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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 63 (1984), S. 174-176 
    ISSN: 1432-0533
    Keywords: Pseudomeissnerian corpuscles ; Neurofibroma ; Giant nevus ; Immunohistochemistry ; S-100 protein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Immunohistochemical staining for S-100 protein was carried out on one case of von Recklinghausen's neurofibroma and one of giant congenital nevus. Uniformly positive staining was ootained in all cells of the numerous pseudomeissnerian corpuscles in both cases. These structures thus appear, like the true Wagner-Meissner tactile nerve endings, to consist entirely of Schwann cells and not to contain any demonstrable perineurial component.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 85 (1993), S. 227-232 
    ISSN: 1432-0533
    Keywords: Neurofibromatosis ; Hypertrophic neuropathy ; Schwann cells ; Neurinoma ; Proliferative activity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of symmetrical neurofibroma with onion bulbs in various stages of development and progression to microneurinomas is presented. Immunohistochemistry with differentiation and growth factor markers as well as electron microscopy showed a Schwann cell origin of the concentrically arranged cells. The onion bulbs differed from those of hypertrophic neuropathy by their more compact structure. A partial expression of cellular proliferation markers in the onion bulbs was consistent with a multifocal proliferative activity, confirming the neoplastic nature of the lesion.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 57 (1982), S. 197-202 
    ISSN: 1432-0533
    Keywords: Cerebellum, transneuronal degeneration ; Cerebellum, lobular sclerosis ; Hemiplegia, infantile ; Epilepsy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The problem of crossed cerebellar atrophy is re-examined in the light of an illustrative case, recent literature and the authors' previous experience. There types of lesions emerge from this analysis: (1) transneuronal atrophy of the nuclei pontis and contralateral middle cerebellar peduncle, (2) atrophy of nuclei pontis with secondary transneuronal degeneration of the granular layer of the cerebellar cortex, and (3) lobular sclerosis of the cerebellar folia of a postictal pattern. The pathogenesis of these lesions is discussed in the light of present-day concepts of transneuronal atrophy and degeneration and of the spread of the epileptic impulse. It is concluded that many cases of crossed cerebellar atrophy represent post-ictal and not transneuronal lesions.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 61 (1983), S. 65-70 
    ISSN: 1432-0533
    Keywords: Cerebellum, hypoplasia ; Brain stem, hypoplasia ; Cerebral cortex, laminar atrophy ; Atrophy, multisystem ; Inheritance, autosomal recessive
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A condition is described in two siblings, dying in early infancy, characterized by an extreme degree of cerebellar hypoplasia, hypoplasia or atrophy of the brain stem with partial preservation of cranial and spinal nerve nuclei, total degeneration of basal ganglia and thalamus, laminar atrophy of the cerebral cortex, and accumulation of sudanophil lipid in astrocytes and macrophages of the hemispheric white matter. It is suggested that this condition, possibly inherited as an autosomal recessive, may represent a progressive multisystem atrophy occurring in utero, affecting various parts of the neuraxis in different stages in their development.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 2 (1963), S. 378-389 
    ISSN: 1432-0533
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Two children, from different families, were abnormal from birth and later showed signs of a rapid mental and physical deterioration, death occurring at 4 1/2 years and 1 1/2 years respectively. Neuropathological examination revealed closely similar changes in both brains. There was severe demyelination in the cerebellar white matter and in the brain stem where certain tracts were particularly involved. The cerebral hemispheres were less affected, but the centrum ovale showed a mild, diffuse pallor of myelin staining, accompanied by fibrous gliosis, and in both cases the visual pathways were conspicuously demyelinated. In the degenerated white matter of the cerebellum the larger vessels were surrounded by dense rings of histiocytes containing an insoluble P.A.S.+ve, nonmetachromatic lipid, and similar material was present in microglial cells free in the tissues. No multinucleated globoid cells or epitheliod cell packets, as in Krabbe's disease, were seen. Sudanophil fat granule cells were numerous in areas of recent demyelination in the first case, but were scanty in the second. The destruction of axis cylinders varied considerably, but except in some of the degenerated long tracts, the loss was rarely commensurate with the demyelination. Fibrous gliosis also varied in intensity and was more marked in the younger case. An important point of distinction from Krabbe's disease was the presence of neuronal lipidosis in the brain stem of both cases, and in the dentate nucleus of the first case. These affected nerve cells contained P.A.S.+ve lipid similar to that found in the histiocytes. There was evidence of malformation in the first case in the form of megalencephaly and symmetrical polymicrogyria of the insula and parietal operculum. Chemical analysis of the white matter confirmed the demyelination observed histologically both in the severely affected cerebellum and in the less affected cerebral hemispheres. Lipid hexosamine was not significantly increased. These cases appear to represent a hitherto unrecognised form of leucodystrophy.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 56 (1982), S. 161-166 
