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)

Progressive multifocal leukoencephalopathy is a virtually always opportunistic infection of central nervous system caused by papova viruses, which clinically presents with symptoms and signs of involvement of different encephalic levels. We report a case with a double interest: on the one hand, both clinical features and lesions were limited to the brainstem and cerebellum; on the other hand, the disease developed in a previously healthy female in whom laboratory evidence of immunodeficiency of unknown origin was demonstrated. A reason for immunodeficiency was also not found at autopsy, being speculated that it could have been iatrogenically associated with antidepressant drugs.
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PMID:[Progressive multifocal leukoencephalopathy associated with leukopenia of unknown (iatrogenic?) origin]. 236 Oct 27

Cerebrospinal fluid (CSF) cytology, white blood cell (WBC) count and protein were evaluated in 32 human immunodeficiency virus (HIV)-infected patients with the acquired immune deficiency syndrome (AIDS) or an AIDS-related complex who manifested a variety of neurologic symptoms. Of 17 patients with AIDS-related encephalitis (ARE), 13 had hypocellular CSFs; elevated WBCs and pleocytosis were noted in 4, multinucleated giant cells in 2 and elevated CSF protein was found in 4 of 8 specimens tested. Three patients with central nervous system (CNS) toxoplasmosis had unremarkable CSF cytology findings, but all had elevated CSF proteins. In five patients with cryptococcal meningitis, cytologic examinations demonstrated organisms in four and elevated proteins in three. Of five patients with primary CNS lymphomas, one had cytology positive for large cell lymphoma; two showed suspicious cells and two manifested "atypical lymphocytes." Elevated CSF protein was present in four. Other conditions observed included progressive multifocal leukoencephalopathy, tubercular meningitis and cytomegaloviral (CMV) meningitis or encephalitis. Twenty-five percent of patients with ARE manifested pleocytosis with multinucleated giant cells; pleocytosis with CMV inclusions was noted in a CMV viral radiculitis. The CSF cytologic examination in HIV-infected patients with neurologic complications seems helpful in diagnosing cryptococcal meningitis and lymphoma, but less so for diagnosing toxoplasmosis.
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PMID:Cerebrospinal fluid manifestations of the neurologic complications of human immunodeficiency virus infection. 253 86

The acquired immunodeficiency syndrome (AIDS) is a devastating new disease caused by the human immunodeficiency virus (HIV). This retrovirus causes profound immunoincompetence in its infected hosts, who are thereafter susceptible to develop myriad severe and relapsing protozoal, fungal, bacterial, viral, and arthropodal opportunistic infections, as well as unusual malignancies. The more than 50,000 patients who have developed AIDS in the United States have produced a sudden unexpected deluge of diagnostic dilemmas that are stressing laboratories of pathology everywhere. This paper describes the gross and microscopic pathology of the numerous complications in patients infected by HIV: (a) the prodromal AIDS-related complex with persistent generalized lymphadenopathy, (b) lymphoid infiltration of salivary gland and lung, including the complex of lymphoid interstitial pneumonitis-pulmonary lymphoid hyperplasia, (c) extranodal non-Hodgkin's lymphomas, (d) multifocal mucocutaneous and visceral Kaposi's sarcoma, (e) small cell undifferentiated (oat cell) carcinomas, (f) protozoal infections caused by Pneumocystis carinii, Toxoplasma gondii, Acanthamoeba, Cryptosporidium species (sp.), and Isospora belli, (g) the causes of chronic enteritis, (h) mycotic infections caused by Candida sp., Cryptococcus neoformans, Histoplasma capsulatum, Coccidioides immitis, and Sporothrix schenckii, (i) bacterial infections caused by Mycobacterium avium-intracellulare, M. tuberculosis, M. kansasii, Nocardia sp., Listeria monocytogenes, Legionella sp., Treponema pallidum, and others, (j) viral infections caused by cytomegalovirus, herpes simplex and zoster, polyomavirus (progressive multifocal leukoencephalopathy), hepatitis B, molluscum contagiosum, and papillomavirus, (k) oral hairy leukoplakia, (l) subacute encephalopathy, and (m) Norwegian scabies.
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PMID:The pathology of AIDS. 283 78

