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1.
目的 探讨多发性硬化(MS)患者泼尼松治疗前后外周血Tc1、Tc2、Treg、Th1、Th2及Th17淋巴细胞亚群变化。方法 选取本院45例MS患者(病例组)和35例同期于本院行常规体检的健康人员(对照组),比较病例组治疗前与对照组体检之日T淋巴细胞亚群和血浆细胞因子的水平,并比较病例组治疗前和泼尼松治疗后T淋巴细胞亚群和血浆细胞因子的水平。结果 病例组Tc1,Treg细胞百分率较对照组均明显降低,Th1,Th17细胞百分率较对照组显著增高,Th17/Treg细胞比例较对照组显著增高(P<0.01); 病例组与对照组Tc2,Th2细胞百分率及Th1/Th2细胞比例的比较均无明显差异(P>0.05); 与治疗前比较,病例组治疗1个月后Th1,Th17细胞百分率均明显降低,Treg,Tc1,Tc2细胞百分率均显著增高,Th17/Terg细胞比例显著下降(P<0.01); 病例组治疗1个月后Th2、Th1/Th2细胞比例与治疗前比较均无明显差异(P>0.05)。病例组白细胞介素-4(IL-4)和白细胞介素-10(IL-10)水平较对照组均显著下降,白细胞介素-17A(IL-17A)和 SymbolgA@ -干扰素水平较对照组均明显增高(P<0.01); 与治疗前比较,病例组治疗1个月后IL-4和IL-10水平均明显增高,IL-17A和 SymbolgA@ -干扰素水平显著下降(P<0.01)。结论 MS的发生可能与Treg下调、Th17上调密切相关,推测可能通过MS靶向提高Treg与降低Th17细胞百分率来维持Th17/Treg细胞比例的平衡。  相似文献   

2.
Summary A case of a unique combination of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like syndrome (MELAS) with acanthocytosis is reported. Neuropathological examination revealed pellagra-like change in Betz cells, brain-stem neurons and anterior horn cells as well as findings compatible with mitochondrial encephalomyopathies. Abnormal function of nicotinic acid-related enzymes could be the cause of the complicated clinicopathologic findings in this case. This is the first report of MELAS with acanthocytosis.  相似文献   

3.
We report the first documented example (case 1) of lymphocytic adenohypophysitis (LAH) associated with selective destruction of prolactin cells. The morphologic data are compared to those obtained in another, more typical case (case 2). Case 1 was a 35-year-old woman with remote history of pregnancy who presented with headache, oligomenorrhea and visual disturbances. The blood prolactin level was nearly undetectable, but no deficiency of other pituitary hormones was evident. A sellar and parasellar mass compressing the optic chiasm was removed transsphenoidally. Histology demonstrated massive infiltration with lymphocytes, plasma cells and macrophages causing marked destruction of pituitary acini. Part of the gland was fibrotic. Immunocytochemistry documented all pituitary hormones, but only few cells, probably mammosomatotrophs, were immunoreactive for prolactin. Electron microscopy and immunoelectron microscopy using double gold labeling for growth hormone and prolactin detected no prolactin cells. A striking ultrastructural finding was the prominence of folliculostellate cells in areas of active cell destruction supporting the presumed immune role of these cells. LAH in case 2 (24-year-old woman) became manifest during late pregnancy, causing pituitary enlargement and visual field defects. Pituitary tests showed no major hormonal deficits. Moderate hyperprolactinemia was appropriate for her pregnancy status. A sellar mass, thought to be adenoma, was removed. Histology demonstrated multifocal LAH without major destruction of acinar structures. Immunocytochemistry and electron microscopy documented all pituitary cell types including the marked abundance of prolactin-producing cells, resultant of gestational prolactin cell hyperplasia. In addition to prolactin cells and growth hormone cells, immunoelectron microscopy showed several bihormonal mammosomatotrophs, also appropriate for pregnancy.  相似文献   

4.
A primary cerebellar rhabdomyosarcoma (RMS) in a six and a half year old boy is reported. Microscopy of the surgical material revealed lobules of closely packed cells with a high mitotic rate, pleomorphic hyperchromatic nuclei and scant cytoplasm. At their periphery, the lobules merged with rounded cells with similar nuclei but more abundant cytoplasm. These areas were surrounded by interlacing fascicles of strap cells, which were occasionally multinucleated and showed cross striations. Electron microscopy (EM) revealed the primitive nature of the closely packed cells; however, occasional intermediate size filaments were present within their cytoplasm and focal basement membrane accumulation was observed. Cells with more abundant cytoplasm had large accumulations of thick and thin filaments while strap cells showed well-developed cross striations. Immunohistochemical studies (peroxidase-antiperoxidase technique) showed vimentin in the primitive cells and desmin, myoglobin and adenosine triphosphatase as the tumor cells appeared more differentiated. Immunoreaction with antibodies against glial fibrillary acidic protein, S-100 protein and neurofilament protein were negative. Electron microscopic and immunohistochemical studies in this case demonstrated that this was an exclusively mesenchymal tumor with rhabdomyoblastic differentiation and that the pattern of differentiation follows that seen in normal myogenesis.  相似文献   

