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  • 1
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: We have analyzed free chiral amino acids (aspartate and serine) in the human frontal cortex at different ontogenic stages (from 14 weeks of gestation to 101 years of age) by HPLC with fluorometric detection after derivatization with N-tert-butyl-oxycarbonyl-l-cysteine and o-phthaldialdehyde. Exceptionally high levels of free d-aspartate and d-serine were demonstrated in the fetal cortex at gestational week 14. The ratios of d-aspartate and of d-serine to the total corresponding amino acids were also high, at 0.63 and 0.27, respectively. The concentration of d-aspartate dramatically decreased to a trace level by gestational week 41 and then remained very low during all postnatal stages. In contrast, the frontal tip contained persistently high levels of d-serine throughout embryonic and postnatal life, whereas the d-amino acid content in adolescents and aged individuals was about half of that in the fetuses. Because d-aspartate and d-serine are known to have selective actions at the NMDA-type excitatory amino acid receptor, the present data suggest that these d-amino acids might play a pivotal role in cerebral development and functions that are related to the NMDA receptor.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1920
    Keywords: CT ; Ehlers-Danlos syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Three patients with Ehlers-Danlos syndrome were reported. Unusual findings on computed tomography were seen in two of the three patients. One case showed peculiar and marked dilatation of the 4th ventricle, supracerebellar cistern and lateral ventricle. The other case presented disproportionate enlargement of the anterior horn of the lateral ventricle. These CT findings in the two patients suggest that developmental abnormalities may constitute a structural defect.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1920
    Keywords: Down's syndrome ; CT scanning ; morphometric change ; basal ganglia calcification ; preniature aging ; small posterior fossa
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report characteristic and morphometric changes of cranial computed tomography (CT) with increasing age in 56 patients with Down's syndrome aged from 0 month to 37 years. Patients were compared with 142 normal controls aged 0 to 59 years. Width of ventricles, Sylvian fissures, posterior fossa, pons and cisterna magna were measured on CT. The incidences of the cavum septi pellucidi, cavum vergae and cavum veli interpositi and high density in the basal ganglia were examined. There was high incidence (10.7%) of bilateral calcification of basal ganglia in Down's syndrome, although that of pineal body and choroid plexus calcification was similar in Down's syndrome and controls. Basal ganglia calcification is more frequently seen in young Down's syndrome and may be related to the premature aging characteristic of Down's syndrome. The CT in Down's syndrome showed relatively small posterior fossa, small cerebellum, small brain stem and relatively large Sylvian fissures in those under one year of age. There was a high frequency of midline cava and large cisterna magna. There were no significant atrophic changes on CT except after the fifth decade comparing with controls.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0533
    Keywords: Key words Hamartoma ; Immunohistochemistry ; Tumor suppressor
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have studied immunohistochemically the expression of tuberin, the protein product of the TSC2 gene, in cerebral, renal and cardiac tissues obtained from patients with tuberous sclerosis and from control patients. Tuberin immunoreactivity was moderate to strong in neurons and reactive astrocytes of control brains, but was reduced in brains with tuberous sclerosis. Staining intensity of abnormal giant cells varied from negative to moderate in cortical tubers, subependymal nodules and subependymal giant cell astrocytomas. In control kidneys, uriniferous and collecting tubules showed positive immunoreactivity, whereas a focal decrease in their staining intensity was noted in kidneys with tuberous sclerosis. Renal angiomyolipomas were negative for tuberin. In the heart, cardiac muscles in both control and tuberous sclerosis patients were strongly immunoreactive. Cardiac rhabdomyomas in the latter were stained less intensely. These results provide histological evidence for the loss of tuberin in tuberous sclerosis tissues, which is associated with the development of hamartomas in an organ-specific manner.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0568
    Keywords: Key words PAF acetylhydrolase ; Cerebellum ; Development
