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Volume 22, Issue 6
Displaying 1-13 of 13 articles from this issue
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[in Japanese]1990 Volume 22 Issue 6 Pages 538
Published: November 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSDownload PDF (145K) -
Hisaharu Suzuki1990 Volume 22 Issue 6 Pages 539-545
Published: November 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSI identifed forty-three patients with severe retardation aged 6-14 years in a defined community. More than half of the patients (23/43) had spastic tetraplegia and 9 had spastic diplegia. Only one case showed abnormal increase of muscle tone of dyskinetic type. The period when they had got underlying causes of their handicaps was as follows: congenital (acquired intrauterine, including intractable cases) 25, perinatal 6, and after the neonatal period 12. An increased proportion of congenital cases and a decreased proportion of perinatal cases are noted when the data were compared with previous reports. About a half of the total cases could be considered to be caused by preventable diseases such as perinatal anoxia, purulent meningitis, or intracranial hemorrhage due to vitamin K deficiencyView full abstractDownload PDF (6207K) -
Yoshihide Iwakawa, Masayuki Shimohira, Seiji Kondo, Yoshiaki Sato1990 Volume 22 Issue 6 Pages 546-550
Published: November 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSNeurophysiological studies were performed on 8 patients with group A xeroderma pigmentosum during early childhood. EEG, ABR and NCV were normal during this period. In contrast, various sleep parameters detected by polysomnography showed abnormal findings even in the neurologically normal patient. Decreased % sleep REM was seen in a case, and decreased frequency of REMs were seen in another. Body movements were extremely high or low in frequency in 3 cases in whole night sleep. The distribution of body movements were abnormal; in control subjects, the frequency was higher in SREM and stage 1 than in slow wave sleep; in 7 cases, it was higher in slow wave sleep than in stage 1 or 2, or body movements were extremely frequent.
Neurological examination revealed soft signs in various systems in early childhood. All cases except one showed hypotonia. Many cases were slow in learning to walk and the gait was unstable. Speech delay and decreased deep tendon reflexes, especially of patella, were seen in most cases. Since the neural deficits in XP may be related to the DNA repair defect, these findings indicate the possibility that some endogenous compoundsdistributing all over the nervous system might produce the DNA damages.
Because the body movements during sleep are controlled by the nigrostriatal dopaminergic system, present data indicate that the basal ganglia might be one of the earliest degenerative systems in the CNS.
Recently, some studies have suggested the possibility that oxygen radical mechanisms might be involved in the development of the dopamine neurodegenerative process in Parkinson's disease. Thus, we propose that the oxygen radicals are one of the possible factors causing the neural deficits in XP, since they are produced in any tissues including nervous systems and can damage the DNA of the nuclei under some conditionsView full abstractDownload PDF (562K) -
Kei Shioda1990 Volume 22 Issue 6 Pages 551-559
Published: November 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSNeuropathological examinations were performed on 30 autopsy cases of severely handicapped children. Among them, 11 cases showed bilateral cerebral destructive lesions. The cerebral lesions were divided into three groups; six cases with dominantly grey matter lesions, three with dominantly white matter lesions and two with combined grey and white matter lesions. The cortical lesions were found in the fronto-parietooccipital lobes and cingulate gyri, while undersurface of the temporal lobes showed less destruction. The white matter lesions, consisting of marked gliosis and atrophy accompanied by ventricular dilatation, were remarkable in the area extending from the periventricular region to the centrum semiovale. These changes were more apparent in the occipital lobes.
Cerebellar lesions were found in nine cases, which also were classified into grey matter and white matter lesions. The extent and characteristics of these lesions resembled those of the cerebral lesions. The basal ganglia showed no remarkable destruction in the cases with severe cortical and subcortical damages. It was assumed that these nuclei had survived the disconnection from the cortex.
Thalamic lesions were observed in six cases, mainly restricted to the dorsal and/or lateral nuclei. No relationship was found between these thalamic lesions and the extent or intensity of cerebral destruction. It is suggested that each of the thalamic nuclei has a different characteristic vulnerability to such destructive conditions.View full abstractDownload PDF (6922K) -
I. Copper Contents and Copper Dependent Enzyme Activities in Various OrgansSatoshi Tsurui, Hideo Sugie1990 Volume 22 Issue 6 Pages 560-565
Published: November 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSThe wet weight, copper content, mitochondrial electron-transfer complexes and 2', 3'-cyclic nuculeotide 3'-phosphohydrolase (CNPase) were measured in various organs including brain, liver, kidney, and heart in macular mutant mice which ara considered to be an appropriate model for human Menkes kinky hair disease (MKHD).
