Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A wide variety of neurologic conditions associated with the acquired immunodeficiency syndrome (AIDS) have been attributed to human immunodeficiency virus (HIV) infection of the central nervous system (CNS). Tissue samples from the brains of three patients with AIDS, diagnosed as having CNS toxoplasmosis on the basis of computed tomographic scans of the head, were studied by transmission electron microscopy. In two, HIV particles were observed budding from, in close association with, and in cytoplasmic vacuoles of mononuclear and multinucleated macrophages, but no other cell types. The patient with the greatest number of HIV particles also had large amounts of papovavirus (progressive multifocal leukoencephalopathy) in the nuclei of oligodendroglial cells and in the cytoplasm of astrocytes. These astrocytes often had atypical features at the light microscopic level. Following an initial biopsy that showed only HIV, primary CNS lymphoma was diagnosed by needle biopsy and confirmed at autopsy in a second case. A diagnosis of progressive multifocal leukoencephalopathy was rendered by transmission electron microscopy in a third case, but no HIV was detected. Toxoplasmosis was not confirmed in any of the three cases. Diagnosis of CNS lesions in patients with AIDS should not rely exclusively on radiography but include biopsy for both light and transmission electron microscopy. Transmission electron microscopy can be employed to reveal HIV and papovavirus infections not discernible at the light microscopic level and should be used as a diagnostic tool in HIV-related infections.
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PMID:Human immunodeficiency virus and papovavirus infections in acquired immunodeficiency syndrome: an ultrastructural study of three cases. 334 11

Two human immunodeficiency virus seropositive patients with progressive multifocal leukoencephalopathy (PML) exhibited a dramatic though incomplete recovery of neurologic function and have survived for more than 30 months since the onset of symptoms. PML was the initial manifestation of the acquired immune deficiency syndrome (AIDS) in both patients, though other opportunistic infections have subsequently supervened in one. Brain tissue from both patients obtained by stereotactic biopsy showed the typical features of PML, but was also characterized by an unusually prominent inflammatory response. Neurologic improvement did not appear to correlate with clinical or laboratory measurements of immunologic improvement. One patient continued to display neurologic recovery despite the development of other opportunistic infections. Though atypical, PML in AIDS may be associated with prolonged survival.
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PMID:Prolonged survival and partial recovery in AIDS-associated progressive multifocal leukoencephalopathy. 338 23

Magnetic resonance brain scans of 30 patients with either acquired immunodeficiency syndrome (AIDS) or AIDS-related complex were reviewed. Twenty patients had focally abnormal neurological examination results at the time of scanning. Pathological diagnosis was available in nine. Four patterns of abnormality were observed on T2-weighted images. Multiple discrete high-signal foci (pattern A) were found in patients with toxoplasmosis and progressive multifocal leukoencephalopathy. Large, bilateral patchy to confluent high-signal areas within the white matter (pattern B) represented a white matter encephalitis secondary to cytomegalovirus or human immunodeficiency virus. Generalized enlargement of the cortical sulci and ventricles (pattern C) probably reflected atrophic changes from the chronic human immunodeficiency virus infection and prolonged debilitating illness. Solitary high-signal-intensity lesions (pattern D) suggested a nonviral opportunistic infection. Differential diagnosis of brain abnormalities in patients with AIDS can be assisted by recognition of these characteristic patterns.
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PMID:Acquired immunodeficiency syndrome. Magnetic resonance patterns of brain involvement with pathologic correlation. 339 27

Recognition of the neurological symptoms and signs of the acquired immunodeficiency syndrome (AIDS) since 1982 has demonstrated the involvement of the nervous system in approximately one third of the cases. Certain opportunistic infections or tumors had been previously described in the course of immunodeficiency states of other origins: cerebral toxoplasmosis, cryptococcosis, progressive multifocal leukoencephalopathy, atypical mycobacteriosis and cerebral lymphoma. Other disorders such as subacute encephalitis raise specific etiopathogenic questions. Several of these affections can be associated or succeed each other and this is the natural course in AIDS. The detection of those conditions that are curable, among which toxoplasmosis, is of primary importance.
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PMID:[Neurologic manifestations of the acquired immunodeficiency syndrome]. 352 87

