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)

The neuropathologic findings in the spinal cord were reviewed in 138 consecutive autopsies of patients with the acquired immunodeficiency syndrome. In all cases both the brain and spinal cord were examined by conventional histologic techniques, and in 63 cases immunohistochemistry was used to detect human immunodeficiency virus (HIV), Toxoplasma gondii, cytomegalovirus, and JC papovavirus antigens. The most common observation was a normal spinal cord (60%). Vacuolar myelopathy (VM) was observed in 23 (17%) cases. Human immunodeficiency virus myelitis was evident in 8% of cases. Human immunodeficiency virus myelitis was associated with HIV encephalitis in 65% of the cases. Opportunistic infections of the spinal cord were uncommon, consisting of cryptococcosis (five cases), cytomegalovirus (four cases), toxoplasmosis (one case), and progressive multifocal leukoencephalopathy (one case), and almost always were seen with cerebral and/or systemic infection by these agents. Malignant lymphoma rarely involved the spinal cord (four cases); all were B-cell lymphomas and were associated with cerebral and/or systemic lymphoma. Other abnormalities rarely observed were Wallerian degeneration of the corticospinal tracts or posterior columns (6%) and focal microinfarcts. Most cases of VM (78%) were not associated with HIV myelitis, and in the five patients with both VM and HIV myelitis, HIV-infected cells were not found in the regions affected by VM. In contrast, 65% of cases with VM were associated with HIV encephalitis. The pathogenesis of VM remains unknown; it is probably not due to direct infection by HIV.
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PMID:Neuropathology of the spinal cord in the acquired immunodeficiency syndrome. 139 40

We examined spinal cords, nerve roots, or peripheral nerves of 27 patients who died with acquired immunodeficiency syndrome (AIDS) for the presence of cytomegalovirus (CMV) and human immunodeficiency virus (HIV) by immunoperoxidase techniques in paraffin-embedded tissue. Vacuolar myelopathy was seen in 8 of 26 spinal cords (31%) and microglial nodules were seen in 13 (50%). All of the patients with lateral column vacuolar myelopathy showed severe brain pathology. HIV antigens had been detected in the brains of 15 (55%) of the 27 patients but were detected in only 3 (11%) of 26 spinal cords and were not localized to regions of vacuolar myelopathy. This suggests that the vacuolar myelopathy may be due to a remote or indirect effect of HIV or other infectious agent. CMV antigens were detected in none of the patients who showed vacuolar myelopathy but were detected in 2 of the 13 with microglial nodules. Focal nerve root or peripheral nerve inflammation was seen in 7 patients; 4 had CMV antigens and none had HIV antigens. CMV appears to be an important cause of inflammatory peripheral neuropathy in AIDS patients.
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PMID:Spinal cord and peripheral nerve pathology in AIDS: the roles of cytomegalovirus and human immunodeficiency virus. 254 60

Vacuolar myelopathy (VM) is a frequent neurological complication of the acquired immune deficiency syndrome (AIDS). A suspected connection between VM and human immunodeficiency virus (HIV) has been based only on HIV isolation from affected spinal cord tissue. We report here an AIDS patient dying after 14 months of progressive dementia, including 3 months of spinal signs and symptoms. At autopsy, the brain revealed moderate diffuse damage of the white matter compatible with HIV-induced progressive diffuse leukoencephalopathy. The spinal cord showed VM mainly in the lateral and the posterior columns. Mono- and multinucleated macrophages were localized within intramyelinic and periaxonal vacuoles. Light and electron microscopic immunocytochemistry revealed the presence of HIV antigens restricted to mono- and multinucleated macrophages within the spongy lesions. Productive HIV infection is documented for the first time within VM lesions of this case. Therefore, VM should be included among HIV-induced lesions of the central nervous system. The intimate relation of infected macrophages to vacuolar myelinopathy could suggest secretion of a myelinotoxic factor by macrophages productively infected by HIV. Immune electron microscopy appears as promising tool to detect HIV in tissue even when the density of virus may be low.
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PMID:Vacuolar myelopathy with multinucleated giant cells in the acquired immune deficiency syndrome (AIDS). Light and electron microscopic distribution of human immunodeficiency virus (HIV) antigens. 268 61

Vacuolar myelopathy is the most frequent spinal syndrome in patients infected with the human immunodeficiency virus, presently considered to be related to a direct action of the virus. The authors review the historical, clinical, pathological and etiopathogenetic aspects of this new entity, stressing the difficulties posed by its differential diagnosis and its scarce therapeutic possibilities.
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PMID:[Spinal cord involvement by the human immunodeficiency virus. Vacuolar myelopathy]. 270 Feb 99

Aseptic meningitis, subacute encephalitis, and vacuolar myelopathy are the three diseases of the central nervous system that are specifically related to or associated with human immunodeficiency virus (HIV) infection. HIV encephalitis initially is associated with myelin pallor and gliosis of the centrum semiovale, which is found in more than 90% of brains from patients dying with the acquired immunodeficiency syndrome. With increased severity of disease, multiple glial nodules with the multinucleated cells characteristic of HIV encephalitis are present throughout the cerebral white matter, basal ganglia, and cerebral cortex, and also may be found in cerebellum, brainstem, and spinal cord. HIV has been demonstrated in monocytes and multinucleated cells by electron microscopy, immunohistochemical techniques, and in situ hybridization. Vacuolar myelopathy occurs in approximately 30% of patients and is characterized by vacuolation of the white matter of the spinal cord that is most prominent in the posterior and lateral columns at thoracic levels. The severity of the pathological lesions correlates not only with symptoms and signs of spinal cord disease but also with dementia. Although the incidence of vacuolar myelopathy is increased in patients with HIV encephalitis, its etiology is not yet established.
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PMID:Review of central nervous system pathology in human immunodeficiency virus infection. 327 4