Copper contents were decreased markedly in liver, brain, and heart. However a significant increase was noted in kidney, suggesting a disproportionate distribution of copper contents in each organ in this mutant mouse. Regarding mitochondrial electron-transfer complexes, only cytochrome c oxidase, a copper dependent enzyme, was found to be decreased in heart and brain. This alteration in the brain was already demonstrated at 2 days. CNPase was not decreased in its activity at 7 days, but decreased at 14 days, supporting progressive demyelination. These results suggested that this mutant mouse would be a useful animal model for clarifing the pathogenesis in human MKHD.View full abstractDownload PDF (707K) -
II. Analysis of Brain Metabolism Using 31P-and1H-Nuclear Magnetic Resonance SpectroscopySatoshi Tsurui, Hideo Sugie1990 Volume 22 Issue 6 Pages 566-572
Published: November 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSWe studied the brain metabolism in macular mutant mice (Ml/y, +/y), an appropriate model of Menkes kinky hair disease, using 31P-and 1H-NMR spectroscopy to clarify the pathophysiological mechanism of disturbed nervous function.
An analysis of in vivo 31P-NMR spectra showed a decreased phosphocreatine (PCr)/inorganic phosphate (Pi) ratio and decreased ATP levels and decreased intracellular pH in Ml/y mice at 9 days, suggesting energy failure in the brain. This associated decline in ATP levels may reflect multiple causative factors including disturbed mitochondrial respiration and ischemia secondary to circulatory failure.
Brain metabolites, including PCr, creatine, lactate and 7 amino acids were easily detectable quantitatively and qualitatively by in vitro 1H-NMR spectrum. An elevation in lactate levels and a decline in PCr/creatine ratio in Ml/y mice at 9 days were also noted with an in vitro study, supporting the in vivo data.
NMR spectroscopy is a useful and promising tool to obtain the information on brain metabolismView full abstractDownload PDF (766K) -
I. Developmental Changes of Normal BabiesKeiko Koterazawa, Soichi Kodama, Hajime Nakamura1990 Volume 22 Issue 6 Pages 573-581
Published: November 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSPower spectral analysis of the EEG at the neonatal period was performed in 50 premature babies and 36 mature babies. The gestational age ranged from 26 to 40 weeks, and birth weight from 762 g to 3, 232g. All EEGs were recorded when the babies reached to 37-41 weeks of conceptional age, and analyzed using fast Fourier transformation. The relative power in active sleep was evaluated in this study.
The percent power of the delta 1 band ranging from 0.5 to 2.0 Hz decreased immediately with increasing conceptional age, while that of the delta 2 (2.2-3.8 Hz), theta 1 (4.0-5.4 Hz) and theta 2 (5.6-7.8) increased with conceptional age. However, no significant change was recognized in the alpha (8.0-12.8 Hz) and beta (13.0-29.8 Hz). These results suggest that the development of the EEG depends on the post conceptional age.
The percent power of delta 1 to beta bands was compared among the frontal, central, parietal and occipital regions. The development of EEG in the central region was prominant among other 3 regions in EEG development. The EEGs of premature babies were compared to those of mature babies at the same conceptional age.
The percent power of premature babies was higher at the delta 1 band, whereas lower at the delta 2, theta 1 and theta 2 bands than that of mature babies. The data showed that premature babies were more immature than age-matched mature babies in the development of EEGs.View full abstractDownload PDF (797K) -
II. The Power Spectral Changes of the Infants with Neonatal AsphyxiaKeiko Koterazawa, Soichi Kodama, Hajime Nakamura1990 Volume 22 Issue 6 Pages 582-588
Published: November 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSPower spectral analysis of the EEG at the neonatal period was performed in 33 babies with an Apgar score of 6 or less 1 minute after delivery. Their gestational ages ranged from 27 to 40 weeks, and birth weight from 1, 013g to 4, 416g. They were followed up until 18 months of age. All EEGs were recorded when the babies reached to 37-41 weeks of conceptional age, and analyzed using fast Fourier transformation. The percent power in active sleep was evaluated in this study.
