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Cytoplasmic Inclusions (cytoplasmic + inclusion)

Distribution by Scientific Domains

Medical Sciences	76%
Life Sciences	23%

Distribution within Medical Sciences

Pathology	60%
Neurology	19%
Hematology	9%

Kinds of Cytoplasmic Inclusions

glial cytoplasmic inclusion

neuronal cytoplasmic inclusion

Selected Abstracts

Intranuclear cytoplasmic inclusions in fine-needle aspiration smears of papillary thyroid carcinoma: A study of its morphological forms, association with nuclear grooves, and mode of formation

DIAGNOSTIC CYTOPATHOLOGY, Issue 5 2005
D.SC., Dilip K. Das M.B.B.S., Ph.D.
Abstract Intranuclear cytoplasmic inclusion (INCI) and nuclear grooves in fine-needle aspiration (FNA) smears of papillary thyroid carcinoma (PTC) represent cytoplasmic invaginations into the nucleus. Although formation of INCIs is linked to nuclear grooves, they seldom exist together. This study was undertaken to find out the various morphological forms of INCIs, their relation to nuclear grooves, and mode of formation. FNA smears of 54 PTC cases were studied for various forms of INCI, nuclear chromatin pattern, nuclear grooves, and nuclear notches. A differential count of INCIs was made in 19 cases having ,10 INCIs per 200 neoplastic cells. INCIs were present in 48 (88.9%) of 54 PTC cases. Round INCIs were present in 46 (85.2%) cases, followed by oval (48.1%), tear drop/flask-shaped (18.5%), irregular (14.8%), planoconvex/semicircular (13%), rectangular (9.3%), spindle-shaped (3.7%), and bilobed (3.7%). A differential count of INCIs also showed that the round form was the commonest (76.3%). The oval and other forms constituted 19.5 and 4.2%, respectively. INCIs and nuclear grooves were present together in a cell in 15 (27.8%) cases. The formation of INCIs as a cytoplasmic invagination into the nucleus was shown cytomorphologically in rare cells. For the first time in this study, the various morphological forms of INCIs, and the extent of their coexistence with nuclear grooves, (have been highlighted) and showed their mode of formation shown at a light microscopic level. Diagn. Cytopathol. 2005;32:264,268. © 2005 Wiley-Liss, Inc. [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 nuclei

NEUROPATHOLOGY, Issue 3 2001
Teruo 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]

Dopamine D2 receptor knockout mice develop features of Parkinson disease,

ANNALS OF NEUROLOGY, Issue 4 2009
Rogan B. Tinsley PhD
Objective This study questions whether increased dopamine (DA) turnover in nigral neurons leads to formation of Lewy bodies (LBs), the characteristic ,-synuclein,containing cytoplasmic inclusion of Parkinson disease (PD). Methods Mice with targeted deletion of the dopamine D2 receptor gene (D2R[,/,]) have higher striatal and nigral dopamine turnover and elevated oxidative stress. These mice were examined for evidence of histological, biochemical, and gene expression changes consistent with a synucleinopathy. Results LB-like cytoplasmic inclusions containing ,-synuclein and ubiquitin were present in substantia nigra pars compacta (SNpc) neurons of older D2R(,/,) mice, and were also occasionally seen in aged wild-type mice. These inclusions displaced the nucleus of affected cells and were eosinophilic. Diffuse cytosolic ,-synuclein immunoreactivity in SNpc neurons increased with age in both wild-type and D2R(,/,) mice, most likely because of redistribution of ,-synuclein from striatal terminals to SNpc cell bodies. Gene and protein expression studies indicated endoplasmic reticulum (ER) stress and changes in trafficking and autophagic pathways in D2R(,/,) SNpc. These changes were accompanied by a loss of DA terminals in the dorsal striatum, although there was no evidence of progressive cell death in the SNpc. Interpretation Increased sprouting and DA turnover, as observed in PD and D2R(,/,) mice, augments LB-like inclusions and axonal degeneration of dopaminergic neurons. These changes are associated with ER stress and autophagy. Ann Neurol 2009;66:472,484 [source]

