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  • Brain  (6)
  • Development  (3)
  • Biochemistry  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Reactions of vessel walls and brain parenchyma to the accumulation of Gaucher cells in the Norrbottnian type (type III) of Gaucher disease (1988)
    Springer
    Acta neuropathologica 75 (1988), S. 385-390 
    Details
    ISSN: 1432-0533
    Keywords: Gaucher disease ; Brain ; Immunohistochemistry ; Macrophages ; Astrocytes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Splenectomy in children with the Norrbottnian type of Gaucher disease is followed by increased blood levels of glucosylceramide and imparied neurological and mental status. High blood levels are associated with an increased accumulation of glucosylceramide in perivascular Gaucher cells in the brain compared to non-splenectomised cases. Surrounding the Gaucher cell infiltrates there is loss of neurons and slight demyelinaton in the brain parenchyma. The brains of four cases with the Norrbottnian type of Gaucher disease were examined by immunohistochemical stains in an attempt to further characterize the perivascular Gaucher cells and to examine the reactions of the vessel walls and brain parenchyma to the accumulation of Gaucher cells. The perivascular storage cells showed granular staining with antibodies to muramidase and α1-antichymotrypsin confirming that they are blood-derived macrophages belonging to the monocyte-macrophage system. The Gaucher cells contained material positive for antisera to plasma proteins strongly suggesting that large molecules (including glucosylceramide) can escape from the blood and be taken up by the macrophages in Gaucher disease. The storage cells were surrounded by a reticulin network stained by antisera to collagen type III, type IV and laminin. The infiltrates were bounded from the brain parenchyma by a membrane strongly positive with antiserum for the basal lamina protein collagen type IV and laminin. The formation of a basal lamina around the Gaucher cell cuffs probably constitutes a protective phenomenon governing the brain parenchyma against the foreign cells. A focal loss of neurons but only minor loss of axons could be demonstrated with the antiserum to neurofilament. The brain parenchyma surrounding the Gaucher cell infiltrates showed marked astrogliosis in the anti-glial fibrillary acidic protein stain. In the two cases previously shown to have higher blood levels of glucosylceramide there were astrocytes positive for plasma proteins indicating passage of plasma proteins into the brain, this was not seen in the non-splenectomised cases. The additive effect of low-grade tissue damage in the vicinity of the Gaucher cell infiltrates is probably enough to explain the increased neurological symptoms and mental retardation following splenectomy in the Norrbottnian type of Gaucher disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687792
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  • 2
    Electronic Resource
    Electronic Resource
    Angioarchitectonics of rat cerebellar cortex during pre- and postnatal development (1980)
    Springer
    Acta neuropathologica 50 (1980), S. 131-138 
    Details
    ISSN: 1432-0533
    Keywords: Rat ; Cerebellum ; Vessels ; Development
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The adult arrangement and the development of stem vessels and capillaries was studied in the rat cerebellum. In principle, stem vessels branch and terminate at three levels: (1) the molecular layer, (2) the Purkinje cell-granular layer, and (3) the cerebellar white matter. All stem vessels are interconnected by the capillary network which is most dense in the Purkinje cell—granular layer. As in the neocortex, the stem vessels of the cerebellum are formed successively during development, so that the later they are formed the more superficial are their terminations. The formation of multiple stem vessels in the depths of fissures and sulci during both pre- and postnatal development may correlate to regional variations in, e.g., mitotic frequency or thickness of the external granular layer. The earliest “endo-parenchymal” branches are formed before the first neurons are present. Capillary growth by sprouting during the postnatal period parallels known regional differences in the timing of the neuronal maturation, e.g., increased synaptic density and oxidative metabolism. The findings in this investigation confirm and extend the results of an earlier morphometric study on capillary development in the cerebellar cortex. Although the angiogenetic factors remain unknown, the hypothesis of a link between the vascularization and the functional maturation of the brain is corroborated by the results. Knowledge of the normal vascular development seems necessary for an understanding of brain morphogenesis and for interpretation of primary pathogenetic mechanisms in various intoxications etc.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00692863
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  • 3
    Electronic Resource
    Electronic Resource
    Neuropathology of the Norrbottnian type of Gaucher disease (1984)
    Springer
    Acta neuropathologica 65 (1984), S. 99-109 
    Details
    ISSN: 1432-0533
