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Glial Cytoplasmic Inclusions (glial + cytoplasmic_inclusion)
Selected AbstractsGlial cytoplasmic inclusions and tissue injury in multiple system atrophy: A quantitative study in white matter (olivopontocerebellar system) and gray matter (nigrostriatal system)NEUROPATHOLOGY, Issue 3 2008Keisuke Ishizawa Glial cytoplasmic inclusions (GCIs) and microglia were quantified in 12 cases of multiple system atrophy (MSA) with special reference to their association with histologically defined lesion severity. The targets of the analysis were white matter (cerebellum, pontine base) and gray matter (putamen, substantia nigra). First, the lesion severity was defined: for white matter, the degree of demyelination and tissue rarefaction were semi-quantified on Klüver-Barrera (KB) sections as grade I (mildly injured), II (moderately injured), and III (severely injured); for gray matter, neurons and astrocytes were counted on KB and glial fibrillary acidic protein-immunostained sections, respectively. Next, the GCI burden was quantified on sections immunostained for ,-synuclein, phosphorylated ,-synuclein, and ubiquitin and the microglial burden was quantified on sections immunostained for HLA-DR. In white matter, the GCI and microglial burdens were the greatest when the tissue injury was mild and/or moderate (grade I and/or grade II), and they became less prominent when the tissue injury became more severe (grade III). In gray matter, in contrast, the GCI and microglial burdens failed to show significant correlations with the lesion severity. Our result suggests that the amount of GCIs as well as that of microglia is reduced when the tissue injury becomes severe in vulnerable white matter areas, but not in vulnerable gray matter areas, of MSA. It also suggests that there seems to be a difference between gray matter and white matter in the way GCIs and microglia participate in the degenerative process of MSA. [source] Gene expression changes in postmortem tissue from the rostral pons of multiple system atrophy patientsMOVEMENT DISORDERS, Issue 6 2007Anna Jelaso Langerveld PhD Abstract Multiple system atrophy (MSA) is a neurodegenerative disease characterized by various degrees of Parkinsonism, cerebellar ataxia, and autonomic dysfunction. In this report, Affymetrix DNA microarrays were used to measure changes in gene expression in the rostral pons, an area that undergoes extensive damage in MSA, but not other synucleinopathies. Significant changes in expression of 254 genes (180 downregulated and 74 upregulated) occurred in pons tissue from MSA patients when compared with control patients. The downregulated genes were primarily associated with biological functions known to be impaired in Parkinson's disease (PD) and other neurological diseases; for example, downregulation occurred in genes associated with mitochondrial function, ubiquitin-proteasome function, protein modification, glycolysis/metabolism, and ion transport. On the other hand, upregulated genes were associated with transcription/RNA modification, inflammation, immune system function, and oligodendrocyte maintenance and function. Immunocytochemistry, in conjunction with quantitative image analysis, was carried out to characterize ,-synuclein protein expression as glial cytoplasmic inclusions in the pontocerebellar tract in rostral pons tissue and to determine the relationship between the amount of aggregated ,-synuclein protein and changes in specific gene expression. Of the regulated genes, 86 were associated with the amount of observed aggregated ,-synuclein protein in the rostral pons tissue. These data indicate that cells in the pons of MSA patients show changes in gene expression previously associated with the substantia nigra of PD patients and/or other neurological diseases, with additional changes, for example related to oligodendrocyte function unique to MSA. © 2007 Movement Disorder Society [source] A quantitative study of the pathological changes in white matter in multiple system atrophyNEUROPATHOLOGY, Issue 3 2007Richard A. Armstrong The density and spatial distribution of the vacuoles, glial cell nuclei and glial cytoplasmic inclusions (GCI) were studied in the white matter of various cortical and subcortical areas in 10 cases of multiple system atrophy (MSA). Vacuolation was more prevalent in subcortical than cortical areas and especially in the central tegmental tract. Glial cell nuclei widespread in all areas of the white matter studied; overall densities of glial cell nuclei being significantly greater in the central tegmental tract and frontal