Effusion cytomorphology and immunocytochemistry of malignant melanoma: Five cases of melanotic melanoma and one case of amelanotic melanoma

DIAGNOSTIC CYTOPATHOLOGY, Issue 7 2009
I.A.C., Katsuhide Ikeda C.T.
Abstract Effusion cytological analyses of amelanotic malignant melanoma (AMM) are very rare and no concise description of AMM related cytomorphologic features using effusion have been reported. Here, we report the cytomorphological, immunohistochemical, and immunocytochemical findings in the effusion cytology of six cases of malignant melanoma (MM), one case of AMM, and five cases of melanotic malignant melanoma. Papanicolaou-stained smears exhibited conspicuous nucleoli, multinucleation, and cytoplasmic vacuolization in all of the MM cases. In addition, the AMM case displayed numerous mitotic figures and intranuclear cytoplasmic inclusions. With regard to the immunohistochemistry findings, all six cases of melanoma were positive for Melan-A/MART-1, HBME-1, and S-100. In the immunohistochemistry analyses, five of six cases of melanoma were positive for WT-1, as was the AMM specimen. Furthermore, because the effusion analysis of malignant mesothelioma proved positive for WT-1, it should be noted that WT-1 effusion analysis is not an appropriate means to distinguish between MM and malignant mesothelioma. We suggest that it is important to recognize cytomorphologic characteristics, such as melanin pigment, conspicuous nucleoli, multinucleation, and cytoplasmic vacuolization, and to choose appropriate antibodies for the correct diagnosis of MM in effusion. Diagn. Cytopathol., 2009. © 2009 Wiley-Liss, Inc. [source]

Primary pleural epithelioid hemangioendothelioma with rhabdoid phenotype: Report and review of the literature

DIAGNOSTIC CYTOPATHOLOGY, Issue 4 2007
Anjali Saqi M.D.
Abstract Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular tumor described in diverse locations including lung and liver. Relative to these sites, primary EHE of the serous cavities is uncommon. EHE in the serous cavities mimics mesothelioma and adenocarcinoma clinically, radiographically, cytologically, and histologically. EHEs have plasmacytoid epithelioid cells with cytoplasmic vacuoles. In addition to these features, we noted eccentric nuclei with abundant eosinophilic cytoplasm and nuclei displaced peripherally by globular cytoplasmic inclusions imparting a ,rhabdoid' phenotype. These cells were often seen surrounding a hyaline core. Rhabdoid features are not unique to a single entity, and a comprehensive immunohistochemical panel is essential. We report the occurrence of pleural EHE with rhabdoid features presenting in a pleural effusion, and review the literature of primary serosal EHEs. Diagn. Cytopathol. 2007;35:203,208. © 2007 Wiley-Liss, Inc. [source]

Intranuclear cytoplasmic inclusions in fine-needle aspiration smears of papillary thyroid carcinoma: A study of its morphological forms, association with nuclear grooves, and mode of formation

Detection of unique neutrophil non-muscle myosin heavy chain-A localization by immunofluorescence analysis in MYH9 disorder presented with macrothrombocytopenia without leukocyte inclusions and deafness

EUROPEAN JOURNAL OF HAEMATOLOGY, Issue 1 2005
Shinji Kunishima
Abstract:,MYH9 disorders are autosomal-dominant macrothrombocytopenias with leukocyte inclusions caused by mutations in the MYH9 gene, which encodes the non-muscle myosin heavy chain-A (NMMHCA). We report a patient with an MYH9 disorder who presented with macrothrombocytopenia without leukocyte inclusions and severe bilateral sensory deafness. Conventional May,Grünwald,Giemsa staining failed to detect granulocyte cytoplasmic inclusions, whereas immunofluorescence analysis clearly demonstrated abnormal neutrophil NMMHCA localization. Genetic analyses revealed a novel heterozygous 18 base deletion in MYH9, leading to a six-amino acid in-frame deletion (N76_S81del) in NMMHCA. These results further support the usefulness of immunofluorescence analysis in differential diagnosis of MYH9 disorders. [source]