    Keywords: Lipidosis ; Brain ; Human ; Gaucher disease ; Morphology ; Biochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The Norrbottnian type of Gaucher disease is characterized by infantile or juvenile onset and variable degrees of neurological symptoms, some of which develop only after splenectomy. A full neuropathological description of this type of Gaucher disease has not been reported previously. The brains of five patients were examined morphologically and biochemically. All presented typical accumulations of glucosylceramide storing cells in the adventitia of vessels in the cerebral and cerebellar sub-cortical white matter (s.c.w.m.). There were differences between the five cases with regard to the accumulation of adventitial storage cells and to the fatty acid pattern of the glucosylceramide isolated from the s.c.w.m., which implicate that the accumulation of glucosylceramide in adventitial cells in the brain is dependent on the generalized lipid storage process and enhanced by splenectomy. Loss of neurones and myelin was noted in the vicinity of accumulations of storage cells in two cases. The five cases whowed varying degrees of nerve cell loss, satellitosis and neuronophagia. Lipofuscin with simple and complex lipids but no glycolipids could be demonstrated in neurones light-microscopically. Utrastructural examination revealed inclusion bodies with bilayers in neurones of the cerebral and cerebellar cortex, dentate nucleus and pons. Because of the bilayered structure of Gaucher cell inclusions the bilayers in neurones are assumed to be formed by glucosylceramide. The fatty acid composition of glucosylceramide isolated from cerebral cortex in all cases suggested that cerebral gangliosides were its main precursor. The highest levels of psychosine (glucosylsphingosine) were seen in the cases with the most advanced nerve cell loss. The morphological and biochemical findings indicate that the neuronopathic process is associated with accumulation of glucosylceramide and psychosine in neurones.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00690463
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  • 4
    Electronic Resource
    Electronic Resource
    Low-dose lead encephalopathy in the suckling rat (1983)
    Springer
    Acta neuropathologica 60 (1983), S. 1-8 
    Details
    ISSN: 1432-0533
    Keywords: Lead intoxication ; Rat ; Growth development ; Lead determination ; Light microscopy ; Brain
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Retardation of growth has often confounded the interpretation of the results from experimental studies on lead intoxication. An attempt was therefore made to establish a daily dose of lead which, when given to suckling rags, results in a lead encephalopathy without concomitant reduction in body weight. Lead was administered i.p. as lead nitrate. Experimental animals were given 25, 10, or 5 mg lead nitrate/kg b.wt. daily during the first 20 days postnatally (p.n.). One group was given 10 mg/kg daily during the first 15 days. Controls were injected with vehicle without lead nitrate. Mortality was high in the group given 25 mg/kg b.wt. daily. Animals in this group exhibited a marked weight loss after 10 days. A slight but significant reduction in body weight was seen at 20 days in animals receiving 10 mg/kg b.wt. from day 1 to 20. The body weight gain of animals given 10 mg/kg during 15 days and of animals given 5 mg/kg during 20 days did not significantly differ from that of controls. Lead content in blood and brain was determined using a Carbon Rod Atomizer. Lead levels were elevated in all experimental animals. Light-microscopic findings in the cerebellum of animals given 25 and 10 mg/kg b.wt. daily were similar to those previously reported in experimental lead encephalopathy. The changes were dose-dependent, lesions being devastating in rats given 25 mg/kg b.wt. daily and discrete in rats given 10 mg/kg b.wt. daily. No pathologic change could be demonstrated on the light-microscopic level in the cerebellum or cerebrum of rats given 5 mg/kg b.wt. daily. The lack of growth retardation in encephalopathic rats makes the model valuable for further investigations on lead neurotoxicity.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00685340
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  • 5
    Electronic Resource
    Electronic Resource
    The early internal vascularization of the rat brain (1980)
    Springer
    Acta neuropathologica 50 (1980), S. 221-226 
    Details
    ISSN: 1432-0533
    Keywords: Rat ; Brain ; Vessels ; Prenatal ; Development ; Protein deprivation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The internal vascularization of the brain was studied in foetuses of normal and protein-deprived rats from embryonic day (E) 12 to 15. The position of vascular branches showed distinct relations to the various zones of the neuroepithelium. The possibility that various parts of the vascular system may differ in function, maturation, and morphogenetic relations to the neuroepithelium must be considered. The distinct vascular layers were therefore given names relating them to the respective wall zone. The ingrowth of straight stem vessels from the epiparenchymal vascular plexus into the neuroepithelium and the formation of vascular branches close to the ventricular system were referred to as stage I of the internal vascularization. The resulting plexus was called the deep vascular plexus of the ventricular zone. Its formation followed the same temporospatial gradients as the formation of the marginal zone. Following the formation of the intermediate zone, more stem vessels entered the neuroepithelium and a superficial vascular plexus of the ventricular zone was formed (stage II). This plexus was positioned close to the border between the ventricular zone and the intermediate zone. Subsequently, vascular branches also formed plexuses of the intermediate and subventricular zones (stage III). No “intraepithelial” vessels were seen on E 12. The temporospatial gradients in the telencephalic vesicles were caudal to rostral and lateral to medial, starting in the parts corresponding to the ganglionic eminence in the floor of the lateral ventricle on E 13. Only the dorsomedial angles of the hemispheres showed no vessels on E 15. No obvious differences were seen between the normal and the protein-deprived foetuses regarding the timing and extent of vascularization or the size and appearance of wall zones in the immature central nervous (I-CNS).