Neurologic disease occurs frequently in patients infected with the human immunodeficiency virus (HIV), and a large body of literature now exists detailing the various infections, neoplasms, and other conditions that can affect the central nervous system (CNS) or the peripheral nervous system in children and adults with AIDS, persistent generalized lymphadenopathy, or (in some cases) only serologic evidence of retroviral infection. Although certain opportunistic infections (toxoplasmosis, cryptococcosis, progressive multifocal leukoencephalopathy, and herpesviral infections) and CNS lymphomas often produce CNS disease in patients with AIDS, it is now clear that many cases of neurologic disease are caused by a group of disorders thought to be related to direct CNS infection by the AIDS retrovirus. Disease of the peripheral nervous system is also being increasingly recognized; some cases probably have an autoimmune basis.
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PMID:Neurologic disorders associated with AIDS retroviral infection. 283 38

Of the 93 acquired immune deficiency syndrome (AIDS) patients autopsied between 1983 and 1986, 27 had evidence of viral encephalitis of which 3 had progressive multifocal leukoencephalopathy (PML), confirmed by electron microscopy. Using in situ hybridization with biotinylated JC virus probes, paraffin sections from the brains of these 27 patients were examined. JC virus was found only in those patients with histologically proven PML, while no evidence of JC virus was detected in the brains of the other 24 AIDS patients despite the presence of white matter pathology. Brain biopsies of the PML patients demonstrated human immunodeficiency virus (HIV)-infected macrophages infiltrating regions of demyelination. When the patients died (2 to 6 months after diagnosis of PML), many more macrophages contained HIV antigens and some had fused to form multinucleated giant cells. These findings suggest that in AIDS patients, papovaviruses not only cause damage by directly infecting oligodendroglia but causes additional damage by eliciting the ingress of macrophages latently infected with HIV. As was seen with other infections (e.g., cytomegalovirus) of the CNS this might be a general mechanism of HIV entry into the brain.
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PMID:Human immunodeficiency virus (HIV) and JC virus in acquired immune deficiency syndrome (AIDS) patients with progressive multifocal leukoencephalopathy. 284 3

Antigens of human polyomaviruses, the etiologic agents of progressive multifocal leukoencephalopathy (PML), and of human immunodeficiency virus were localized in paraffin sections from brains of six patients who died with the acquired immunodeficiency syndrome. Immunostaining revealed polyomaviral antigens in oligodendrocytes and in some astrocytes. Human immunodeficiency (retro) virus antigens were immunostained in mononuclear macrophages, glial cells, and vascular endothelial cells. Both viral types were found ultrastructurally. The lesions of PML were more destructive than is usually seen in cases without the acquired immunodeficiency syndrome. The retroviral encephalitis could have occurred before the onset of PML. However, a secondary retroviral encephalitis could have resulted if the monocytes responding to an initial polyomaviral lesion were already infected with human immunodeficiency virus before they differentiated into macrophages.
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PMID:Progressive multifocal leukoencephalopathy and retroviral encephalitis in acquired immunodeficiency syndrome. 284 83

Nearly 40% of AIDS patients develop neurological complications during the course of their illness, and about 10% experience neurological symptoms as the initial manifestations of AIDS. The most common neurological complication (14% of AIDS patients) is human immunodeficiency virus (HIV) encephalopathy, but opportunistic viral and nonviral infections and neoplasms are also quite common; the most frequent among these are cryptococcal meningitis, toxoplasmosis, primary central nervous system (CNS) lymphoma, progressive multifocal leukoencephalopathy, and herpesvirus infections. Most of the nonviral infections and neoplasms are potentially treatable. Neurological syndromes include diffuse and regional encephalopathies, myelopathy, meningitis, intraaxial cranial neuropathies, and retinopathy. About 10% of AIDS patients develop a CNS mass lesion; the chief causes of these lesions are toxoplasmosis and primary CNS lymphoma. Since the clinical profiles of the various diseases overlap to a great extent, differential diagnosis requires a thorough workup, including magnetic resonance imaging or computed tomography brain scanning, examination of the cerebrospinal fluid, and, frequently, brain biopsy. Because AIDS patients have a high incidence of multiple intracranial pathologies, the diagnostic workup may have to be repeated to identify all of the diseases present.
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PMID:Central nervous system dysfunction in acquired immunodeficiency syndrome. 306 5