5.
A 76-year-old man with herpetic vesicle in the right auricle developed ipsilateral 5th, 6th, 7th, and 8th cranial nerves involvement and pain in the dermatome of the second cervical nerve. The CSF study revealed elevated opening pressure up to 220 mmH2O, and pleocytosis up to 151 cells/mm3. Ninety-nine percent of the CSF cells were mononuclear cells. CSF protein was 47 mg/dl, and CSF glucose was 62 mg/dl. On the 24th hospital day the CSF cells decreased to 13/mm3 with 100% mononuclear cells. Titer of varicella-zoster virus (VZV) antibody was significantly elevated in CSF. Brain MRI and ABR demonstrated no abnormality. Although disorders of 5th and 6th cranial nerves and second cervical nerve improved, mild facial nerve palsy lasted and hearing disturbance showed no recovery. There are only seven cases of Ramsay Hunt syndrome associated with external ophthalmoplegia in the literature. However, un like the present case, none of these cases presented disorders of upper cervical nerves. In this case, we speculate that spreading of reactivated VZV caused local meningitis and multiple cranial nerve involvement as well as the second cervical nerve.  相似文献   

6.
Three family members had the unusual combination of severe peripheral neuropathy, atypical hereditary ataxia, spongy degeneration of cerbral hemispheres, and cerebellar and brainstem foci of necrotizing encephalopathy, proved pathologically in one autopsied case. A sural nerve from a patient, devoid of myelinated fibers, was transplanted into thymectomized, lethally irradiated, and bone marrow reconstituted adult mice. A normal number of myelinated fibers was present in the grafts eleven weeks later. Eighteen weeks after grafting, mice were reconstituted with syngeneic thymus to return immunocompetence. Schwann cells in the graft were rejected and axons became totally denuded of myelin after thymus reconstitution. The peripheral neuropathy is thus due to axonal disease since human Schwann cells were capable of normally myelinating regenerating mouse axons. A puzzling feature after rejection was the absence of myelin debris containing macrophages in the grafts. It is suggested that part of the rejection process in this model is mediated by antibody rather than by cellular mechanisms.  相似文献   

7.
Some mutations of the lamin A/C gene may be responsible for a combination of distinct phenotypes, such as muscular dystrophy and peripheral neuropathy. We describe muscle and peripheral nerve lesions in a patient with a dominant lamin A/C missense mutation, E33D. Myopathic and neurogenic patterns coexisted on muscle biopsy specimens, whereas the peripheral nerve presented a mixture of axonopathy and Schwann cell hypertrophy. A few abnormal nuclei were found in muscle fibers and Schwann cells. Our morphological findings in this case attest to the predominant axonal damage, but suggest possible involvement of Schwann cells in neuropathies related to laminopathies.  相似文献   

8.
A 12-year-old, hemophilic boy died with acquired immune deficiency syndrome (AIDS) after a clinical course characterized by progressive psycho-organic syndrome and opportunistic infections. Postmortem neuropathological examination revealed a cerebral form of neoplastic angioendotheliomatosis (NAE), leukoencephalopathy, giant cell encephalitis and meningo-cerebral cryptococcosis. The most unusual finding was the presence of proliferated neoplastic cells within lumina of some blood vessels throughout the central nervous system (CNS). These cells displayed cytologic features of malignancy and stained positively for common leukocyte antigen. Coronal sections showed diffuse cerebral and cerebellar leukoencephalopathy with most pronounced loss of myelin and axons in deep white matter, while the subcortical arcuate fibers and the corpus callosum were partially spared. In these areas numerous small foci of severe myelin loss were present. Microglial nodules and distinctive multinucleated giant cells (MGC) were numerous. Intracytoplasmic and intranuclear acidophilic inclusions were found in a few multinuclear and mononuclear cells. Close contact between mononuclear and multinuclear cells suggesting their fusion was also observed. As far as we know this is the first case of NAE encountered in AIDS, one of the rare primary cerebral forms and the youngest reported case of NAE up to now. This case could be considered as one proof more that NAE is a special form of malignant lymphoma.  相似文献   