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00688758
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  • 6
    Electronic Resource
    Electronic Resource
    Vulnerability to lead in protein-deprived suckling rats (1984)
    Springer
    Acta neuropathologica 62 (1984), S. 276-283 
    Details
    ISSN: 1432-0533
    Keywords: Rat ; Brain ; Lead intoxication ; Protein deprivation ; Growth and development
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Most studies on lead toxicity in the suckling rat have been performed with doses leading to growth retardation. In a previous paper (Sundström et al. 1983), the effects of different lead doses on normal suckling rats were described. The dose of 10 mg/kg body weight daily given on days 1–15 pp produced minute hemorrhagic lesions on day 15 in the cerebellum, whereas rats given 5 mg/kg body weight daily lacked microscopically discernible pathologic changes in the brain. None of these groups exhibited growth retardation. To further elucidate the association between lead encephalopathy and malnutrition, lead was administered to protein-deprived suckling rats. Protein deprivation was achieved by a diet with 50% reduction of protein content. The móthers of the pups were fed this diet from 2 weeks before conception throughout the experiment. Experimental animals were injected i.p. with 5 mg or 10 mg lead nitrate/kg b.wt. daily. Littermates, injected with vehicle without lead nitrate served as controls. Protein-deprived rats without either treatment were “external” controls. Animals were killed at 10, 15, and 20 days age for determination of lead content in blood and brain and for light-microscopic examination. The protein-deprived rats given 10 mg/kg b.wt. daily were growth-retarded as compared to unexposed protein-deprived rats. The mortality was almost 100% at 15–20 days pp. At 15 days, the cerebellum of these rats showed abundant hemorrhages, and the cerebrum was also hemorrhagically discolored. Protein-deprived rats given 5 mg/kg b.wt. daily did not differ significantly from unexposed protein-deprived rats with regard to body weight gain. They presented a mortality of about 20% on days 15–20. At 15 days, cerebellar hemorrhages were a regular finding, though not as devastating as in the animals given 10 mg lead nitrate/kg b.wt. daily. The results imply an increased vulnerability to lead in protein-deprived rats as compared to normal rats. The severe encephalopathy in protein-deprived rats was associated with higher blood lead levels than in normal rats exposed to an equivalent lead burden.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687609
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  • 7
    Electronic Resource
    Electronic Resource
    The influence of protein deprivation during development on amino acid incorporation into cerebral proteins (1982)
    Springer
    Research in experimental medicine 180 (1982), S. 41-50 
    Details
    ISSN: 1433-8580
    Keywords: Protein deprivation ; Protein synthesis ; Growth ; Development ; Brain ; Liver ; Muscle
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The incorporation of3H-valine into protein of cerebrum, liver, and muscle was studied in rats fed a low protein diet. Animals, 15–50 days old, were used to cover the period during and after the most rapid phase of cerebral protein synthesis. The precursor was administered in doses resulting in brain concentrations of free valine in the 0.1–0.3 mM range. Two series of experiments were run and the injected dose varied for comparison of protein synthesis at different concentrations of precursor valine. By concomitant amino-acid analysis it was shown that the concentration of other amino-acids in the brain was not changed by the injected valine. The changes in the amino-acid pool with age in control rats were in agreement with previous findings. Only small alterations were seen in the amino acid pool as an effect of protein deprivation, the results being consistent with those of some previous reports. Uptake of valine into the brain appeared to be decreased only in 15–17-day-old proteindeprived rats. Valine incorporation into cerebral protein was decreased in the youngest age group but no effect was seen in older animals. Liver protein incorporation was slightly decreased by low protein diet but a marked reduction was observed for muscle protein. The changes in brain protein synthesis during normal development were in agreement with previous studies and differed qualitatively from those in liver and muscle.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01852231
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