Fourteen patients with AIDS were treated for 23 neurologic complications: four episodes of acute meningoencephalitis; eight episodes of subacute encephalopathy; two cases of progressive multifocal leukoencephalopathy; and nine cases of polyneuropathy. Nine patients were treated with 9-(1,3-dihydroxy-2-propoxymethyl)guanine (DHPG), one with 3'-azido-3'-deoxythymidine (AZT), and four initially with DHPG directed against cytomegalovirus (CMV) retinitis or encephalitis and subsequently with AZT against human immunodeficiency virus (HIV) encephalopathy. CMV retinitis was a helpful clinical observation indicating neurologic involvement. DHPG produced improvement in two of three cases of acute meningoencephalitis but was ineffective in cases of subacute encephalopathy or neuropathy. AZT therapy resulted in resolution in both of the two treated cases of acute confusional state and in two of the four treated cases of polyradiculoneuropathy with paraparesis but was ineffective in the late stage of subacute encephalopathy. These results suggest that CMV is important in some cases of acute meningoencephalitis, whereas HIV is a dominant pathogen in subacute dementia and polyneuropathy in patients with AIDS. DHPG may be beneficial in the former, whereas AZT appears to be effective in the latter complications.
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PMID:Responses of neurologic complications of AIDS to 3'-azido-3'-deoxythymidine and 9-(1,3-dihydroxy-2-propoxymethyl) guanine. I. Clinical features. 316 17

A review of the magnetic resonance (MR) images of 365 patients with acquired immunodeficiency syndrome (AIDS) revealed that 112 (31%) had signal abnormalities confined to the white matter. Four patterns were observed: (a) diffuse: widespread involvement of a large area; (b) patchy: localized involvement with ill-defined margins; (c) focal: well-defined areas of involvement; and (d) punctate: small foci less than 1 cm in diameter. Clinical or pathologic findings were available in 60 of the 112 patients and were correlated with the white matter patterns seen on MR images. The diffuse pattern correlated with AIDS dementia complex (ADC), which was the most common clinical diagnosis. Patchy or punctate lesions may be seen with ADC but are less common. Focal white matter lesions were not seen in patients with ADC but were seen in all six patients with progressive multifocal leukoencephalopathy, in both patients with lymphoma, and in one patient with toxoplasmosis. The authors conclude that white matter lesions are are common in AIDS and are often secondary to direct infection of the brain with human immunodeficiency virus, which causes the ADC and usually produces a diffuse white matter pattern. Biopsy is probably not indicated in these patients. Focal white matter lesions suggest a focal infection or tumor, and biopsy may be warranted.
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PMID:White matter disease in AIDS: findings at MR imaging. 317 91

Progressive multifocal leukoencephalopathy, a common complication of infections with human immunodeficiency virus (HIV), occurs in as many as 3.8% of patients with the acquired immunodeficiency syndrome (AIDS). We report 16 cases and review 12 previously reported cases of progressive multifocal leukoencephalopathy associated with HIV infection. This illness was the presenting manifestation of HIV infection in 8 cases. Limb weakness, gait abnormalities, visual loss, aand altered mental status were the commonest initial complaints. Computed tomography of the brain frequently showed hypodense, nonenhancing white matter lesions. Magnetic resonance imaging was more sensitive than computed tomography in detecting lesions. Cerebrospinal fluid analysis and electroencephalography were nondiagnostic. Impaired cell-mediated immunity was typically noted, even in the absence of other immunodeficiency-associated illnesses. Death occurred within 10 days to 18 months of the onset of symptoms in 22 patients. However, 4 patients remain alive at 3 to 23 months; of these 4, 2 have had significant improvement without treatment. Various therapies were unsuccessful.
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PMID:Progressive multifocal leukoencephalopathy associated with human immunodeficiency virus infection. A review of the literature with a report of sixteen cases. 329 1


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