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)

Children with hypertension, seizures, lethargy, encephalopathy, headache, and occipital blindness are reviewed. After undergoing antihypertensive therapy, most children improve. Some patients have a similar syndrome associated with chemotherapy, transplantation, transfusion, or human immunodeficiency virus-1 (HIV-1) infection. These latter children can develop symptoms with only minimal or no discernible elevations in blood pressure and improve, in the case of cancer-associated encephalopathy, after discontinuing chemotherapy. The reported children with this distinctive clinical condition are compared to adults with reversible posterior leukoencephalopathy syndrome. Since both gray and white matter are involved, we had suggested previously that the name be changed to (reversible) occipitoparietal encephalopathy syndrome. However, reversible posterior leukoencephalopathy has been used in the adult population and probably should be employed in children for the sake of uniformity, since both children and adults have the same clinical presentation and presumably a similar pathophysiology for the encephalopathy syndrome. The diagnosis is confirmed by reversible posterior abnormalities seen on T2-weighted brain magnetic resonance imaging, and by the presence of either headache, altered mental status, seizures, or visual disturbances.
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PMID:Hypertensive encephalopathy, reversible occipitoparietal encephalopathy, or reversible posterior leukoencephalopathy: three names for an old syndrome. 1034 93

Highly active antiretroviral treatment (HAART), which has been available for most AIDS patients in France since 1996, has resulted in a dramatic improvement of the progression of the disease. From the survey of our series of 343 brains with acquired immunodeficiency syndrome (AIDS) from patients who died between 1985 and 2002, we found both quantitative and qualitative changes in the pattern of human immunodeficiency virus (HIV) neuropathology. Quantitatively, despite a dramatic decrease in the number of autopsies, brain involvement remained a major cause of death. There was an overall decrease in incidence of cerebral toxoplasmosis, cytomegalovirus encephalitis (CMVE), and HIV encephalitis (HIVE), for which successful treatment is available. This contrasted with the unchanged incidence of progressive multifocal leukoencephalopathy (PML) and malignant non-Hodgkin lymphomas (MNHL). However, when looking closer at the 3 last years, the incidence of diseases affecting patients with severe immunodepression (CMVE, PML, and MNHL) decreased between 2000 and 2002, whereas infections occurring in patients with milder immunodeficiency, toxoplasmosis, varicella-zoster encephalitis (VZVE), or herpes simplex virus encephalitis (HSVE) became more frequent. In addition, we found uncommon types of brain infection such as BK virus encephalitis or general paresis. Finally, we described new variants of HIVE: severe leukoencephalopathy with intense perivascular macrophage and lymphocyte infiltration, possibly due to an exaggerated response from a newly reconstituted immune system, and chronic "burnt out" forms of HIVE as VZVE, toxoplasmosis, or PML, possibly associated with prolonged survival, in which neither inflammation nor organisms could be detected. These findings are compared with those reported in other neuropathological studies from different developed countries.
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PMID:The changing pattern of HIV neuropathology in the HAART era. 1276 83

Introduction of Highly Active Antiretroviral Treatment (HAART) which is available for most AIDS patients in France since 1996, has resulted in a dramatic improvement of the disease course. From the survey of our autopsy series of (AIDS) cases and the review of other neuropathological studies from different developed countries, we found quantitative and qualitative changes in the pattern of human immunodeficiency virus (HIV) neuropathology. Quantitatively, there was a dramatic decrease in the number of autopsy cases but brain involvement remained a major cause of death in AIDS patients. There was an overall decrease of cerebral toxoplasmosis, cytomegalovirus encephalitis (CMVE) and HIV encephalitis (HIVE) for which successful treatment is available. This contrasted with the unchanged incidence of progressive multifocal leucoencephalopathy (PML) and primary malignant non Hodgkin brain lymphomas (PMBL). However, when looking closer at the last three years, the incidence of diseases affecting patients with severe immunodepression (CMVE, PML, PMBL) decreased in 2000-2002, whereas infections occurring in patients with milder immunodeficiency (toxoplasmosis, varicella-zoster encephalitis (VZVE) or herpes simplex virus encephalitis (HSVE) became more frequent. Qualitatively, there were uncommon types of brain infections, such as BK virus encephalitis or general paresis. Finally, new forms of HIVE were reported: severe leukoencephalopathy with intense perivascular macrophage and lymphocyte infiltration possibly due to an exaggerated response from a newly reconstituted immune system; and also chronic "burnt out" forms of HIVE as VZVE, toxoplasmosis, or PML in which no inflammation and no infectious agent could be detected, likely due to prolonged survival.
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PMID:[The neuropathology of HIV infection in the era of highly active antiretroviral therapy]. 1475 84

A multiple sclerosis (MS)-like illness has been reported in human immunodeficiency virus (HIV)-infected patients, usually in the early stages of HIV infection. We report 3 patients with advanced HIV infection (CD4 lymphocyte count under 200/mm(3)) presenting with monophasic focal leukoencephalopathy, in whom biopsy demonstrated demyelinating lesions compatible with acute MS lesions. In 1 patient, recently started on highly active antiretroviral therapy, MS-like disease could represent an immune reconstitution syndrome. The lesions were reversible in 2 patients, but rapidly fatal in the third patient. These cases show that an MS-like disease may present in advanced HIV infection as focal monophasic leukoencephalopathy with either a reversible or fulminating course, mimicking progressive multifocal leukoencephalopathy.
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PMID:Focal monophasic demyelinating leukoencephalopathy in advanced HIV infection. 1523 51