    ISSN: 1432-0533
    Keywords: Malformations, cerebral ; Cerebellum, agenesis of vermis ; Cerebellum, fusion of hemispheres ; Septo-optic dysplasia ; Neurohypophysis agenesis ; Phencyclidine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Agenesis of the cerebellar vermis with fusion of the dentate nuclei and cerebellar hemispheres (rhombencephalosynapsis) is a rare cerebral malformation. We report the case of a 7-h-old girl whose mother had taken the drug phencyclidine during the first 6 weeks of pregnancy. Absence of the septum pellucidum, hypoplasia of the commissural system, optic nerves, chiasm and tracts, moderate hydrocephalus, and agenesis of the posterior lobe of the pituitary were also found. Extracranial congenital anomalies involved the cardiovascular, respiratory, urinary, and musculoskeletal systems. Death was secondary to severe respiratory distress and bradycardia. The literature concerning this rare form of cerebellar malformation is reviewed.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 62 (1984), S. 332-339 
    ISSN: 1432-0533
    Keywords: Epilepsy ; Cerebral hemiatrophy ; Crossed cerebellar atrophy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case is presented of a 4.5-year-old child who died 5 days after the onset of a continuous hemiconvulsion and compared with three cases of established cerebral hemiatrophy. The laminar necrosis in the first case was strikingly similar in severity and distribution with the laminar cell loss in the others, thus producing additional evidence in support of the postictal aetiology of diffuse cerebral hemiatrophy. Particular attention was focussed on the pontocerebellar lesions. Three types of lesions were observed in the ipsilateral nuclei pontis: (1) focal neuronal necrosis in the acute stage, (2) focal neuronal loss, presumably the end result of the preceding lesion and (3) transneuronal atrophy. The contralateral cerebellar hemisphere showed the following abnormalities, singly or in combination: (1) necrosis of Purkinje cells in the acute stage, (2) loss of Purkinje cells and lobular sclerosis in advanced cases and (3) reduction in bulk of the hemisphere due to lesions in the nuclei pontis and degeneration of pontocerebellar fibres. These findings of the so-called crossed cerebellar atrophy.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 71 (1986), S. 171-175 
    ISSN: 1432-0533
    Keywords: Porencephaly ; Retrograde and transneuronal degeneration ; Thalamus ; Basal forebrain nuclei ; Cerebellum
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Two cases of prenatal porencephaly in young adults are reported with the aim of studying retrograde and transynaptic degeneration and comparing the findings with similar situations developing postnatally. The thalamic nuclei with cortical projections and the nuclei of the basal forebrain complex showed hypoplasia rather than typical retrograde degeneration, while the locus ceruleus was unaffected. The nuclei pontis and the cerebellum were essentially normal in spite of severe loss of corticopontine fibers. These striking differences between the effects of prenatal and postnatal lesions are ascribed to the greater plasticity of the developing, as opposed to the mature, brain.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 74 (1987), S. 402-404 
    ISSN: 1432-0533
    Keywords: Sarcoidosis ; Choroid plexus ; Optic nerves ; Hydrocephalus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 43-year-old woman, known to have suffered from chronic multisystem sarcoidosis since the age of 28, developed a posterior fossa mass and obstructive hydrocephalus 2 years before death. At autopsy she was found to have hydrocephalus involving all four ventricles, mass lesions in the brain stem and cerebellum, and severe involvement of the choroid plexuses. The optic nerves and chiasm were also infiltrated with granulomas. Only minimal lesions were found in other parts of the brain.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 83 (1992), S. 207-210 
    ISSN: 1432-0533
    Keywords: Gliofibroma ; Immunohistochemistry ; Astrocytes ; Desmoplasia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of gliofibroma occurring in an adult patient as a large circumscribed supratentorial tumor is reported. The bimorphic pattern was substantiated and further analyzed by immunohistochemistry. Some evidence in favor of collagen production by mesenchymal and/or inflammatory cells leading to a progressive fibrous replacement of the glial cells in this particular tumor type is presented.
    Type of Medium: Electronic Resource
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