Cases reported "AIDS Dementia Complex"

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1/17. Relapsing and remitting human immunodeficiency virus-associated leukoencephalomyelopathy.

    We describe a 33-year-old homosexual man with a steroid-responsive, remitting and relapsing leukoencephalopathy associated with recent human immunodeficiency virus type 1 (hiv-1) seroconversion. biopsy of a parieto-occipital lesion revealed demyelination and astrogliosis with focal necrosis. Detailed investigations demonstrated no pathogens in the brain other than hiv-1. This patient illustrates that a neurological disorder clinically indistinguishable from multiple sclerosis may be the presenting manifestation of hiv-1 infection and may occur in the absence of clinically significant immunosuppression.
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keywords = leukoencephalopathy
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2/17. An emerging severe leukoencephalopathy: is it due to HIV disease or highly active antiretroviral therapy?

    We report an individual who had HIV-associated dementia, but a good clinical response to antiretroviral therapy, with a rising CD4 count and undetectable viral load. A severe leukoencephalopathy was noted at postmortem; however, no HIV immunopositive cells were found in the brain, suggesting that this new severe leukoencephalopathy is associated with immune reconstitution.
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ranking = 6
keywords = leukoencephalopathy
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3/17. dementia associated with human immunodeficiency virus: subcortical or cortical?

    Two different types of dementia and corresponding neuropathological findings of patients with human immunodeficiency virus (HIV) infection are presented. In one case, "subcortical" dementia with slow movements and mental processes as well as problems in active recall but without focal defects corresponded to diffuse leukoencephalopathy. In another case, "cortical" dementia with impaired abstraction and memory as well as several focal defects corresponded to microglial nodules in cortical and in deep grey matter, with only a mild diffuse leukoencephalopathy. Thus, in contrast to earlier interpretations, subcortical dementia does not appear to be the only form of dementia in HIV-infected patients, and cortical dysfunction may also occur.
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ranking = 2
keywords = leukoencephalopathy
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4/17. Severe encephalitis resulting from coinfections with HIV and jc virus.

    We observed 3 cases of progressive multifocal leukoencephalopathy (PML) among frozen CNS samples obtained at autopsy from 102 adult AIDS patients. In 2 patients, PML was associated with severe HIV encephalitis. In those 2 cases, the areas of extensive JC-induced demyelination were massively infiltrated by HIV infected macrophages/microglial cells with evidence for localized increase of HIV encephalitis in PML lesions. Using immunohistochemistry and in situ hybridization, we demonstrated that each virus infects, in a latent or productive fashion, different CNS cell populations. Therefore, the extension of HIV encephalitis could not be related to an intracellular transactivation of 1 virus by the other. However, the results are consistent with dissemination of viral infection by the recruitment of HIV-infected macrophages to damaged areas of the brain. This phenomenon might be generalized to other pathogens that are frequently associated with HIV CNS infection. Early detection and treatment of opportunistic CNS lesions could be important to prevent extension of HIV encephalitis.
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ranking = 1
keywords = leukoencephalopathy
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5/17. Neoplastic angioendotheliomatosis (NAE) of the CNS in a patient with AIDS subacute encephalitis, diffuse leukoencephalopathy and meningo-cerebral cryptococcosis.

    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.
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ranking = 6
keywords = leukoencephalopathy
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6/17. Vacuolar myelopathy with multinucleated giant cells in the acquired immune deficiency syndrome (AIDS). light and electron microscopic distribution of human immunodeficiency virus (HIV) antigens.

    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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ranking = 1
keywords = leukoencephalopathy
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7/17. CT-guided stereotactic biopsy of nonenhancing brain lesions.

    The majority of computer tomography stereotactic biopsy lesions enhance after administration of intravenous contrast, whereas patients with nonenhancing lesions are often followed conservatively or undergo craniotomies. There are few studies showing the effectiveness of stereotactic biopsies of nonenhancing cerebral lesions. Stereotactic biopsies were performed on 19 patients with lesions that did not enhance on CT after intravenous contrast. Pathological diagnoses were made in 90% (17/19) of patients. Four HIV-positive patients had progressive multifocal leukoencephalopathy, 11 patients had gliomas (4 astrocytomas, 6 anaplastic astrocytomas, and 1 ganglioma), 1 had multiple sclerosis, and 1 had herpes encephalitis. In 2 patients multiple biopsies revealed only gliosis. There was no morbidity or mortality. Stereotactic biopsies for nonenhancing brain lesions have a high diagnostic yield and can favorably alter the treatment course.
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ranking = 1
keywords = leukoencephalopathy
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8/17. AIDS presenting as progressive multifocal leukoencephalopathy with clinical response to zidovudine.

    Progressive multifocal leukoencephalopathy (PML) due to jc virus can be the initial manifestation of AIDS. A 40-year-old man seropositive for hiv-1 presented with aphasia, hemiparesis, and hemianopsia, and with magnetic resonance imaging of the brain typical of PML. He quickly became bed bound, incontinent, and mute. The diagnosis of PML was established by histopathology in a brain biopsy with positive immunocytochemistry for polyomavirus capsid proteins, and detection of JCV dna by polymerase chain reaction using JCV-specific primers. High dose zidovudine therapy was initiated (1200 mg/day). Within two weeks the patient began to respond, and after three months he was able to walk and care for himself and was discharged. He lived for two years from the onset of PML. While cytarabine has been the drug most widely used for PML treatment, this is the second confirmed case with apparent response to zidovudine. High dose zidovudine may benefit some previously untreated AIDS patients with onset as PML.
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ranking = 5
keywords = leukoencephalopathy
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9/17. Isolated motor control dysfunction related to progressive multifocal leukoencephalopathy during AIDS with normal MRI.

    We describe the case of a human immunodeficiency virus-infected 34-year-old man with progressive multifocal leukoencephalopathy (PML). His case displayed unusual features, including a bizarre movement disorder, predominant involvement of the subcortical U fibers on neuropathologic examination, and the absence of MRI abnormalities suggestive of PML. Anatomic-clinical correlations are discussed.
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ranking = 5
keywords = leukoencephalopathy
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10/17. Integrative agnosia following progressive multifocal leukoencephalopathy.

    A 43 year-old man with presumed progressive multifocal leukoencephalopathy developed difficulty recognizing objects and faces in the presence of adequate visual acuity and visual fields. His copying and matching of line drawings was intact, suggesting that his agnosia was associative. However, he had difficulty perceiving overlapping forms and drawings of single objects as integrated wholes. Unlike control subjects, he made fewer errors identifying silhouettes compared to line drawings with internal details. These alterations, together with his feature-by-feature descriptions of objects and copying, suggest that his agnosia was due to a disturbance in integrating local form features, as described by Riddoch and Humphreys (1987). This interpretation is supported by the findings that his tactile recognition and semantic and structural knowledge of the objects he could not identify visually were intact. Furthermore, his deficient performance in categorical matching of photographs to objects was dependent upon the perceptual complexity of the photographs. Similar deficits in early form processing described by other investigators are discussed.
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ranking = 5
keywords = leukoencephalopathy
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