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  • 1
    ISSN: 1432-0533
    Keywords: Parkinsonian syndrome ; Cerebral lymphoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The clinical history and postmortem neuropathologic findings of a case of cerebral lymphoma revealed by a typical parkinsonian syndrome are reported. The clinical symptoms initially improved with dopa therapy. The tumor was classified as a diffuse, non-cleaved, large-cell lymphoma of B-cell origin producing monoclonal lambda light chains. The substantia nigra was infiltrated by the tumor and showed neuronal loss and extraneuronal pigment. The most striking histological feature was the presence of neuronophagic-like nodules composed of lymphomatous cells both in the locus niger and in the left thalamus. No tumoral mass effect and no histological stigmata of other etiologies of parkinsonian syndromes were observed. A hypothesis is that the neuronophagic-like nodules may reflect a neuronotoxic activity of the lymphoma and may be considered at the origin of parkinsonism.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Monstrocellular brain tumor ; Malignant glioma ; Foamy cells
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A man of 32 years was admitted with a 3-month history of temporal lobe epilepsy. CT-Scan showed a well-circumscribed area of heterogenous contrast enhancement in the right temporal lobe. Gross total resection was performed but the tumor recurred: the patient died 6 months after the onset of symptoms. There was no autopsy. Histology revealed a highly pleomorphic neoplasm with extensive zones of necrosis. Monster cells, up to several hundred micrometers in diameter, with multiple and/or multilobed nuclei were numerous and showed emperipolesis for polymorphonuclear, mononuclear, and small tumor cells. Abundant mitoses were observed. Tumor cells of all sizes had ground-glass or vacuolated cytoplasm which obscured their glial nature. GFAP was demonstrated in some neoplastic cells. Reticulin fibers were confined to perivascular areas where mononuclear inflammatory cells were sometimes noted. Vascular proliferation was mild. Electronmicroscopic study revealed that the cytoplasms of the tumor cells contained abundant lipid droplets, numerous mitochondria, and glio-filaments. Such a tumor has been reported recently as “malignant glioma with heavily lipidized tumor cells”. This rare entity, previously reported as xanthosarcoma of the brain, represents a subgroup of primitive monstrocellular cerebral tumors.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 66 (1985), S. 78-82 
    ISSN: 1432-0533
    Keywords: Pallidal atrophy ; Corpus Luysii ; Amyotrophic lateral sclerosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The clinical and pathologic findings in a 34-year-old woman with basal ganglia degeneration and amyotrophic lateral sclerosis are reported. The duration of symptoms was 2 years. A maternal uncle had a parkinsonian syndrome with onset at 45 years of age. Neuropathologic examination revealed extensive neuronal loss and gliosis in the corpus Luysii. Nerve cell loss and gliosis also involved both parts of the globus pallidus, and the substantia nigra. The corticospinal tracts were demyelinated in the spinal cord, and neuronal loss was observed in the anterior horns. Only one similar case of pallido-luyso-nigral atrophy associated with amyotrophic lateral sclerosis has, to our knowledge, been reported previously. Such an association may represent more than a coincidental occurrence.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1459
    Keywords: Leucoencephalopathy, progressive multifocal ; Laryngeal neoplasm ; Motor neglect
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case is reported of the rare association between progressive multifocal leucoencephalopathy and carcinoma. A 66-year-old man underwent a laryngectomy for carcinoma. Four years later he developed a local recurrence. Simultaneously there were behavioural disturbances and a left motor neglect followed by dense hemiplegia and coma. The patient died a further 5 months later. Anergy was demonstrated by skin tests. CT scan showed asymmetrical non-enhancing low-density areas in the hemispheric white matter, brain-stem and cerebellum and neuropathological examination confirmed extensive myelin loss with typical papovavirus inclusions in oligodendrocytes identified by electron microscopy.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 540 (1988), S. 0 
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1459
    Keywords: Mitochondrial DNA deletion ; Ragged red fibres ; Ophthalmoplegia ; Mitochondrial encephalomyopathies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Various mitochondrial DNA abnormalities have been described in patients with encephalomyopathies. We performed Southern blot analysis of skeletal muscle mitochondrial DNA in nine adult patients with clinical features and ragged red fibres suggesting mitochondrial dysfunction. Two patients with encephalomyopathy and two with the MERRF syndrome (myoclonus epilepsy with ragged red fibres) had the normal PvuII restriction pattern of muscle mitchondrial DNA. In contrast, mitochondrial DNA deletion was observed in two of six patients with ophthalmoplegia. One suffered from typical Kearns-Sayre syndrome and the other from isolated external ophthalmoplegia. None of these patients had affected relatives. The detection of mitochondrial DNA deletion in external ophthalmoplegia and their site and size support previously reported data.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-0533
    Keywords: Creutzfeldt-Jakob disease ; Cerebral amyloid angiopathy ; Prion protein β/A4 amyloid
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract An 83-year-old female with no personal or familial neurological history developed progressive gait and speech disturbance and left motor deficit. She suffered intractable seizures and died 3 months after the onset of neurological signs. Neuropathology showed severe spongiosis and gliosis in the cortex and basal ganglia, and diffuse cerebral amyloid angiopathy. Immunostaining for prion protein (PrP) showed intense PrP positivity in areas of confluent spongiosis and some granular staining in astrocytes. The cortical vessel walls stained positively for β/A4 amyloid but not for PrP amyloid. Both types of amyloid were only observed in pericapillary parenchyma, in areas with severe spongiosis. There were only a few tangles and neuritic plaques in the temporal cortex; amyloid plaques were not present either by silver stains or immunostains. There was neither arteriopathic leukoencephalopathy nor cerebral hemorrhage. Immunoblot analysis of brain extracts revealed an abnormal proteinase K-resistant isoform of PrP. Association of Creutzfeldt-Jakob disease and Cerebral amyloid angiopathy in the absence of Alzheimer changes is unusual. The association of PrP and β/A4 amyloid deposits could have been fortuitous in an 83-year-old patient. An etiopathogenic relationship between β/A4 amyloid deposition and PrP accumulation may also be considered.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Neuroradiology 35 (1993), S. 529-531 
    ISSN: 1432-1920
    Keywords: Stroke ; Lyme disease ; Magnetic resonance imaging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 28-year-old female farmer, without vascular risk factors, developed a limited infarct of the pons, associated with a lymphocytic cerebrospial fluid (CSF) pleocytosis. Titres of specific antibodies againstBorrelia burgdorferi were high in serum and CSF. MRI confirmed an infarct in the territory of the medial pontine arteries, but angiography showed no, evidence of cerebral angiopathy. Antibiotic therapy rapidly led to a return to normal of CSF cytology and serology. We suggest that Lyme disease is a possible cause of cerebral ischaemia.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-1920
    Keywords: Wilson disease ; MR study
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 53-year-old patient with Wilsons's disease and without autonomic dysfunction presented on T2-weighted MR study an atypical decreased signal intensity of the putamina and the caudate nuclei. Possible explanations of such a signal abnormalities are discussed.
    Type of Medium: Electronic Resource
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