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  The 45K subunit of platelet-activating factor acetylhydrolase (PAFAH-45K) is the product of a candidate gene for Miller-Dieker lissencephaly. We studied the expression of this protein in the developing mouse cerebellar cortex by immunochemical and immunohistochemical methods. Western blotting studies indicated that PAFAH-45K is more abundant in the fetal than the postnatal period. Immunohistochemical studies revealed developmental changes in the localization of PAFAH-45K-immunoreactivity, which shifted from the somata of Purkinje cells to the neuropil of the molecular layer. Our findings indicate that PAFAH expression is developmentally regulated and suggest its role in histogenetic processes in the cerebellar cortex other than neuronal migration.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0533
    Keywords: Karyorrhexis ; Pontosubicular necrosis ; Perinatal brain damage ; Brain maturation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Pontosubicular neuronal necrosis is characterized by neuronal karyorrhexis, showing a peculiar distribution. In infants delivered at more than 29 gestational weeks (GW), neuronal karyorrhexis is restricted to the pons and subiculum, while in very premature infants (delivered at less than 28 GW), neurons in other brain regions, such as the inferior olivary nucleus, cerebellum, basal ganglia, thalamus and cerebral cortex, are also involved. Thus, karyorrhexis is more widely distributed in the more immature brain, implicating neuronal maturation as one of the pathogenetic factors relevant to this type of neuronal cell death.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 90 (1995), S. 400-402 
    ISSN: 1432-0533
    Keywords: Key words Leukomalacia ; Neonate ; Prematurity ; Cerebellum
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cystic necrosis in the cerebellar white matter was found in three premature infants. The necrosis was characteristically localized in the center of the white matter of the superficial cerebellar folia, sparing the overlying cortex. The patients were aged between 28 and 34 gestational weeks, and had a clinical history of severe systemic hypotension. Thus, cystic leukomalacia represents a characteristic brain lesion in premature infants which may be caused by cerebellar hypoperfusion.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-0533
    Keywords: Key words Microtubule-associated proteins ; Epilepsy ; Migration disorders ; Cortical dysplasia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report the enhanced expression of early forms of microtubule-associated proteins (MAPs) in cortical dysplasia in surgical resections from 17 children with intractable epilepsy. Large neurons, which represent one of the characteristic cellular features of cortical dysplasia, showed strong immunoreactivity for MAP1B, as well as the low-molecular-weight isoform of MAP2 (MAP2c). Insitu hybridization with MAP1B antisense riboprobe showed markedly increased hybridization signal intensities in the large neurons, whereas neurons in the normal-appearing cortex and most of the normal-sized neurons in the dysplastic cortex had faint signals. Because MAP2c and MAP1B are early forms of MAPs, which are abundantly expressed in the developing brain and down-regulated in the adult, and are thought to be involved in neuronal outgrowth and plasticity, our results suggest that the structural remodeling of neuronal processes is activated in cortical dysplasia.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 90 (1995), S. 400-402 
    ISSN: 1432-0533
    Keywords: Leukomalacia ; Neonate ; Prematurity ; Cerebellum
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cystic necrosis in the cerebellar white matter was found in three premature infants. The necrosis was characteristically localized in the center of the white matter of the superficial cerebellar folia, sparing the overlying cortex. The patients were aged between 28 and 34 gestational weeks, and had a clinical history of severe systemic hypotension. Thus, cystic leukomalacia represents a characteristic brain lesion in premature infants which may be caused by cerebellar hypoperfusion.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-0533
    Keywords: Key words Apolipoprotein-E ; Pontosubicular neuron ; necrosis ; Pyramidal cell ; Brain damage ; Karyorrhexis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract An apolipoprotein-E (Apo-E) immunohistochemical study was performed on neonates with pontosubicular neuron necrosis (PSN), aged 38–42 weeks of gestation, and compared to findings for age-matched neonates without PSN. Apo-E was expressed in neurons in both the pontine nuclei and pyramidal layer of the hippocampus, as well as astrocytes of only the PSN cases. The immunoreactive neurons did not exhibit karyorrhexis and were found in neonates by the age of 6 days. Apo-E may be produced by astrocytes and taken up by neurons on membrane remodeling during early responses to cerebral hypoxic or ischemic insult in PSN neonates.
    Type of Medium: Electronic Resource
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