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  • 1
    ISSN: 1520-5126
    Source: ACS Legacy Archives
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Key words Basilar artery ; Vasoconstriction ; Cyclosporine A ; Bone marrow transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report here the case of a 32-year-old woman who suffered from a unique angiopathy in the central nervous system (CNS). She died of multiple infarcts in the brain stem and cerebellum during treatment with cyclosporine A after bone marrow transplantation for refractory anemia with excess of blasts. The autopsy findings showed segmental narrowing of the basilar artery, in which circumferential dissection of the internal elastic lamina had occurred. The distal portion of the basilar artery was obstructed by upward dislocation of the dissected intima. Similar angiopathy was also observed at multiple sites along the basilar artery branches. These findings suggest endothelial damage, including vasoconstriction and dissection of the CNS arteries.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 99 (2000), S. 691-694 
    ISSN: 1432-0533
    Keywords: Key words Cattle ; Intracytoplasmic neuronal ¶inclusions ; Hyaline (colloid) inclusion in man
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report deals with the large intracytoplasmic hyaline inclusions observed in hippocampal large pyramidal cells of two Holstein-Friesian cattle. These inclusions were round to elongated polyhedral in shape with consistently homogeneous glassy appearance; they varied in size and were positive for the periodic acid-Schiff reaction and silver impregnation. Electron microscopic examination revealed that the inclusions consisted of granular materials showing moderate electron density and were bounded by a unit membrane. On the external surface of the unit membrane, there were direct connections to cellular organelles, including ribosome, rough endoplasmic reticulum, and mitochondria. These findings suggest that the inclusions might be derived from neuronal endoplasmic reticulum.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0533
    Keywords: Key words Dentatorubral-pallidoluysian atrophy ; Cerebellar dentate nucleus neuron ; Skein-like inclusion ; Polyglutamine ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have recently reported that, in addition to the widespread occurrence of ubiquitinated neuronal intranuclear inclusions (NIIs), the restricted occurrence of ubiquitinated intracytoplasmic filamentous inclusions in the neurons of the cerebellar dentate nucleus (CDN) is a characteristic feature of dentatorubral-pallidoluysian atrophy (DRPLA). Interestingly, these neuronal intracytoplasmic filamentous inclusions (NIFIs) were morphologically indistinguishable from the skein-like inclusions (SLIs) described previously in the spinal anterior horn cells in amyotrophic lateral sclerosis (ALS). In the present study, we examined immunohistochemically the CDN in ten patients with clinicopathologically and genetically confirmed DRPLA and the spinal anterior horns in five patients with sporadic ALS, using a monoclonal antibody (1C2) directed against long polyglutamine stretches. In all of the patients with DRPLA, both the NIFIs and the NIIs were visualized clearly with 1C2. Conversely, in the patients with ALS all structures, including the SLIs, were completely negative. These findings indicate that in DRPLA, the NIFIs in the CDN are an alteration that is directly related to the causative gene abnormality (an expanded CAG repeat encoding polyglutamine) and that, from the molecular point of view, they are distinct from the SLIs in ALS.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0533
    Keywords: Acute porphyria ; Porphyric neuropathy ; Axonal degeneration ; Uroporhyrinogen I synthetase ; Ferrochelatase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An autopsy case of a 37-year-old woman with acute porphyria is reported. The patient began to complain of severe menstrual pains, and later developed serious peripheral neuropathy and various autonomic nervous symptoms. The autopsy revealed a marked loss and degeneration of axons and myelin sheaths in the peripheral nervous system (PNS), and prominent central chromatolysis of the spinal anterior horn cells. The predominant process of the peripheral neuropathy appeared to be axonal degeneration. Biochemical analysis showed a marked increase of delta-aminolevulinic acid (ALA), porphobilinogen, uroporphyrin, and coproporphyrin in the urine, and an increase of coproporphyrin and protoporphyrin in the stools and blood. In the analysis of the enzymatic activities of the liver and bone narrow, the activity of ALA synthetase (ALA-S) was markedly increased, and the activities of both uroporphyrinogen I synthetase (URO-S) and ferrochelatase were decreased. It was characteristic in this case that the enzymatic abnormalities found in both acute intermittent porphyria (AIP) and variegate porphyria (VP) coexisted. Biochemical analysis of the sciatic nerve showed an increase of ALA-S activity and a decrease of both URO-S and ALA dehydrase activities. This was the first report that indicated the presence of abnormal activities of the heme biosynthetic enzymes in the peripheral nerves of porphyric patients. The possibility was discussed that these enzymatic abnormalities of the heme biosynthesis in the peripheral nerve itself might be strongly related to the pathogenesis of the porphyric neuropathy.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0533
    Keywords: Chronic manganese poisoning ; Pallidal lesion ; Distribution of manganese
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An autopsy case of a 52-year-old man suffering from chronic manganese poisoning (CMP) is reported with determination of the manganese distribution in the brain. The patient had been working in a manganese ore crushing plant since 1965. In 1967 he began to complain of difficulties in walking and diminished libido. Later, he developed various neuro-psychiatric symptoms including euphoria, emotional incontinence, masked face, monotonous speech, “cock-walk”, increased muscle tone, weakness of upper and lower extremities, tremor of the eye lids, and exaggeration of knee jerks. The major neuro-pathological change was degeneration of the basal ganglia, in which the pallidum was severely affected. The pallidum discolsed a loss and degeneration of nerve cells, which was especially marked in the medial segment, a prominent decrease of myelinated fibers, and moderate astrocytic proliferation. The substantia nigra was intact. Distribution of manganese in the brain of the present case of CMP was determined using flameless atomic absorption spectrometry and compared with control cases and also a case of Parkinson's disease (PD). There was no significant difference between the control cases and the case of PD in average concentration of manganese and its ditribution in the brain. The present case of CMP showed no elevation in average concentration of manganese in the brain. However, there were some changes in its distribution. Thus, the continuance of neurological disorders in CMP is not linked to an elevated manganese concentration itself in the brain. CMP appears to be different from PD in neuropathology and manganese behavior in brain.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-0533