Hypothalamic,endocrine aspects in Huntington's disease

EUROPEAN JOURNAL OF NEUROSCIENCE, Issue 4 2006
Åsa Petersén
Abstract Huntington's disease (HD) is a hereditary and fatal disorder caused by an expanded CAG triplet repeat in the HD gene, resulting in a mutant form of the protein huntingtin. Wild-type and mutant huntingtin are expressed in most tissues of the body but the normal function of huntingtin is not fully known. In HD, the neuropathology is characterized by intranuclear and cytoplasmic inclusions of huntingtin aggregates, and cell death primarily in striatum and cerebral cortex. However, hypothalamic atrophy occurs at early stages of HD with loss of orexin- and somatostatin-containing cell populations. Several symptoms of HD such as sleep disturbances, alterations in circadian rhythm, and weight loss may be due to hypothalamic dysfunction. Endocrine changes including increased cortisol levels, reduced testosterone levels and increased prevalence of diabetes are found in HD patients. In HD mice, alterations in the hypothalamic,pituitary,adrenal axis occurs as well as pancreatic ,-cell and adipocyte dysfunction. Increasing evidence points towards important pathology of the hypothalamus and the endocrine system in HD. As many neuroendocrine factors are secreted into the cerebrospinal fluid, blood and urine, it is possible that their levels may reflect the disease state in the central nervous system. Investigating neuroendocrine changes in HD opens up the possibility of finding biomarkers to evaluate future therapies for HD, as well as of identifying novel targets for therapeutic interventions. [source]

Pro-apoptotic protein glyceraldehyde-3-phosphate dehydrogenase promotes the formation of Lewy body-like inclusions

EUROPEAN JOURNAL OF NEUROSCIENCE, Issue 2 2005
Katsumi Tsuchiya
Abstract Glyceraldehyde-3-phosphate dehydrogenase (GAPDH) has long been recognized as a classical glycolytic protein; however, previous studies by our group and others have demonstrated that GAPDH is a general mediator initiating one or more apoptotic cascades. Our most recent findings have elucidated that an expression of a pro-apoptotic protein GAPDH is critically regulated at the promoter region of the gene. Apoptotic signals for its subsequent aggregate formation and nuclear translocation are controlled by the respective functional domains harboured within its cDNA component. In this study, coexpression of GAPDH with either wild-type or mutant (A53T) ,-synuclein and less likely with ,-synuclein in transfected COS-7 cells was found to induce Lewy body-like cytoplasmic inclusions. Unlike its full-length construct, the deleted mutant GAPDH construct (C66) abolished these apoptotic signals, disfavouring the formation of inclusions. The generated inclusions were ubiquitin- and thioflavin S-positive appearing fibrils. Furthermore, GAPDH coimmunoprecipitated with wild-type ,-synuclein in this paradigm. Importantly, immunohistochemical examinations of post mortem materials from patients with sporadic Parkinson's disease revealed the colocalized profiles immunoreactive against these two proteins in the peripheral zone of Lewy bodies from the affected brain regions (i.e. locus coeruleus). Moreover, a quantitative assessment showed that about 20% of Lewy bodies displayed both antigenicities. These results suggest that pro-apoptotic protein GAPDH may be involved in the Lewy body formation in vivo, probably associated with the apoptotic death pathway. [source]

The expression of tubulin polymerization promoting protein TPPP/p25, is developmentally regulated in cultured rat brain oligodendrocytes and affected by proteolytic stress