Vacuolar myelopathy (VM) and tract pallor are poorly understood spinal tract abnormalities in patients with the acquired immunodeficiency syndrome (AIDS). We studied the ability of magnetic resonance imaging (MRI) to detect these changes in spinal cord specimens postmortem and whether criteria could be formulated which would allow these conditions to be differentiated from other lesions of the spinal cord in AIDS, such as lymphoma, cytomegalovirus (CMV) and human immunodeficiency virus (HIV) myelitis. We imaged 38 postmortem specimens of spinal cord. The MRI studies were interpreted blind. The specimens included cases of VM myelin pallor, CMV myeloradiculitis, HIV myelitis, lymphoma as well as normal cords, both HIV+ve and HIV-ve. MRI showed abnormal signal, suggestive of tract pathology, in 10 of the 14 cases with histopathological evidence of tract changes. The findings in VM and tract pallor on proton-density and T2-weighted MRI were increased signal from the affected white-matter tracts, present on multiple contiguous slices and symmetrical in most cases. The pattern was sufficiently distinct to differentiate spinal tract pathology from other spinal cord lesions in AIDS.
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PMID:Spinal tract pathology in AIDS: postmortem MRI correlation with neuropathology. 776 Oct

Vacuolar myelopathy is a common neurological complication in AIDS patients. The pathogenesis of this spinal cord white matter disease remains unclear and it is still debated whether infection of spinal cord with the human immunodeficiency virus type 1 (HIV-1) is causing the disease. We have generated transgenic mice expressing the entire HIV-1 genome under the regulation of an oligodendrocyte-specific promoter. These mice develop spinal cord vacuolar lesions similar to those found in AIDS patients. This animal model provides in vivo evidence linking the expression of HIV-1 proteins in oligodendrocytes to the spinal cord damage found in vacuolar myelopathy.
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PMID:Vacuolar myelopathy in transgenic mice expressing human immunodeficiency virus type 1 proteins under the regulation of the myelin basic protein gene promoter. 864 May 56

The most common disease of the spinal cord in human immunodeficiency virus (HIV) infection is vacuolar myelopathy. Pathology studies have demonstrated that vacuolization in the thoracic spinal cord is present in more than a third of patients with AIDS. The disease, however, manifests clinically only when the vacuolization in the spinal cord has become severe, with prominent myelin loss in the lateral and posterior columns. Vacuolar myelopathy presents usually with slowly progressing spastic paraparesis, accompanied by loss of vibratory and position sense and urinary frequency and urgency. In males, erectile dysfunction can be an early manifestation of the disease. The pathogenesis of vacuolar myelopathy is unknown but may be related to abnormal trans-methylation mechanisms induced by the HIV virus and cytokines. There is no known treatment for the disease, although therapy with methylating agents is being investigated. There are other rarer causes of spinal cord disease in AIDS, including a number of infectious myelitis and neoplastic and vascular myelopathies.
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PMID:Diseases of the spinal cord in human immunodeficiency virus infection. 1071 35

Although infectious myelopathies are rare, timely and accurate diagnosis is essential to improving outcome. There are a number of organisms that may cause infectious myelopathies, including human immunodeficiency virus (HIV), human T-cell lymphotropic virus type I (HTLV-I), herpesviruses, enteroviruses, Treponema pallidum, Mycobacterium tuberculosis, fungi, and parasites. Vacuolar myelopathy, the most common form of spinal cord disease in HIV-infected individuals, is underrecognized clinically. The failure to diagnose this condition is generally a consequence of the attribution of the lower extremity weakness and paresthesias to general debility and concomitant peripheral neuropathy. Tropical spastic paraparesis or HTLV-I-associated myelopathy involves the pyramidal tracts, chiefly at the thoracic level, and results in spastic lower extremity weakness and a spastic bladder. The herpesviruses (varicella-zoster, herpes simplex type 2, cytomegalovirus) and the enteroviruses cause myelitis. Prior to the development of antibiotics, syphilis was the most frequent infectious cause of spinal cord disease. In light of the broad spectrum of pathogens that may affect the spinal cord and the variegate fashion in which these disorders may present, the physician must always consider an infectious etiology in the differential diagnosis for the patient presenting with myelopathy. This review addresses the infectious myelopathies by microorganism.
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PMID:Infectious myelopathies. 1252 58

Vacuolar myelopathy (VM) is a frequent central nervous system complication of human immunodeficiency virus type 1 (HIV-1) infection. We report here that transgenic (Tg) mice expressing even low levels of Nef in oligodendrocytes under the regulation of the myelin basic protein (MBP) promoter (MBP/HIV(Nef)) developed VM similar to the human disease in its appearance and topography. The spinal cords of these Tg mice showed lower levels of the myelin proteins MAG and CNPase and of the 21-kDa isoform of MBP prior to the development of vacuoles. In addition, Tg oligodendrocytes in primary in vitro cultures appeared morphologically more mature but, paradoxically, exhibited a less mature phenotype based on O4, O1, CNPase, and MBP staining. In particular, mature CNPase(+) MBP(+) Tg oligodendrocytes were less numerous than non-Tg oligodendrocytes. Therefore, Nef appears to affect the proper differentiation of oligodendrocytes. These data suggest that even low levels of Nef expression in human oligodendrocytes may be responsible for the development of VM in HIV-1-infected individuals.
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PMID:Oligodendrocyte-specific expression of human immunodeficiency virus type 1 Nef in transgenic mice leads to vacuolar myelopathy and alters oligodendrocyte phenotype in vitro. 1455 59


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