We report two patients with cryptococcal meningitis and combined immunodeficiency with unusual magnetic resonance imaging findings of gadolinium-enhancing white matter lesions, quite different from cryptococcomas and seen prior to anti-fungal treatment. The lesions resembled demyelinating plaques and resolved. In one patient, biopsy of the lesion revealed cryptococci, non-specific inflammatory changes and occasional small perivascular lymphocyte collections, but not demyelination. Leukoencephalopathy, previously rarely observed in Cryptococcal meningitis, was thought to be the sequelae of amphotericin toxicity. Our cases demonstrate cryptococcal meningitis may present with leukoencephalopathy, possibly as an immune response to the organism.
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PMID:Two cases of Cryptococcus meningitis presenting as leukoencephalopathy prior to amphotericin therapy. 1735 61

We describe 2 human immunodeficiency virus-infected patients who developed hypertension and severe neurological abnormalities while receiving successful antiretroviral therapy. Neuroimaging findings were characteristic of reversible posterior leukoencephalopathy syndrome, a brain-capillary leak syndrome with hypertension and endothelial damage. We discuss the role of antiretroviral therapy-associated metabolic alterations in endothelial damage, hypertension, and reversible posterior leukoencephalopathy syndrome.
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PMID:Reversible posterior leukoencephalopathy syndrome in 2 HIV-infected patients receiving antiretroviral therapy. 1817 Dec 42

HIV-related acute inflammatory leukoencephalopathy of undetermined origin (AIL) is characterized by abrupt onset of symptoms generally associated with focal brain lesions and inflammatory CSF findings. A previously asymptomatic 31-year-old HIV+ woman presented with acute cognitive difficulties, right hemiparesis and dysphasia. Brain MRI showed a large contrast-enhancing lesion in the left frontal lobe; brain biopsy revealed an inflammatory process. No etiological agent was found in blood, CSF or brain tissue. The patient was given systemic steroids and gammaglobulins and put on HAART. Clinical conditions progressively and completely recovered. Further brain MRI showed the shrinkage of the lesion with no contrast enhancement. Our case could be classified as AIL in HIV resembling ADEM pattern and highlights the importance of taking into consideration. ADEM in the diagnostic process of HIV-related leukoencephalopathy even if the typical features are lacking, as immunodeficiency could modify both presentation and disease course.
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PMID:Acute transient inflammatory leukoencephalopathy in HIV. 2123 74

Progressive multifocal leukoencephalopathy (PML) is a frequently fatal demyelinating disease of the brain caused by activation of the John Cunningham virus. It typically occurs in immunocompromised patients, including transplant recipients on immunosuppressant medications, patients receiving chemotherapy for hematologic malignancies, and patients with human immunodeficiency virus. Unfortunately, there is no effective treatment for PML. By contrast, reversible progressive leukoencephalopathy syndrome (RPLS) is a generally treatable disorder that is diagnosed based on clinical symptoms (eg, altered mental status, visual abnormalities, headache, and seizures) and neuroradiographic changes (eg, cerebral edema). It is classically associated with malignant hypertension and immunosuppressive medications. Symptoms usually resolve over time, or with treatment of the underlying cause. Amid the relatively recent withdrawal of efalizumab from the US market because of its association with PML, and the added warning found on ustekinumab describing RPLS as a possible adverse effect, there has been an increasing level of concern in dermatology that biologics and other systemic medications used in the treatment of psoriasis may be related to an increased risk of specific leukoencephalopathies. In this review, we evaluate the association of prebiologics (eg, cyclosporine, methotrexate, acitretin) and biologics (eg, adalimumab, alefacept, efalizumab, etanercept, infliximab, rituximab, and ustekinumab) with the potential risk of developing PML and RPLS.
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PMID:Progressive multifocal leukoencephalopathy and reversible progressive leukoencephalopathy syndrome in dermatologic therapy. 2337

Mothball ingestion has been previously cited to induce toxic-leukoencephalopathy, secondary to the destructive effects of paradichlorobenzene on central nervous system white matter. This case presents a 37-year-old woman who experienced a neuropsychiatric syndrome consistent with paradichlorobenzene-induced toxic leukoencephalopathy after two decades of mothball abuse. Her clinical presentation was insidious, involving fluctuating cognitive decline, depression, and psychosis. This was further complicated by an human immunodeficiency virus infection and concomitant cocaine abuse. Ultimately, her clinical findings were attributed to a reversible toxic-leukoencephalopathy from mothball ingestion, and her magnetic resonance imaging findings were consistent with symmetric leukoencephalopathy and atrophy. Though leukoencephalopathy in human immunodeficiency virus has numerous potential etiologies, a patient with a history of substance abuse warrants consideration of toxin-induced leukoencephalopathy, and further inquiry regarding abuse of other substances is appropriate.
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PMID:A Case of Reversible Neuropsychiatry Symptoms in HIV due to Toxic Leukoencephalopathy. 2430 79

We herein report a 52-year-old man infected with human immunodeficiency virus (HIV) who was referred to our hospital due to the development of severe neurocognitive disorders and bilateral leukoencephalopathy. He has been treated with antiretroviral agents for 17 years, but low-level viremia has been detected consistently prior to admission. Drug resistant testing of the serum and the cerebrospinal fluid (CSF) both demonstrated a M184V mutation. A brain biopsy revealed perivascular CD8(+) T-lymphocyte infiltration, leading to the diagnosis of CD8 encephalitis. The clinical symptoms improved drastically after changing to a nucleoside reverse transcriptase inhibitor sparing regimen, which subsequently decreased the HIV viral load to an undetectable level in both the serum and CSF.
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PMID:CD8 Encephalitis Caused by Persistently Detectable Drug-resistant HIV. 2718 53


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