GLIA, Issue 16 2008
Olaf Goldbaum
Abstract The tubulin polymerization-promoting protein (TPPP)/p25, was identified as a brain specific protein, is associated with microtubules (MTs) in vitro and can promote abnormal MT assembly. Furthermore it has aggregation promoting properties and is a constituent in pathological protein deposits of neurodegenerative diseases. In the brain, TPPP/p25, is present in myelinating oligodendrocytes. Here we show, using cultured rat brain oligodendrocytes, that TPPP/p25, expression is increasing during development in culture, and particularly in immature cells is associated with the centrosome. MT binding properties in oligodendrocytes are rather low, however, when MTs are disassembled by nocodazole, TPPP/p25, accumulates in the perinuclear region. Treatment of oligodendrocytes with the proteasomal inhibitor MG-132 (1 ,M; 18 h) caused an increase in the amount of TPPP/p25, by about 40%, a decrease in its solubility, and led to the appearance of TPPP/p25,-positive cytoplasmic inclusions, which stained with thioflavin S and resembled inclusion bodies. Hence, it might be speculated that acute or chronic malfunction of the proteasomal degradation system, leading to the accumulation of aggregation prone proteins and the pro-aggregatory protein TPPP/p25, or to the aggregation of TPPP/p25, on its own, is causally related to the protein aggregation process in a variety of neurodegenerative diseases. © 2008 Wiley-Liss, Inc. [source]

Synphilin-1 degradation by the ubiquitin-proteasome pathway and effects on cell survival

JOURNAL OF NEUROCHEMISTRY, Issue 2 2002
Gwang Lee
Abstract Parkinson's disease is characterized by loss of nigral dopaminergic neurons and the presence of cytoplasmic inclusions known as Lewy bodies. ,-Synuclein and its interacting partner synphilin-1 are among constituent proteins in these aggregates. The presence of ubiquitin and proteasome subunits in these inclusions supports a role for this protein degradation pathway in the processing of proteins involved in this disease. To begin elucidating the kinetics of synphilin-1 in cells, we studied its degradation pathway in HEK293 cells that had been engineered to stably express FLAG-tagged synphilin-1. Pulse-chase experiments revealed that this protein is relatively stable with a half-life of about 16 h. Treatment with proteasome inhibitors resulted in attenuation of degradation and the accumulation of high molecular weight ubiquitinated synphilin-1 in immunoprecipitation/immunoblot experiments. Additionally, proteasome inhibitors stimulated the formation of peri-nuclear inclusions which were immunoreactive for synphilin-1, ubiquitin and ,-synuclein. Cell viability studies revealed increased susceptibility of synphilin-1 over-expressing cells to proteasomal dysfunction. These observations indicate that synphilin-1 is ubiquitinated and degraded by the proteasome. Accumulation of ubiquitinated synphilin-1 due to impaired clearance results in its aggregation as peri-nuclear inclusions and in poor cell survival. [source]

RNA interference-mediated knockdown of ,-synuclein protects human dopaminergic neuroblastoma cells from MPP+ toxicity and reduces dopamine transport

JOURNAL OF NEUROSCIENCE RESEARCH, Issue 2 2007
Timothy M. Fountaine
Abstract The critical observation in the pathology of Parkinson's disease (PD) is that neurodegeneration is largely restricted to dopaminergic neurons that develop cytoplasmic inclusions called Lewy bodies. These aggregations contain the protein ,-synuclein. Furthermore, it is becoming apparent that ,-synuclein expression levels are a major factor in PD pathogenesis. Patients with additional copies of the ,-synuclein gene develop PD with a severity proportional to levels of ,-synuclein overexpression. Similarly, overexpression of ,-synuclein in in vitro and in vivo models has been shown to be toxic. However, little is known about the effects of reducing ,-synuclein expression in human neurons. To investigate this, we have developed a system in which levels of ,-synuclein can be acutely suppressed by using RNA interference (RNAi) in a physiologically relevant human dopaminergic cellular model. By using small interfering RNA (siRNA) molecules targeted to endogenous ,-synuclein, we achieved 80% protein knockdown. We show that ,-synuclein knockdown has no effect on cellular survival either under normal growth conditions over 5 days or in the presence of the mitochondrial inhibitor rotenone. Knockdown does, however, confer resistance to the dopamine transporter (DAT)-dependent neurotoxin N-methyl-4-phenylpyridinium (MPP+). We then demonstrate for the first time that ,-synuclein suppression decreases dopamine transport in human cells, reducing the maximal uptake velocity (Vmax) of dopamine and the surface density of its transporter by up to 50%. These results show that RNAi-mediated ,-synuclein knockdown alters cellular dopamine homeostasis in human cells and may suggest a mechanism for the increased survival in the presence of MPP+, a toxin used extensively to model Parkinson's disease. © 2006 Wiley-Liss, Inc. [source]