The abnormal EEG patterns in infants with neurological damage were classified into the following groups;(1) Increase of the percent power of the delta 1 band, (2) Decrease of the percent power of the deha 1. On the other hand, the EEG patterns at the neonatal period in infants with normal development who suffered from neonatal asphyxia were similar to those of age-matched controls. These results suggest one of the most important factors deciding the prognosis of the brain damage is the presence of a normal percent pattern.
In conclusion, power spectral analysis of EEG at the neonatal period is useful in predicting the outcome of brain damage if it is used with visual inspection.View full abstractDownload PDF (776K) -
Clinical Features of the Propositus and Comparison with the Previously Reported CasesShuji Wakai, Nobutada Tachi, Yukitoshi Ishikawa, Minoru Okabe, Ryoji M ...1990 Volume 22 Issue 6 Pages 589-595
Published: November 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA patient with benign familial neonatal convulsions was presented. The patient had the first episode of cyanosis on the second day of life. Thereafter, he also experienced focal clonic and/or multifocal clonic seizures. The interictal EEG showed no definite abnormality. Between the seizures he appeared well and physi-cal examination was essentially normal. Treatment with phenobarbital (4 mg/kg/day, P. O.) was started and subsequently he had no further seizures until 3 months. At the age of 4 months, he was admitted to the hospital again because of generalized tonic-clonic seizures. The interictal EEG showed sporadic spikes dominantly in the right central area. The findings of ictal EEG at that time are characterized by fast spiking of increasing amplitude during the tonic phase. During the clonic phase, there are repetive bursts of spikes and sharps mixed with persisting muscle potential. The termination of the convulsion is characterized by general voltage depression. Clinical characteristics such as seizure types, EEG findings, responses to antiepileptic drugs and recurrence of the seizures found in our propositus were compared with those of the patients previously reported in the literature.View full abstractDownload PDF (862K) -
Tatsuya Koeda, Ikuo Suganuma, Yoshiyasu Kono1990 Volume 22 Issue 6 Pages 596-601
Published: November 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSSeventeen children with clinical evidences of preterm birth and spastic diplegia (SD) were studied about the relationship between clinical severity and laterality of cerebral function. The result showed the patients with the left-handed SD were more serious than those with the right-handed in their intelligence and activity of daily living. As the severity increased, motor difficulty was also observed at upper limbs, and especially spasticity was detected at the right hand. The dichotic listening test showed the right ear dominace only in the patients with the right-handed SD. MRI examinationw as performedi n 10 cases, a nd periventricular leukomalasia (PVL) was detected in all cases. There was few correlation between PVL and clinical laterality. We speculated that right-hand spasticity which was seen in severe cases was due to the fragility of periventriculars tructure in the left hemisphereView full abstractDownload PDF (3320K) -
Yumi Arai, Junichi Satoh, Yoshio Morimatsu, Masaharu Hayashi, Masako S ...1990 Volume 22 Issue 6 Pages 602-607
Published: November 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESSA woman, w ho had been developementallyd elayed since her birth, further deteriorateda fter she got an episode of high fever of unknown origin at 2 years of age. At the age of 52 years, she died of liver cancer after a long-standing HB virus carrier state. Neuropathologicael xaminationr evealedb rain atrophy, narrowed white matter, myelin pallor, fibrillary gliosis and status marmoratus of the thalamus. These findings suggested the primary cause of the brain pathology in this case to be a developmental destructive process which shouldh ave taken place in the early stage of brain development.I n addition, n eurofibrillaryt angles (NFT) were noticed in the hippocampus, l ocus ceruleus and nucleusb asalis of Meynert, b ut no senile plaques were found anywhere. These NFT changes seemed to be closely resembling to those of the Fukuyama type congenital muscular dystrophy. But the reason why NFT occur in this case is still obscure and probably different from that in the Down syndromeo r in physiologicals enilityView full abstractDownload PDF (3247K) -
[in Japanese], [in Japanese]1990 Volume 22 Issue 6 Pages 609-610
Published: November 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS -
[in Japanese], [in Japanese], [in Japanese], [in Japanese], [in Japane ...1990 Volume 22 Issue 6 Pages 610-612
Published: November 01, 1990
Released on J-STAGE: August 10, 2011
JOURNAL FREE ACCESS
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