9.
We describe a case of a desmoplastic brain tumor which was initially resected from the right fronto-temporal region in a 2 year-old boy. This nodular, calcified tumor was vascularized by the internal carotid artery and the middle meningeal artery branches. Grossly, it contained several mucoid cysts. Light microscopy showed cords or nests of small cuboidal cells surrounded by a loose connective tissue and desmoplasic areas containing fibers and spindle cells. The cuboidal cells expressed epithelial, neuronal and neuroendocrine markers. Some foci of spindle cells showed glial differentiation. The tumor recurred 16 months later and displayed some characteristics of the small cell neuroepithelial component, mitoses being conspicuous. Electron microscopy revealed undifferentiated clear cells, some containing neurosecretory granules. Karyotyping demonstrated the following formula: < 15 > 46, t(8;11) (a13; q11). The chromosome 11 breakpoint was different from that described in Ewing's sarcoma. This isolated translocation has not been previously reported to our knowledge. These unusual features lead us to report this case and to discuss its pathogenesis.  相似文献   

10.
Uptake kinetics for glutamate, GABA, and glutamine were determined in primary cultures of cerebral neurons, a predominantly GABA-ergic cell population, and of cerebellar granule cells, a predominantly glutamatergic cell population. A specially high Vmax for GABA uptake into the former and for glutamate uptake into the latter cells suggests that considerable amounts of released transmitters may be reaccumulated into appropriate nerve terminals. Nevertheless, the glutamate uptake into the cerebellar granule cells was less intense than that previously observed into corresponding cultures of astrocytes, whereas GABA was accumulated more intensely into neurons than into astrocytes. This suggests that especially glutamatergic neurons may be depleted for their transmitter by accumulation into adjacent astrocytes. If a glutamine flow astrocytes back to neurons served the purpose of balancing this transfer, it should be expected that glutamine accumulation was more intense in the glutamatergic than in the GABA-ergic cell population. This was not the case, suggesting that such a glutamate-glutamine cycle may not be operating to a major extent.  相似文献   

11.
We present a case of a mixed glial tumor (oligoastrocytoma) with signet-ring cells. This cellular feature is a rare differentiation in glial tumors of the central nervous system. Histological, immunohistochemical and ultrastructural findings have been analyzed. Signet-ring cells showed intense expression with GFAP, S-100 and vimentin. A differential diagnosis with other primary brain tumors and cerebral metástases with signet-ring cell differentiation was discussed.  相似文献   

12.
A fatal case of carnitine deficiency is described. The patient had intermittent metabolic acidosis, fluctuating hepatomegaly, and progressive muscle weakness since 22 months of age. One of two liver biopsies revealed lipid accumulation in the hepatocytes, and a muscle biopsy at age 5 years showed a lipid storage myopathy. Type 1 fibres were the most severely affected. Satellite and vascular endothelial cells also contained abnormal lipid deposits. Quantitative electron microscopy demonstrated an approximately 50-fold increase in lipid material, and a twofold increase in mitochondria in myofibres. The muscle carnitine level was less than one-seventh of the lowest value encountered in 74 biopsies from non-weak or neuromuscular disease controls. The basic abnormality in this patient is assumed to be a defect in carnitine biosynthesis.  相似文献   

13.
In a clinically unremarkable 39 year old sister of a patient afflicted with late adult metachromatic leukodystrophy, metachromatic deposits in the epithelial cells of the urine sediment, a high sulphatide excretion in the urine, and a deficiency of arylsulphatase A in urine and leucocytes were found. The motor nerve conduction velocity of the peripheral nerves in upper and lower extremities was distinctly decreased. Cerebral disturbances were not evident. It is surmised that this patient is a case of late adult metachromatic leukodystrophy in an early stage of the disease without obvious clinical signs. The peripheral neuropathy found by neurophysiological examination is interpreted as an early symptom of the disease.  相似文献   

14.
Summary A case is described of paraneoplastic cortical cerebellar degeneration in a patient with a small cell carcinoma of the lung. Following therapy, clinical improvement of cerebellar ataxia had been observed. The most severe degeneration was found in the superior aspects of the vermis and in the anterior and simple lobes as well as in the inferior aspects of the hemisphere. In addition to this distribution of degenerative lesions, uneven loss of Purkinje cells was apparent. Such distribution patterns in this case were apparently compatible with those of alcoholic cortical cerebellar degeneration (ACD), although the lesions were less severe than in ACD. Furthermore, dendritic changes in the Purkinje cells including loss of the spiny branchlets, focal swelling of the dendrites, and disappearance of secondary and tertiary branches were remarkable. It is noteworthy that these cells showed various stages of degeneration before cell loss occurred. These data suggest that the degree of vulnerability varies among Purkinje cells, and that this could be related to the uneven loss of these cells. It is proposed that, although this case and cases of ACD have both similarities and differences in their neuropathological aspects, it is apparent that both conditions have some common morphopathogenetic factor.  相似文献   

