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  • 1
    ISSN: 1432-0533
    Keywords: Acquired immune deficiency syndrome (AIDS) ; Human immune deficiency virus (HIV) ; Cerebral toxoplasmosis ; Progressive multifocal leucoencephalopathy ; Papovavirus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 29-year-old homosexual male with AIDS presented with progressive encephalopathy and cytomegalovirus (CMV) pneumonia. Neuropathological examination revealed toxoplasma abcesses in corpus callosum, basal ganglia and cerebellar white matter; demyelinating foci in the parietal white matter, with microscopic changes typical of progressive multifocal leucoencephalopathy and intranuclear papovavirus inclusions in oligodendrocytes; and lesions of subacute encephalitis in the periventricular regions with large cells positive by immunostaining for CMV. Diffuse myelin loss was observed in the cerebral white matter. Multinucleated giant cells were numerous in the demyelinated areas, they were also observed in close relationship with papova, CMV and Toxoplasma lesions. Immunostaining of these cells was positive for histiocyte markers and negative with the leucocyte common antigen monoclonal antibody. Some of them contained virus-like particles measuring around 100 nm similar to human immune deficiency virus (HIV) as observed in human brain.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: IgM neuropathy ; Anti-myelin-associated glycoprotein (MAG) antibody ; Tomacula ; Cell-cell adhesion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Nerve biopsy in peripheral neuropathies associated with an IgM monoclonal gammopathy may occasionally display focal myelin thickenings. In a patient with such an IgM neuropathy, in whom an anti-myelin-associated glycoprotein (MAG) antibody activity was present in the serum, single-fiber preparations revealed 34% of internodes bearing myelin swellings. The morphometric, morphological and ultrastructural findings were reminiscent but not identical to those of the hereditary tomaculous neuropathy with liability to pressure palsies. Atypical features for tomacula included lack of spiralization of the redundant loops of myelin around the axons and their predominant external situation with regard to the myelin sheath. The frequent colocalization of myelin thickenings and the widening of myelin lamellae typical of IgM neuropathies, are highly suggestive of some pathogenetic link between the two abnormalities. The redundant loops of myelin in IgM neuropathies possibly result from a defect in the axon-myelin adhesion secondary to the binding of IgM on an epitope of MAG directly involved in cell-cell adhesion.
    Type of Medium: Electronic Resource
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  • 3
    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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  • 4
    ISSN: 1432-1920
    Keywords: Key words Aqueduct ; stenosis ; Fistula ; arteriovenous ; dural ; Magnetic resonance imaging ; Embolisation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report aqueduct compression by venous ectasia in a 65-year-old man with a dural arterio-venous fistula in the posterior cranial fossa draining into a superior vermian vein. Conventional and phase-contrast MRI showed the aqueduct stenosis and the causative dilated vein.
    Type of Medium: Electronic Resource
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