Novel fibrinogen mutation ,314Thr,Pro (fibrinogen AI duPont) associated with hepatic fibrinogen storage disease and hypofibrinogenaemia

LIVER INTERNATIONAL, Issue 10 2010
Stephen O. Brennan
Abstract Mutation in fibrinogen genes may lead to quantitative or qualitative disorders that result in bleeding, thrombosis or hepatic fibrinogen storage disease. Only three mutations in the fibrinogen , gene have been identified that cause hepatic endoplasmic reticulum storage of mutant fibrinogen. To investigate the possibility of hepatic fibrinogen storage disease in a 4-year-old male with persistently elevated serum aminotransferases and preserved synthetic function except for a prolonged INR. After informed consent, liver and blood samples were obtained. Liver sections were examined by light microscopy, anti-fibrinogen immunolabelling and electron microscopy. Purified fibrinogen was analysed by sodium dodecyl sulphate-polyacrylamide gel electrophoresis and reverse phase high performance liquid chromatography; DNA sequencing was performed using a BigDye Terminator (v. 3.1) cycle sequencing kit. Four-year-old male with persistently elevated transaminases with an INR 1.5 but otherwise normal synthetic function. Fibrinogen activity and thrombin clotting time were abnormal at 0.47 g/L and 46 s respectively. Hepatic histological examination revealed portal inflammatory infiltrates with bridging fibrosis. Clumped eosinophilic material was observed in hepatocytes that was immunoreactive to fibrinogen antisera. Ultrastructural examination showed cytoplasmic inclusions arrayed in fingerprint-like patterns. DNA sequence analysis revealed heterozygosity for a novel ,314Thr ,Pro mutation (fibrinogen AI duPont) in the fibrinogen , gene. Protein analyses showed normal patterns of A,, B, and , chains suggesting that the variant , allele was not expressed in plasma fibrinogen. We describe only the fourth mutation to be identified, ,314Thr,Pro (fibrinogen AI duPont), giving rise to hypofibrinogenaemia and hepatic fibrinogen storage disease. [source]

Ultrastructure of cyst shell and underlying membranes of three strains of the brine shrimp Artemia (Branchiopoda: Anostraca) from South India

MICROSCOPY RESEARCH AND TECHNIQUE, Issue 12 2006
V. Sugumar
Abstract The cyst of Artemia has shell and membranous coverings over the embryo. The membranous coverings have special adaptive features to allow the physical changes accompanying repeated hydration and dehydration cycles that might occur and adversely influence postembryonic development. Whole and slices of cryptobiotic cysts were processed for electron microscopy to study the internal details and to compare the morphological architecture of three Artemia strains of South India. Surface topography of scanning electron microscopic (SEM) studies revealed distinct button shaped structures on the cyst of Puthalam strain. Transmission electron microscopic (TEM) studies of the cysts displayed the conventional pattern of anostracan crustaceans with outer cortex and alveolar layer, cuticular membranes, and the cytoplasmic inclusions namely nucleus, yolk droplets, lipoid bodies, and mitochondria. The prominent wavy outer cortex layer of Puthalam cysts corroborates the results of SEM studies. Microsc. Res. Tech. © 2006 Wiley-Liss, Inc. [source]

Gene expression changes in postmortem tissue from the rostral pons of multiple system atrophy patients

MOVEMENT DISORDERS, Issue 6 2007
Anna 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]

Presynaptic parkinsonism in multiple system atrophy mimicking Parkinson's disease: A clinicopathological case study

