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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 25 (1973), S. 188-195 
    ISSN: 1432-0533
    Keywords: Agammaglobulinaemia ; Encephalitis ; Polymyositis ; Immunosuppression
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of agammaglobulinaemia Bruton-type is reported, in a 5-year-old boy who, after polymyositis treated with corticosteroids, developed an encephalitis. The observation of intranuclear inclusion bodies in Purkinje cells strongly suggests the viral origin of the encephalitis. The evolution varies at different levels in the central nervous system, viz., lesions are chronic in the brain cortex (where there are superposed anoxic changes), subacute in the brain stem and active in the cerebellar cortex. An attempt is made to correlate immunological deficiency with viral agression: 1. Agammaglobulinaemia may facilitate the penetration of the virus at the respiratory mucosa level; 2. Prolonged treatment with corticosteroids results in cellular immunity deficiency superposed to the humoral immunity deficiency already existent, the consequence being an atypical encephalitis.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 66 (1985), S. 311-317 
    ISSN: 1432-0533
    Keywords: Encephalomyeloneuropathy ; Sensory neuropathy ; Brain stem encephalitis ; Limbic encephalitis ; Ganglioradiculitis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The clinical and postmortem findings in three cases of encephalomyeloneuropathy are reported. Two patients presented with subacute sensory neuropathy and one with amnesia and confusion. In none of these cases was a tumour detected clinically or at autopsy. Neuropathological examination showed inflammatory lesions in the brain, spinal cord and posterior root ganglia indistinguishable from encephalomyeloneuropathy occurring as a remote effect of carcinoma.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Human immunodeficiency virus ; Acquired immune deficiency syndrome (AIDS) ; Encephalopathy ; Cortical atrophy ; Morphometry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 25-year-old homosexual AIDS patient presented with progressive cognitive, motor and behavioral disturbances consistent with HIV encephalopathy. CT scans demonstrated progressive diffuse brain atrophy. Neuropathology showed predominant cortical changes including severe neuronal loss corroborated by morphometry. Only minimal changes were found in the white matter and basal ganglia. Immunocytochemistry for HIV stained occasional microglial cells more markedly in the cerebral cortex. This suggests that HIV infection of the brain may cause predominant cortical nerve cell loss, and that HIV encephalopathy is not necessarily due to white matter lesions.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 58 (1982), S. 48-54 
    ISSN: 1432-0533
    Keywords: Machado-Joseph disease ; Heredode-generative disease ; Ataxias
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The clinical and pathological findings in a boy suffering from Machado-Joseph disease are described. The patient was the son of two affected parents and signs first appeared at the age of 8 years. A younger brother also became affected at the age of 7. The patient presented all the characteristic features of the disease which consist of progressive cerebellar ataxia, pyramidal signs, progressive external ophthalmoplegia with variable degrees of extrapyramidal and peripheral signs. He died at the age of 15, after an unusually short duration of the disease. Pathological examination showed degeneration and mild gliosis of the substantia nigra, dentate, pontine and cranial nerve nuclei, anterior horns and Clarke's columns. Additional findings, not previously described were the involvement of sensory ganglia, intermediolateral columns and gracile and cuneate nuclei. It is suggested that the present case is homozygous for the gene of Machado-Joseph disease. The pattern of distribution of the pathological lesions and the sparing of some anatomical structures confirm our view that Machado-Joseph disease should be considered a distinct clinico-pathological entity within the group of the autosomal dominant ataxias.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0533
    Keywords: AIDS ; HIV encephalopathy ; Immunohistochemistry ; Cortex
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The encephalopathy resulting from direct infection of the brain by human immunodeficiency virus (HIV), which correlates clinically with the AIDS dementia complex, has been reported as being localized to the white matter where it induces myelin loss, gliosis and perivascular infiltration by mononuclear macrophages and multinucleated giant cells. Damage to the cortical grey matter in HIV encephalopathy was investigated in nine randomly selected HIV-positive cases with or without clinical or morphological evidence of encephalopathy and in five age-matched controls, using routine histology and immunohistochemical methods [glial fibrillary acidic protein (GFAP), microglia and HIV antibodies]. Increased numbers of GFAP-expressing astrocytes andRicinus communis agglutinin 1–120-expressing microglial cells were found in all the HIV-positive cases (including asymptomatic) and their severity could be correlated with the severity of the encephalopathy in the white matter: the increase in number of cells expressing GFAP was diffuse and the intensity of the staining higher than that of microglial cells. The subpial region was the most severely involved. It is suggested that involvement of the cortical grey matter is more common in HIV infection than previously suspected and that clinical evidence of a dementing process in AIDS is not necessarily due only to white matter lesions.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 55 (1981), S. 125-133 
    ISSN: 1432-0533
    Keywords: Allyl chloride ; Neurotoxicity ; Axonopathy ; Peripheral nerves