Progressive multifocal leukoencephalopathy and malignant lymphoma of the brain were noted at postmortem examination in a 68-year-old white woman who was treated with immunosuppressive agents after renal transplantation. The two diseases are not uncommon in patients with immunodeficiency, but their occurrence in the same patient is extremely rare. This association suggests the oncogenicity of papova viruses in man. However, no papovavirus was demonstrated in the tumor by electron microscopy and immunohistochemical staining. The immunohistochemical staining of routine histology sections for the common antigen of polyomaviruses by the peroxidase anti-peroxidase technique is shown to be simple and specific for the detection of polyomaviruses in the demyelinated areas of progressive multifocal leukoencephalopathy.
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PMID:Progressive multifocal leukoencephalopathy and malignant lymphoma of the brain in a patient with immunosuppressive therapy. 700 52

Progressive multifocal leukoencephalopathy is a viral-induced demyelinating disease of the central nervous system usually occurring in the immunocompromised individual. The incidence of progressive multifocal leukoencephalopathy has risen sharply over the past decade because of widespread human immunodeficiency virus infection leading to immunodeficiency. This increased incidence of progressive multifocal leukoencephalopathy may also be due to better recognition of its clinical signs, and more rapid and reliable laboratory diagnosis of JC virus, the etiologic agent. There have also been advances in the molecular detection of the JC virus and the identification of variations in the viral genome sequence that may affect its multiplication cycle in different tissues. Clinical and basic research have resulted in a better understanding of the pathogenesis of progressive multifocal leukoencephalopathy and have provided sufficient information to plan new approaches for treatment.
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PMID:Progressive multifocal leukoencephalopathy: clinical and laboratory observations on a viral induced demyelinating disease in the immunodeficient patient. 755 Nov 17

Progressive multifocal leukoencephalopathy (PML) is a lytic infection of oligodendrocytes by the human papovavirus JC. Patients with defects in cell-mediated immunity are at risk for active disease: a usually lethal demyelination of the brain. PML develops in at least 4% of patients with the acquired immunodeficiency syndrome (AIDS). Definitive diagnosis currently requires brain biopsy. Previous attempts to detect JC virus DNA by polymerase chain reaction in cerebrospinal fluid of PML patients, particularly those with human immunodeficiency virus type 1 (HIV-1) infection, have been of low sensitivity. In the present study, cerebrospinal fluid was assayed by polymerase chain reaction from 26 HIV-1-positive patients with PML, 114 HIV-1-positive control subjects, and 16 control subjects who were HIV-1 negative or were without risk factors for HIV disease. Polymerase chain reaction conditions were optimized to detect a single copy of viral DNA in 50 microliters of cerebrospinal fluid. Specificity of the polymerase chain reaction product was confirmed by size on gel electrophoresis and Southern blot hybridization. JC virus DNA was detected in 24 of 26 samples from patients with PML: 8 of 8 with tissue diagnosis and 16 of 18 with strong clinical and magnetic resonance imaging evidence of PML. Among control subjects, 11 of 130 samples were positive for JC virus: 10 of 114 samples from HIV-infected patients and one from an HIV-negative patient with risk factors for PML and an unexplained hemiparesis. Overall sensitivity was 92% (24/26); specificity was, at minimum, 92% (119/130). Treatments for PML are now in clinical trials.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:JC virus DNA in cerebrospinal fluid of human immunodeficiency virus-infected patients: predictive value for progressive multifocal leukoencephalopathy. 769 39