MOVEMENT DISORDERS, Issue 4 2002
José Berciano PhD
Abstract We describe the clinicopathological findings in a patient aged 63 years at death who, at age 55 years, developed levodopa-responsive parkinsonism with no atypical features. A diagnosis of idiopathic Parkinson's disease (PD) was made. During the clinical course, fluctuations and dyskinesias appeared. Eight years after onset, he was successfully treated with subthalamic nucleus stimulation but died 3 weeks postoperatively from pulmonary embolus. Brain autopsy showed marked neuronal loss and gliosis in the substantia nigra and locus coeruleus, and, to a much lesser extent, in the basis pontis, inferior olivary nuclei, and cerebellar cortex. Striatum was normal. There were numerous oligodendroglial and neuronal cytoplasmic inclusions and neuropil threads, the highest density being localized in the pons and cerebellar white matter. No Lewy bodies were observed. We conclude that nigral, presynaptic parkinsonism may occur in multiple system atrophy, which even in the long run can be indistinguishable from PD. Putaminal preservation accounts for good response to both levodopa therapy and subthalamic nucleus stimulation. © 2002 Movement Disorder Society [source]

Meningeal alveolar soft part sarcoma confirmed by characteristic ASPCR1-TFE3 fusion

NEUROPATHOLOGY, Issue 4 2009
Istvan Bodi
Sarcoma metastatic to the brain is uncommon and rarely occurs as the initial manifestation of tumor. Alveolar soft part sarcoma (ASPS) is a rare but well-studied subtype of sarcoma. A 39-year-old man presented with seizures due to a left temporal meningeal-enhancing lesion with striking brain edema on MRI. The patient underwent neurosurgical resection for suspected meningioma. Histology showed large tumor cells clustering and forming small nests, in places with pseudoalveolar pattern. Diastase-resistant periodic acid-Schiff revealed very rare granular and rod-like cytoplasmic inclusions. Immunohistochemistry showed convincing positivity only with vimentin and smooth muscle actin. The histological features were strongly suggestive of ASPS. At the molecular level RT-PCR and sequencing analysis demonstrated ASPCR1-TFE3 fusion confirming the histological diagnosis of ASPS. There was no evidence of primary extracranial tumor by physical examination and on chest and abdominal CT scan 11 months after presentation. ASPS typically arise from the soft tissues of the extremities and develop multiple metastatic deposits usually with a long clinical course. This case may represent primary meningeal ASPS although metastatic deposit from an undiscovered primary site cannot be entirely excluded. [source]

Glial 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 2008
Keisuke 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]

A quantitative study of the pathological changes in white matter in multiple system atrophy

NEUROPATHOLOGY, Issue 3 2007
Richard 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 atrophy

NEUROPATHOLOGY, Issue 4 2006
Koichi 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 nuclei

Proposed neuropathological criteria for the post mortem diagnosis of multiple system atrophy

NEUROPATHOLOGY & APPLIED NEUROBIOLOGY, Issue 6 2007
J. 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]

Chronic lymphocytic leukemia cytoplasmic inclusions,

AMERICAN JOURNAL OF HEMATOLOGY, Issue 4 2010
Namrata Setia
No abstract is available for this article. [source]

Changes in the amount of proteins, glycogen and lipids in porcine oocytes during in vitro meiotic maturation

ANIMAL SCIENCE JOURNAL, Issue 5 2002
Sueo NIIMURA
ABSTRACT Changes in the cytoplasmic inclusions during meiotic maturation were histochemically examined in cultured porcine oocytes. The oocytes contained a small amount of protein and glycogen granules throughout the maturation culture, as well as Sudanophilic lipids composed of small, medium and large droplets. Soon after collection, the amount of Sudanophilic lipid droplets of small and medium size was small and there were 167 ± 11.2 large droplets. After being cultured for 22 h, the number of large lipid droplets decreased remarkably, while the number of small and medium ones increased. There were no differences in the number of Sudanophilic lipid droplets of different sizes between ovulated oocytes and the oocytes cultured for 44 h. The oocytes always contained a large amount of neutral fats and lipoids, but not cholesterols. In the oocytes cultured for 22 h with olomoucine, both the resumption of nuclear maturation and the decrease in the size of the Sudanophilic lipid droplets were inhibited. From the present findings, it appears that the change in the size of the Sudanophilic lipid droplets in the cytoplasm of porcine oocytes is closely related to nuclear maturation. [source]