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Allyl chloride is known to produce a neuropathy in man after occupational exposure to its vapour. The present study describes the neuropathy which develops in mice given allyl chloride by mouth. Mice were dosed three times weekly with 300 or 500 mg/kg allyl chloride for periods from 2–17 weeks. Functional disability was observed in some animals. Apart from evidence of focal kidney damage in 70% of dosed mice, pathological changes were restricted to the nervous system. Nerve fibre degeneration was found in many peripheral nerves and in roots, tending to be more marked distally and to affect more motor than sensory nerves. Degenerated fibres were also found in dorsal, ventral and lateral columns of the spinal cord. Males were more severely affected than females. Increased numbers of filaments were an early axonal change, occurring multifocally and apparently preceding axonal degeneration. No neuronal death was observed, but occasional anterior horn and dorsal root ganglion cells showed some morphological changes. Vacuolated lesions mainly due to swelling of astrocytes and their processes were found in the ventral horn in cervical and lumbar regions of spinal cord. Animals appeared to become tolerant to allyl chloride after continuous dosing. This neuropathy appears to be a centralperipheral distal type of axonopathy.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 71 (1986), S. 119-124 
    ISSN: 1432-0533
    Keywords: Machado-Joseph disease ; Peripheral neuropathy ; Heredodegenerative disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Peripheral nerve biopsies were taken from 11 patients with Machado-Joseph disease (MJD), a heredodegenerative disease within the group of autosomal dominant ataxias. On the basis of the clinical symptoms, 2 patients were found to suffer from type I, 4 from type II and 5 from type III. All cases shared the same pathological features, which consisted of a reduction in density of myelinated and unmyelinated fibres and an increase in endoneurial collagen. It was also observed that some Schwann cells were not related to axons, whilst others showed numerous budding processes. The intensity of the changes varied considerably: it was mild in type I and II and severe in type III. Peripheral nerve changes in MJD are compared with those previously described in other forms of heredo-ataxias. It is concluded that involvement of peripheral nerves is a significant feature in this group of diseases and that peripheral nerve biopsy could be useful in the identification of the subtypes of MJD.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 68 (1985), S. 110-114 
    ISSN: 1432-0533
    Keywords: Brain tumour ; Cerebral neuroblastoma ; Neuroectodermal tumour ; Maturation ; Tumour ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of primary intracerebral neuroblastoma with long survival is reported. The initial biopsy at the age of 3 years showed a primitive, poorly differentiated tumour. Nine years later, the recurrent tumour examined by light and electron microscopy had the appearance of a cerebral neuroblastoma with many mature neurons and occasional abnormal astrocytic elements.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-0533
    Keywords: Human immunodeficiency virus ; Cytomegalovirus ; Acquired immune deficiency syndrome ; Mulunucleated giant cell
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 25-year-old homosexual male with AIDS presented with a cauda equina syndrome clinically suggestive of cytomegalovirus (CMV) myeloradiculitis. He was treated with ganciclovir with transient improvement of neurological signs and died 4 months after onset of neurological signs. Neuropathological examination revealed human immunodeficiency virus (HIV) encephalitis, CMV subependymal encephalitis and CMV myeloradiculitis. The latter was characterised by myelin loss, Schwann cell proliferation and presence of CMV early antigens in the nuclei of S-100 protein-positive cells in the spinal roots. In the subependymal regions, morphologically characteristic multinucleated giant cells, positive for CD68, contained early CMV antigens (E13) in their nuclei and HIV antigens (gp41 and p24) in their cytoplasm. The observation that HIV and CMV can co-infect the same cell in vivo raises the possibility of a direct synergistic interaction of both viruses at cell level. This suggests that CMV may play a role as a co-factor in the pathogenesis of HIV encephalopathy.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-0533
    Keywords: AIDS ; HIV ; Posterior root ganglia ; Gracile tract pallor
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The spinal cord and the thoracic and lumbar posterior root ganglia (PRGs) of 14 HIV-positive men and 7 age- and sex-matched controls were studied by routine histology, morphometric analysis of the number of nodules of Nageotte (nN) and the diameters of sensory ganglion cells, immunohistochemistry and in situ hybridization. In 7 patients (2 of whom had evidence of cytomegalovirus ganglionitis) there were increased numbers of nN and diffuse, mild infiltration with CD45R+ T lymphocytes; no B lymphocytes were observed. Macrophages were increased in number in all cases. Whenever more than one ganglion was examined from the same patient, the appearances were similar in all. There was no alteration in the distribution of ganglion cell diameters. Changes in the spinal cord included vacuolar myelopathy (5 cases), HIV myelitis (1 case), microglial nodules (3 cases) and pallor of the gracile tracts (GTP) in 7 cases, in 6 of whom it co-existed with increased numbers of nN. Seven cases had no abnormalities, except the increase in number of macrophages in PRGs. In spite of a correlation between sensory nerve cell loss and GTP our findings suggest that other mechanisms, such as ‘dying back’ may contribute to the pathogenesis of GTP. Moreover, sensory disturbances were found most commonly in association with nerve cell loss; however, loss of sensory ganglion cells was not necessarily associated with evidence of sensory impairment.
    Type of Medium: Electronic Resource
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