Cases reported "AIDS Dementia Complex"

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1/7. Immunohistochemical characterization of multinucleated giant cells in the brain of a Japanese AIDS patient.

    In an autopsy case of a 35-year-old Japanese hemophiliac with acquired immune deficiency syndrome (AIDS), many multinucleated giant cells (MGCs) were observed throughout the central nervous system. Immunohistochemically, MGCs possessed surface and cytoplasmic macrophage antigens expressed in the late stage of differentiation indicating them to be macrophages in the terminal stage of differentiation. Fine nuclear extensions connecting one nucleus (or lobe) to another were often observed in the MGCs. This feature was interpreted as multilobulation and considered to be a morphological characteristic of MGCs in AIDS encephalopathy. Similarity between MGCs in AIDS encephalopathy and highly lobulated lymphocytes in adult T cell leukemia is discussed.
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2/7. Multifocal vacuolar leucoencephalopathy: a distinct HIV-associated lesion of the brain.

    A 20-year-old male AIDS patient developed rapidly progressive dementia for more than 3 months prior to death. autopsy showed, in addition to adrenal cytomegalovirus (CMV) infection and focal cerebral necrosis due to toxoplasmosis, multifocal subcortical white matter lesions of the brain which were strikingly similar to the histopathology of vacuolar myelopathy in AIDS. These distinct lesions contained macrophages which were rarely multinucleated and expressed HIV antigens by immunocytochemistry. The distribution of lesions mimics extrapontine myelinolysis and progressive multifocal leucoencephalopathy (PML); PML was excluded by the absence of papovaviruses by immunocytochemistry and by in situ dna hybridization. Tissue damage in multifocal vacuolar leucoencephalopathy is different from hitherto characterized HIV-specific neuropathology such as HIV encephalitis and HIV leucoencephalopathy, and should be included in the list of conditions with damage of the brain white matter in AIDS.
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3/7. 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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4/7. Immunohistochemical localization of an HIV epitope in cerebral aneurysmal arteriopathy in pediatric acquired immunodeficiency syndrome (AIDS).

    A 6-year-old boy with acquired immunodeficiency syndrome (AIDS) developed aphasia and quadriplegia 3 months before his death. Cerebral vascular ectasia and multiple cerebral infarcts were noted on premortem radiological studies. Postmortem evaluation revealed diffuse aneurysmal dilatation of the circle of willis associated with fresh and organizing thrombi, destruction of the elastic lamina, and marked intimal fibroplasia. Multiple cerebral infarcts and subacute AIDS encephalitis with basal ganglia calcification were also present. immunohistochemistry with a monoclonal antibody (anti-gp41) to human immunodeficiency virus (HIV) demonstrated positively stained cells in the arterial wall of the circle of willis and in the cerebral parenchyma. Double immunostaining demonstrated that gp41-positive cells in the circle of willis were also positive for a macrophage marker or leukocyte-common antigen, but not with an endothelial marker. Some macrophages or microglia in the cerebrum were also colabeled with anti-gp41. These results suggest that HIV may be directly involved in vascular pathology associated with pediatric AIDS.
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5/7. 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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6/7. Acute diffuse leukoencephalitis in hiv-1 infection.

    The clinical, neuroradiological, and cerebrospinal fluid findings of a case with acute diffuse leukoencephalitis, a demyelinating disease associated with human immunodeficiency virus infection of the brain, is reported. The patient presented with acute tetraparesis as the primary manifestation of a previously symptom free HIV infection. cerebrospinal fluid analysis showed enhanced inflammatory abnormalities with high concentrations of P24 antigen. MRI showed diffuse white matter hyper-intensities in both hemispheres. In the follow up over 22 months, the neurological deficits disappeared after antiretroviral treatment in good correlation with improvements in MRI as well as in inflammatory cerebrospinal fluid abnormalities.
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7/7. aids dementia complex complicated by cytomegalovirus encephalopathy.

    We have studied longitudinally ten patients with AIDS encephalopathy with respect to pathogenetic roles of human immunodeficiency virus (HIV) and cytomegalovirus (CMV). Three patients manifested typical aids dementia complex (ADC) (initially without retinitis and with slowly progressive cognitive, motor and behavioral abnormalities which were zidovudine-responsive, and relatively preserved CD4 T cells), and seven patients presented with aids dementia complex complicated by CMV encephalopathy (ACE) (with CMV retinitis, peripheral neuropathy, altered sensorium, and rapidly declining clinical and immunological status). Whereas only HIV antibody was elevated in the spinal fluid of patients with ADC, both virus infections were active in the central nervous system of patients with ACE as shown by HIV p24 antigenemia and antigenrrhachia, elevated HIV and CMV antibody in the spinal fluid, disseminated CMV infection with retinitis, and basilar ventriculoencephalitis with multinucleated cytomegalic cells containing CMV and HIV proteins and CMV dna. The recognition of ADC and ACE is important, since some patients with ACE may respond to ganciclovir or foscarnet.
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