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  • 1
    ISSN: 1432-0533
    Keywords: AA protein ; Amyloid ; Oxidation ; Immunoperoxidase ; Senile brain
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Immunoreactivity for AA protein was rarely detected in briefly fixed amyloid of senile plaques and dyshoric angiopathy, but was not observed in Congophilic angiopathy. Plaque and dyshoric amyloid exhibited variable sensitivity to permanganate oxidation; Congophilic angiopathy was resistant to oxidation. In contrast to systemic amyloid composed of AA protein, the rare immunoreactivity of senile cerebral amyloid was lost with prolonged fixation. This study demonstrates that the fibrillar protein of senile cerebral amyloid differs from that of systemic amyloid of AA type.
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  • 2
    ISSN: 1432-0533
    Keywords: Zellweger ; Cytoplasmic striations ; Fatty acids ; Adreno-leukodystrophy ; Neuronal migrations
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Neuropathological examination of three males with cerebro-hepato-renal (Zellweger) syndrome (CHRS) revealed selective neuronal lipidosis and neuroaxonal dystrophy of the dorsal nucleus of Clarke and lateral cuneate nucleus. This lipidotic alteration was visualized as perikaryal or axonal enlargements with cytoplasmic striations. With the light microscope, the striated material was birefringent and resistant to traditional lipid stains; ultrastructurally, it was composed of lipid clefts, lamellae and lamellar-lipid profiles; biochemically, the affected region contained large amounts of cholesterol esterified to very long-chain fatty acids, both saturated and mounounsaturated. This metabolic lesion, though localized to specific sensory neurons, suggests that a more generalized defect in neuronal fatty acid metabolism may be operative in CHRS.
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  • 3
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; Huntington’s ; disease ; Superoxide dismutase ; Heavy neurofilament ; subunit gene
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We present the clinical, molecular genetic and neuropathological findings of an 81-year-old man with concurrent Huntington’s disease (HD) and familial amyotrophic lateral sclerosis (FALS). His mother had been diagnosed clinically as having ALS. There was no known family history of HD, but a maternal uncle had died in a chronic care psychiatric hospital. The diagnosis of HD in the patient was suspected at age 66, after 8 years of personality change, hallucinations, agitation, cognitive decline and choreoathetosis. No symptoms of motor neuron disease were noticed at that time, but progressive weakness developed later. Postmortem examination revealed cerebral atrophy, marked atrophy of basal ganglia (grade 3), and atrophy of brain stem and spinal cord. The neostriatum displayed massive neuronal loss and gliosis. The neocortex showed changes characterisitic of Alzheimer’s disease. Pathological lesions also included loss of neurons and gliosis in the anterior horns, Clarke’s columns and the hypoglossal nuclei; degeneration of the lateral corticospinal tracts, dorsal spinocerebellar tracts and fasciculus gracilis; and rare Bunina bodies and ubiquitin-positive filamentous skeins in motor-neuron perikarya. Molecular analysis demonstrated chromosome 4p16.3 expansion of trinucleotide repeats characteristic of HD. Analysis of Cu,Zn superoxide dismutase gene and heavy neurofilament subunit gene failed to demonstrate mutations. The concurrence of HD and FALS in our patient and three previously reported cases did not appear to be associated with cosegregation in other family members.
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  • 4
    ISSN: 1432-0533
    Keywords: Key words CADASIL ; Electron microscopy ; Granular osmiophilic material ; αB crystallin ; Heat ; shock proteins
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a non-arterio-atherosclerotic, non-amyloidotic arteriopathy affecting preferentially the small arteries and arterioles of the brain. The morphologic hallmark is the presence of a characteristic granular alteration of the arterial media that ultrastructurally corresponds to the accumulation of electron-dense material surrounding the smooth muscle cells. Although the presence of this granular osmiophilic material (GOM) was originally described as limited to brain vessels, identical electron microscopic findings have been demonstrated in the media of peripheral tissue arteries, allowing for a pathologic diagnosis of the disease by a simple skin, muscle or nerve biopsy. We report some atypical features identified in our CADASIL patients that broaden the phenotypic expression of this disease. Firstly, we identified a cortical infarct in an otherwise typical CADASIL patient. Secondly, we observed GOM in skin arteries of a 30-year-old man with hemiplegic migraine, the son of a woman who had died with CADASIL. This confirms that it may be possible to diagnose the disease at a preclinical stage by the ultrastructural evaluation of peripheral tissue biopsy material, particularly for individuals for whom there is a supporting family history. Thirdly, ultrastructural examination of the skin, and subcutaneous and striated muscle of an unrelated and apparently sporadic patient with neuropathologic and neuroradiologic evidence of CADASIL in meningeal and cerebral vessels failed to reveal diagnostic lesions in peripheral arteries. Thus, the possibility of a false-negative pathologic diagnosis in patients with a clinicoradiologic diagnosis of CADASIL, if one relies solely on a peripheral tissue biopsy, does exist. Additionally, we have identified heat shock proteins (Hsp70 and αB crystallin) and ubiquitin in the vascular myocytes of affected arteries. αB crystallin also seemed to be deposited extracellularly, which suggests that GOM also might be immunoreactive for αB crystallin.