Progressive multifocal leucoencephalopathy (PML) is a rarely occurring demyelinating disease of the central nervous system caused by a neurotropic papovavirus named JC virus (JCV). The most frequently affected areas are the cerebral hemispheres, especially the parieto-occipital region, followed by the cerebellum and brain stem. The disease occurs predominantly in individuals with an immunocompromised state and impaired cellular mediated immunity (CMI) due to other underlying illness. More extensive use of irradiation and immunosuppressive therapy in relation to increased transplantational activities as well as treatment of autoimmune diseases and malignancies, in addition to the appearance of the acquired immunodeficiency syndrome (AIDS) as a consequence of infection with the human immunodeficiency virus (HIV), has caused a considerable increase in the occurrence of PML. The course of the disease is still most often rapidly progressive and fatal, but several cases with prolonged survival and even remission have been reported, and various antiviral treatments have been tried. The only drug that until now has shown favourable results is cytosine arabinoside. In HIV-infected PML-patients immunomodulation with AZT/zidovudine may alleviate the course and improve the prognosis in some patients. Suspicion of PML should lead to an extensive immunological investigation before considering of brain biopsy, which is still the only specific test. On the basis of the increased frequency of PML in relation to HIV-infection, it is likely that our knowledge of the pathogenetic aspects will increase, which, hopefully, may lead to an effective therapeutic strategy. A review of this disease, based upon studies of the literature, is presented.
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PMID:[Progressive multifocal leukoencephalopathy]. 784 75

A case of acquired immunodeficiency syndrome (AIDS) with preceding aplastic anemia is reported. The patient was a 36 year old female who had been diagnosed as having aplastic anemia 10 years before and thereafter had received multiple transfusions. Human immunodeficiency virus (HIV)-seropositivity was revealed 10 months prior to her death, but no particular clinical signs indicating HIV infection, pre-AIDS or onset of AIDS were recognized before serological diagnosis, although the slow progression of leukopenia was noted along with thrombocytopenia. Her general condition deteriorated during the last 10 months accompanied by an acute decrease in the CD4/CD8 ratio. Autopsy revealed full-blown AIDS: systemic aspergillosis, progressive multifocal leukoencephalopathy, Epstein-Barr virus-related B cell lymphoma arising in the diaphragm and severe lymphocyte depletion in the lymph nodes and spleen. Markedly hypoplastic bone marrow was considered to be primarily attributable to the aplastic anemia but the affection of AIDS was not excluded. The possible transmission route of HIV and the effect of the preceding aplastic anemia on the infection and clinical course of AIDS are discussed.
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PMID:An autopsy case of acquired immune deficiency syndrome (AIDS) with preceding aplastic anemia. 786 68

We evaluated 49 paired cerebrospinal fluid (CSF) and serum samples of 35 patients infected with the human immunodeficiency virus type 1 (HIV-1) for laboratory evidence of cytomegalovirus (CMV) infection. The patients were grouped according to clinical criteria as probable CMV encephalitis/polyradiculomyelitis, CMV retinitis, cerebral toxoplasmosis, progressive multifocal leukoencephalopathy, HIV-1-related cognitive/motor complex, HIV-1-associated myelopathy, and other neurological diseases. Paired CSF and serum samples were analysed for CMV deoxyribonucleic acid (DNA) by polymerase chain reaction (PCR), quantitative intrathecal synthesis of immunoglobulin G (IgG) antibodies specific for recombinant phosphoprotein 150 (pp150) of CMV and CMV-specific serum IgM. Intrathecal synthesis of pp150-specific IgG was detected in 26% of patients (9/35), serum IgM was found in 23% of patients (8/35), and PCR of CSF was positive in 11% of patients (4/35). Detection of CMV-specific DNA in CSF preceded the intrathecal antibody synthesis in three patients for whom serial samples were available. PCR results of the CSF became negative in one patient with CMV polyradiculomyelitis after successful therapy with 9-[2-hydroxy-1-(hydroxymethyl) ethoxymethyl] guanine (DHPG). PCR has a higher diagnostic specificity in the acute phase of CMV infection than intrathecal antibody synthesis. The serum IgM response to CMV cannot be used to monitor a compartmentalized immune response in the central nervous system while an intrathecal immune response seems to be associated with recovery either spontaneously or as a result of treatment.
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PMID:Comparative analysis of intrathecal antibody synthesis and DNA amplification for the diagnosis of cytomegalovirus infection of the central nervous system in AIDS patients. 793 40


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