cortex compared with areas of the pons. The GCI were present most consistently in the external and internal capsules, the central tegmental tract and the white matter of the cerebellar cortex. The density of the vacuoles was greater in the MSA brains than in the control brains but glial cell density was similar in both groups. In the majority of areas, the pathological changes were distributed across the white matter randomly, uniformly, or in large diffuse clusters. In most areas, there were no spatial correlations between the vacuoles, glial cell nuclei and GCI. These results suggest: (i) there is significant degeneration of the white matter in MSA characterized by vacuolation and GCI; (ii) the central tegmental tract is affected significantly more than the cortical tracts; (iii) pathological changes are diffusely rather than topographically distributed across the white matter; and (iv) the development of the vacuoles and GCI appear to be unrelated phenomena. [source] Cellular pathology in multiple system atrophyNEUROPATHOLOGY, Issue 4 2006Koichi Wakabayashi Multiple system atrophy (MSA) is a sporadic, adult-onset neurodegenerative disease, which is characterized by striatonigral degeneration, olivopontocerebellar atrophy, and preganglionic autonomic lesions in any combination. The histological hallmark is the presence of argyrophilic fibrillary inclusions in the oligodendrocytes, referred to as glial cytoplasmic inclusions (GCIs). Fibrillary inclusions are also found in the neuronal somata, axons, and nucleus. Neuronal cytoplasmic inclusions are frequently found in the pontine and inferior olivary nuclei. Since the discovery of ,-synuclein as a major component of glial and neuronal inclusions in MSA, two neurodegenerative processes have been considered in this disease: one is due to the widespread occurrence of GCIs associated with oligodendroglia,myelin degeneration (oligodendrogliopathy) in the central nervous system, and the other is due to the filamentous aggregation of ,-synuclein in the neurons in several brain regions. These two degenerative processes might synergistically cause neuronal depletion in MSA. [source] Distribution and dynamic process of neuronal cytoplasmic inclusion (NCI) in MSA: Correlation of the density of NCI and the degree of involvement of the pontine nucleiNEUROPATHOLOGY, Issue 3 2001Teruo Yokoyama MSA is a sporadic degenerative disease that occurs in striatonigral degeneration (SND), SDS and most cases of sporadic OPCA. Oligodendroglial inclusion is a hallmark of MSA. Recently there have been a small number of reports of neuronal argyrophilic inclusions. To clarify the distribution and dynamic process of neuronal cytoplasmic inclusions (NCI), 31 cases of MSA were studied using histology, immunohistochemistry, and electron microscopy. The inclusions were exclusively found in the pontine nucleus and there was a correlation between the incidence of NCI and the severity of OPCA, but not of SND. NCI were increased to some extent in the cases with moderate OPCA and decreased in number in proportion to devastation of the pontine nuclei. Immunohistochemical and ultrastructural features of NCI were virtually identical to those of glial cytoplasmic inclusions (GCI), which gives some clues to the pathogenesis of MSA. It is tempting to interpret this as NCI playing a significant role in the degenerative changes of the neurons at least in the pons. Further systematic studies on NCI in the other brain regions are necessary to elucidate the pathogenesis of neuronal degeneration in MSA. [source] Proposed neuropathological criteria for the post mortem diagnosis of multiple system atrophyNEUROPATHOLOGY & APPLIED NEUROBIOLOGY, Issue 6 2007J. Q. Trojanowski This report summarizes the recommendations of the multiple system atrophy (MSA) Working Group on Diagnostic Neuropathology Criteria for MSA that was part of an international MSA Workshop held on 26 and 27 April 2007 in Boston, MA. The workshop was supported by a grant from the National Institute of Neurological Disorders and Stroke that was intended to convene a group of international experts to revise and update criteria for the clinical and neuropathological diagnosis of MSA. The MSA Workshop recognized the glial cytoplasmic inclusions (GCIs) composed of filamentous alpha-synuclein as a defining morphological feature of MSA, and it recommends that widespread GCIs should be a criterion for the definite neuropathological diagnosis of MSA. The deliberations and recommendations of the Working Group on Diagnostic Neuropathology Criteria for MSA are summarized in this report. [source] | ||||||||||