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  • 5
    ISSN: 1432-0533
    Keywords: Amyloid ; Arteritis ; Congophilic angiopathy ; Intracerebral hemorrhage ; Giant cells
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report a case of intracerebral hemorrhages due to sporadic cerebral amyloid angiopathy in a 43-year-old male with a luxuriant giant cell reaction. The amyloid was resistant to potassium permanganate-sulfuric acid oxidation and reacted with an antiserum to synthetic β-protein. The distribution and histologic characteristics of the multinucleated giant cell reaction suggest that it represents a foreign-body reaction rather than giant cell arteritis.
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  • 6
    ISSN: 1432-0533
    Keywords: Lhermitte-Duclos disease ; Proliferating cell nuclear antigen ; Calbindin ; L7 ; PEP 19
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We have evaluated a recurrence of Lhermitte-Duclos disease by immunohistochemistry for Purkinje cell markers and proliferative activity (proliferating cell nuclear antigen), by electron microscopy and for DNA ploidy (image analysis). While most of the abnormal neurons in the lesion appear to be derived from granule cells, several Purkinje cell specific polyclonal and monoclonal antibodies, including L7, PEP 19 and calbindin, labeled a minor subpopulation. Staining with monoclonal antibodies to proliferating cell nuclear antigen and measuring cell DNA index and ploidy with a cell image analyzer revealed no proliferative activity. Electron microscopy findings were similar to those previously reported. In spite of its recurrence, our findings support the notion that Lhermitte-Duclos disease is malformative, not neoplastic, and that the characteristic neurons are derived predominantly but not exclusively from a non-Purkinje cell source, probably the granule cell.
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  • 7
    ISSN: 1432-0533
    Keywords: Allergic Encephalomyelitis ; Astrocytes ; Demyelination ; Electron microscopy ; Multiple sclerosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 6-year-old boy developed post-infectious encephalomyelitis and underwent a brain biopsy (10 days after the onset of neurologic symptoms). Electron microscopic analysis of brain showed demyelinated axons, thinly myelinated axons, aberrant remyelination, and numerous phagocytes containing myelin debris. Physical stripping of myeling by pseudopodial extensions of macrophages, as reported in experimental allergic encephalomyelitis, was noted. Hypertrophic and hyperplastic astrocytes were prominent among the phagocytic cells and played an unexpectedly active role in demyelination.
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  • 8
    ISSN: 1432-0533
    Keywords: Key words Apoptosis ; Atrophy ; Calcium ; Phytanic acid ; Purkinje cell
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cerebellar atrophy, consequent to the postdevelopmental degeneration and loss of Purkinje cells and granular neurons, has been identified in three patients with rhizomelic chondrodysplasia punctata (RCDP). Cerebellar atrophy in our two chronic patients was symmetrical, but the vermis and medial portions of both hemispheres, particularly the dorsal lobules, displayed more severe atrophy than the lateral hemispheres. The distal tips of folia showed the greatest neuronal loss. Residual Purkinje cells showed progressive degenerative changes that appeared to be due, in part, to their topography. The precise mode of death of Purkinje cells in RCDP has not been established, but it does not appear to be mediated by entrance into the cell cycle or by ubiquitination; however, alterations in intracellular calcium levels and mitochondria may be involved. Elevated serum/CSF phytanic acid, decreased levels of tissue plasmalogens and increased chronological age are believed to play synergistic pathogenetic roles in this lesion.
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  • 9
    ISSN: 1432-0533
    Keywords: Key words Meningoencephalitis ; Human herpesvirus 6 ; Lymphocyte ; Progressive multifocal leukoencephalopathy ; HIV
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Human herpesvirus 6 (HHV6) has been reported as a rare cause of meningoencephalitis and leukoencephalitis. We present an HIV-infected patient with ¶lesions of progressive multifocal leukoencephalopathy (PML), but also meningoencephalitis apparently due to HHV6. Immunohistochemistry for HHV6 antigens and in situ polymerase chain reaction for HHV6 genome showed many positive lymphocytes and microglia in the meningeal and cortical lesions. More importantly, dead and dying neurons were conspicuous; some were undergoing neuronophagia and some displayed evidence of HHV6 infection. A pathogenic role for this almost universal, and usually commensal, virus in inflammatory brain lesions and PML is briefly discussed.
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  • 10
    ISSN: 1432-0533
    Keywords: Wolman's disease ; Lipid storage ; Peripheral nerve-glia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report the first case of Wolman's disease in which the fine structure of either the peripheral or the central nervous system has been examined. We confirm ultrastructurally the presence of lipid within endothelial and pericytic cells. Several cell types previously believed to be uninvolved in this storage process demonstrate lipid inclusions characteristic of Wolman's disease: perineurial, endoneurial and Schwann cells of peripheral nerve, and oligodendrocytes and astrocytes of the central nervous system.
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