15.
Preproenkephalin mRNA in T-cells, macrophages, and mast cells   总被引:3,自引:0,他引:3  
The presence of preproenkephalin mRNA in tumor cell lines derived from myeloid and mast cells was analyzed by RNA dot blot hybridization. A B-cell lymphoma was negative for preproenkephalin mRNA, but several T-cell lymphomas were positive. A mastocytoma and two macrophage cell lines were found to have high levels of preproenkephalin mRNA. Purified natural macrophages and mast cells also possessed easily detectable levels of this mRNA.  相似文献   

16.
We have reported the clinical and autopsy findings in a case with generalized seizures, myoclonus, blindness and deafness which was accompanied by stroke-like episodes. This case was diagnosed as mitochondrial encephalomyopathy, lactic acidosis & stroke-like episodes (MELAS) from these findings. Solitary and continuous lesions of softening were distributed in both hemispheres, more severely in the frontal and occipital poles. These lesions did not correspond to a vascular supply. The pulvinar, lateral and medial geniculate body of the thalamus, cerebellar vermis and dentate nucleus had small lesions of softening. The cortical lesions occurred mainly in layer 4, and the most prominent lesions among them appeared cystic, involving the subcortical white matter, but nerve cells in layer 1 and 2 were preserved. Proliferation of small blood vessels was seen around the softening areas. Electron microscopy revealed increased mitochondria in endothelial cells of these vessels, abnormal dense bodies in skeletal muscle cells and tightly packed mitochondria in choroid plexus epithelial cells. Immunohistochemical study suggested that vimentin positive cells were seen around lesions and proliferated vessels are different from those seen in the intact tissues.  相似文献   

17.
A case of a pituitary adenoma in an acromegalic man is presented in which growth hormone secretion was demonstrated by explant culture. Light microscopy showed the tumor to consist of acidophil "signet ring" cells which contained growth hormone on immunocytochemical studies. Electron microscopy showed a mixed population of densely and sparsely granulated growth hormone adenoma cells. This latter feature has never previously been described, and may represent dual cellular differentiation from an acidophil stem cell precursor.  相似文献   

18.
Summary A case of gliofibroma occurring in an adult patient as a large circumscribed supratentorial tumor is reported. The bimorphic pattern was substantiated and further analyzed by immunohistochemistry. Some evidence in favor of collagen production by mesenchymal and/or inflammatory cells leading to a progressive fibrous replacement of the glial cells in this particular tumor type is presented.  相似文献   

19.
Summary Four tumors consisting of pituitary adenomatous cells (AD) intricated with ganglion cells (GC) were studied. Each case was associated with a different clinical syndrome: acromegaly, amenorrheagalactorrhea, Cushing's disease and isolated tumoral syndrome with no hormonal hypersecretion. (a) In the case with acromegaly, immunoreactive growth hormone (IR-GH) was present in 80% of AD. IR-vasoactive intestinal peptide (VIP) was found in 5%–10% of AD and in few GC. Rare GC and processes showed IR-GH-releasing hormone (GRH),-somatostatin (SRIH),-gonadotropin-relasing hormone and-adrenocorticotropin-releasing hormone. (b) In the case with amenorrhea-glactorrhea, IR-prolactin (PRL) was seen in 90% of AD. IR-PRL and-VIP were present in rare GC. (c) In the case with Cushing's disease, 60% of AD and very few GC contained IR-adrenocorticotropin (ACTH) and -lipotropin. Rare GC processes contained IR-SRIH. (d) In the case without pituitary hormone hypersecretion, PRL was localized in rare AD and GC. Pituitary hormone and neuropeptides were nerver colocalized in the same cells. No case displayed IR-neurophysins or-thyroliberin. Pituitary hormones were localized by ultrastructural immunogold labeling. These findings show that: (i) in three cases, pituitary hormones (PRL and ACTH), and, in one case, VIP could be localized in both adenomatous and ganglion cells; (ii) the pituitary hormone-containing cells in the tumors could be related to the hypersecretory syndromes; (iii) intratumoral IR-VIP and-GRH might be involved in GH and PRL hypersecretion in the cases with acromegaly and amenorrhea-galactorrhea.Supported in part by a grant from the Fondation pour la Recherche Médicale (Paris) (JYL)  相似文献   

20.
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