermis and hypoplasia of brainstem. CT scan and echography of the kidney disclosed the bilateral multiple cysts. Liver was hyperechoic in echographic study this finding is consistent with fatty change. EEG showed dysrhythmic slow wave activity with sporadic spike and wave complex.
Compared with previously reported cases, the present case has the following features:(1) slowly progressive renal insufficiency, (2) generalized tonic cloniconvulsion developing from the age of 11 months, (3) ABR abnormalities including the right-sided shortening of wave I-II interpeak latency and bilateral ill-defined wave V.
Slow progress of renal failure in our case may reflect the mild pathological process of the kidney with sparing functional nephrons. It shows the diversity of the kidney pathology in Arima's syndrome. Epilepsy is a less common association in the syndrome, whereas EEG abnormalities were reported. ABR abnormalities may reflect the morphological alteration of the brainstem structure including auditory pathway. In our case it is uncertain whether the neonatal tachypnea was due to birth asphyxia or brainstemalformation responsible for abnormal respiration as suggested in Joubert's syndrome.

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Single photon emission tomography (SPECT) using Tc-99m-d, 1-hexarnethylpropylenamine oxime (HMPAO) was performed in two children with epileptic disorders during both sleeping and waking diurnal stages. Immediate postictal and interictal SPECT scans were obtained for a child with partial epilepsy. They demonstrated an interictal decrease in the regional cerebral blood flow (rCBF) and a more remarkable immediately postictal decrease in rCBF. The focus was in the same region of the SPECT for both interictal and postictal spikes. Another patient was diagnosed as having continuous spike-waves during slow sleep without epileptic seizures. Although SPECT during wakefulness showed no asymmetry, SPECT during sleep revealed decreased rCBF at the same location as the predominant area of diffuse epileptic discharges. We conclude that HMPAO SPECT is useful in investigation of the etiological mechanisms of seizures and epileptic discharges in epileptic disorders in childhood.

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A female patient who had clinical characteristics of MELAS but with no apparent muscle symptoms was reported. She was in good health until 12 years and 5 months of age when she began to have afebrile generalized tonic-clonic convulsions.
Thereafter, she had repeated stroke-like episodes, including headache, vomiting, convulsions, hemiparesis and left ehemianopsia. She had neither muscle weakness, fatigability nor atrophy. Laboratory examinations disclosed elevated lactate and pyruvate levels in the serum and cerebrospinal fluids, transient focal low density areas on brain CT and right sensorineural deafness by audiometry. No ragged-red fibers (RRF) were found in the first biopsy at 13 years and 6 months of age, and two RRF-like fibers containing red granular materials in the subsarcolemnal regions in the second at 15 years and 3 months of age.
A biochemical assay on the two biopsied muscles demonstrated normal enzyme activities in the mitochondrial electron transport system. She was diagnosed as having MELAS because of remarkable mitochondrial abnormalities in smooth muscle cells in the intramuscular arterioles which were clearly demonstrated by succinic dehydrogenase (SDH) stain and on electron microscopy.
It was suggested that the stroke-like episodes in this patient were induced by a preferential damage to the mitochondria in the blood vessel walls. Thus, we conclude that a simple method of identifying the strongly SDH-reactive blood vessels (SSV) in frozen sections is critical in supporting or making diagnosis of MELAS.

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A 12-year-old girl with multiple sclerosis (MS) with periodic synchronous discharge (PSD) on electroencephalogram (EEG) is reported. The patient developed clonic seizure of both arms at the age of 10. The muscle strength of left hand and both legs were decreased and her school records were declined at the age of 12. On neurological examination, the patient showed mild intellectual disturbance, mild weakness of face and extremities and bilateral decreased DTRs. Her gait was slightly ataxic.
Cerebrospinal fluid (CSF) revealed an oligoclonal band. Serial CT scans disclosed ring enhancements in the regions corresponding to clinical symptoms (right central gyrus on Sep.'85, right lower and middle temporal gyri, and mesial site of occipital lobe on Oct.'85, right cerebellum on Jan.'86). The same lesions were visualized as a high signal intensity on T2 weight images and as a low signal intensity on Ti weight images on MRI.
The attack of seizure occurs more frequently in children with than adults with MS. However, as far as we know in the cases of children with MS, there were no reports of PSD which was usually seen in the cases of subacute sclerosing panencephalitis. The pathomechanism of PSD is speculated to be suffered from subcortical damage.

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A seven-month-old girl with atypical West syndrome with pedaling automatism was reported. She started to have early infantile epileptic encephalopathy with suppression-burst (EIEE) at age 14 days followed by infantile spasms at 3 months of age. She began to have spasms with automatism at 4 month, mainly consisting of pedaling movements of both feet associated with tonic spasms of upper extremities and writhing of trunk. Ictal EEG showed irregular slow waves with occasional spikes predominantly over the right hemisphere mixed with artifacts. Interictal EEG during sleep showed suppression-burst patterns. Cerebral atrophy and microcephaly were revealed on brain CT, immature myelination on MRI and decreased blood flow in the frontal, temporal, and parietal lobes on the right on PET scan. Pedaling automatism is rare in infancy and its clinical significance was discussed in relation to West syndrome and complex partial seizures.

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[in Japanese]

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[in Japanese]

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[in Japanese]

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