    Keywords: Motor neuron disease ; Amyotrophic lateral sclerosis ; Extrapyramidal system Reticular formation ; Ophthalmoparesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We carried out a postmortem examination on two Japanese patients, 64- and 80-year-old men whose survival was prolonged with an artificial respirator. They had no family history of neuropsychiatric disorders and were suspected, clinically, as having a motor neuron disease that differed from amyotrophic lateral sclerosis (ALS). As well as upper and lower motor neuron impairment, they showed a variety of symptoms, such as sensory disturbances, hypohidrosis, impotence, ophthalmoparesis and/or atonic neurogenic bladder, and their protein content in cerebrospinal fluid was elevated markedly. Pathological examination revealed the following extensive nervous system involvement: (1) the upper and lower voluntary motor systems, including the IIIrd, IVth and VIth cranial nerve nuclei: (2) the reticular formation and its major afferent pathways; (3) the vestibulospinal and tectospinal systems; (4) the spinocerebellar system and the exteroceptive somatic afferent pathways; (5) the dentatorubral and pallidoluysian system; and (6) the substantia nigra, locus ceruleus and intermediolateral and Onufrowicz's nuclei. Neither Bunina bodies, Lewy body-like hyaline inclusions nor ubiquitin immunoreactive skein-like structures were observed. The distribution of the lesions was quite different from that in patients with ALS and the other known related diseases. Recently, seven autopsied cases with clinical and histopathological similarities to our patients have been reported in Japan. Our conclusion is that our two and these seven patients should be classified as having a new motor neuron disease entity, which can be is differentiated from ALS.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-0533
    Keywords: Demyelination ; Myelinolysis ; Central pontine myelinolysis ; Pontine and extrapontine myelinolysis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Three autopsy cases of pontine and extrapontine myelinolysis are reported; one, a malignant lymphoma in a man of 66 years, the other an alcoholic liver cirrhosis in a man of 54 years, and an esophageal cancer in a woman of 68 years who presented only with extrapontine myelinolysis, but lacked central pontine myelinolysis (CPM). The extrapontine lesions in these three cases revealed a characteristic and common localization; they occurred mostly (1) in the bundles of myelinated fibers in the gray matter, such as in the pons, basal ganglia, and thalamus; and (2) in the white matter surrounded by massive gray matter, such as the deeper layers of the cortex and subjacent white matter of the crowns and sides of the cerebral gyri, the white matter of the cerebellar folia and internal, external, and extreme capsules. Therefore, the third patient was classified as a subtype of pontine and extrapontine myelinolysis, which may be called the “extrapontine form” because of absence of CPM. Moreover, bilateral demyelination of the mamillary body was found in all cases, and laminar cortical astrocytosis and necrosis similar to Morel's cortical laminar sclerosis in two of them. From the clinical and pathologic findings, the significance of the changed osmolarity of the blood as a cause and the importance of some specificity of the tissue architecture in the pathogenesis are discussed.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 66 (1985), S. 170-172 
    ISSN: 1432-0533
    Keywords: Cortical cerebellar degeneration ; Testicular neoplasm ; Paraneoplastic syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Cortical cerebellar degeneration was found in a 28-year-old man with testicular neoplasm. The patient, who had undergone a left orchidectomy for the testicular tumor, developed progressive cerebellar symptoms with mental changes 7 months later. The autopsy revealed the spread of a malignant germ cell tumor of the testis, and cortical cerebellar degeneration in the central nervous system (CNS) which was characterized by almost complete loss of Purkinje cells and degeneration of the both dentate nuclei and superior cerebellar peduncles. The present case is the first of cortical cerebellar degeneration combined with testicular neoplasms.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 79 (1989), S. 240-247 
    ISSN: 1432-0533
    Keywords: Experimental glioma ; Ethylnitrosourea ; Glomeruloid blood vessels ; Endothelial proliferation ; BrdUrd immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Glomeruloid blood vessels (GBVs), a characteristic histological feature of most human malignant gliomas, were recognized with high incidence in autochthonous rat gliomas induced by transplacental administration of ethylnitrosourea. To evaluate some of the biological properties of these GBVs, we carried out a study using histological methods and immunohistochemical staining for glial fibrillary acidic protein, factor VIII-related antigen (VIII Ag) and bromodeoxyuridine (BrdUrd). Of 22 animals with large, massively growing gliomas in the CNS, GBVs including conglomerate aggregations of small blood vessels with endothelial hyperplasia and strong VIII Ag expression were observed in 13 large gliomas histologically consisting of primitive neuroepithelial neoplasms (PNN; so called ependymoma) and mixed-type gliomas in combination with astrocytoma and PNN or anaplastic astrocytoma. The anaplastic gliomas in our series were devoid of GBVs. These findings indicate that GBV formation takes place in a histological variety of experimental gliomas. Furthermore, the GBVs were frequently associated with the vasculo-mesenchymal stroma in the parent gliomas, suggesting an intimate relationship with the morphogenesis of GBVs. In addition, it was shown that the GBVs had a higher BrdUrd-labelling index than that of other blood vessels in gliomas and also that of neoplastic cells in most parent gliomas, except for anaplastic gliomas. Based on these results, the possible mechanism of GBV morphogenesis is discussed with regard to the roles of macromolecules in the induction and regulation of GBVs.
    Type of Medium: Electronic Resource
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