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Results for “Leukoencephalopathy, Progressive Multifocal”

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PubMed2003

Comparison of PCR-amplified JC virus control region sequences from multiple brain regions in PML.

The authors report a 14-year-old boy with Wiskott-Aldrich syndrome complicated by progressive multifocal leukoencephalopathy after allogeneic bone marrow transplantation. Several therapeutic approaches were attempted, but there was no response. The patient died 2 months after the onset of neurologic symptoms. We detected three distinct, rearranged regions of JC virus in the cerebellum, occipital lobe, and brainstem. These findings suggest that the brain lesions had three independent origins.

Adolescent↗

PubMed1985

Intracranial space-occupying lesions in acquired immune deficiency syndrome patients.

The recent description of the acquired immune deficiency syndrome (AIDS) followed the observation of an increased incidence of unusual neoplasms and opportunistic infections in previously healthy homosexual men, intravenous drug abusers, Haitians, hemophiliacs, and certain infants. Active research efforts on this group of patients established a defect in cellular immunity. Six patients with AIDS who underwent neurosurgical procedures for intracranial space-occupying lesions are presented. Two of the patients had toxoplasmosis brain abscesses, one had primary central nervous system lymphoma, one had cytomegalovirus encephalitis, one had progressive multifocal leukoencephalopathy, and one patient remained undiagnosed despite pathological examination of the brain tissue specimen. We recommend brain biopsy in AIDS patients with space-occupying lesions because the regimen for the various conditions differs. Although the central nervous system diseases found in AIDS patients are associated with a high mortality rate, four of the six patients responded favorably to specific treatment.

Acquired Immunodeficiency Syndrome↗

PubMed1994

Catatonic disorder due to general medical conditions.

Catatonia is a neuropsychiatric syndrome that may present a difficult diagnostic dilemma. Catatonic disorder due to general medical conditions must be considered in every patient with catatonic signs. Four patients with this disorder are presented. In these patients, general medical conditions associated with catatonic disorder included dystonia, HIV encephalopathy, progressive multifocal leukoencephalopathy, encephalitis, and renal failure. Three of these patients had multifactorial presentations of medical conditions or prior psychiatric disorders. A critical literature review concerning catatonia and associated nonpsychiatric medical conditions only infrequently supported causal relationships between organic factors and the development of catatonia. The majority of patients have multifactorial etiologies.

AIDS Dementia Complex↗

PubMed1989

A rapid method for in situ hybridization for viral DNA in brain biopsies from patients with AIDS.

Brain biopsy is often necessary in the diagnosis of neurological complications found in AIDS patients. We describe here a rapid method of tissue preparation and in situ DNA hybridization for detecting JC virus DNA in frozen brain biopsy sections which allows the diagnosis of progressive multifocal leukoencephalopathy to be established on the day of surgery. Once the diagnosis is established, therapeutic and management decisions can be made more easily. The commercial availability of biotinylated probes for several of the DNA viruses most frequently encountered in brain infections of AIDS patients will provide wide application of these techniques to patient management.

Acquired Immunodeficiency Syndrome↗

PubMed1979

Detection of polyoma virus DNA in PML-brain tissue by (in situ) hybridization.

The human papova (JC) virus was extracted from brain of a patient with progressive multifocal leukoencephalopathy. A single band of virus was obtained at a density of 1.345 g/ml CsCl.JC virus DNA was purified and a highly specific cRNA was generated in vitro. In situ hybridization with JC virus cRNA and autoradiography on sections of the same brain revealed silver grains over oligodendrocytes, astrocytes and possible vascular endothelial cells, indicating the presence of JC virus DNA in these different cell classes.

Astrocytes↗

PubMed2003

[CNS changes in HIV-infected patients: magnetic resonance spectroscopy].

Changes that may appear in the central nervous system in the course of AIDS either result directly from HIV infection or--as is the case with opportunistic infections and some neoplasms--develop as a secondary consequence of general immunodeficiency. Neuroimaging techniques may be most useful in the differential diagnosis of these lesions. Basic principles of HIV encephalitis and progressive multifocal leukoencephalopathy differentiation in MRI scans are discussed in the paper, and diagnostic possibilities of MR imaging in some other infections (tuberculosis, toxoplasmosis, and cryptococcosis) are outlined. Special attention is paid, on the one hand, to difficulties in the differentiation between toxoplasmosis and lymphoma, and on the other hand--to the growing diagnostic utility of MR-spectroscopy in this respect.

AIDS Dementia Complex↗

PubMed2006

The human polyomaviruses.

The Polyomavirus family includes two members, BK virus (BKV) and JC virus (JCV), that naturally infect humans. These viruses are widely distributed among the population worldwide. Primary infection occurs in early childhood and remains for life clinically unapparent in immunocompetent individuals. In the context of severe immunosuppression and other predisposing factors BKV and JCV may reactivate and cause serious illnesses known as Polyomavirus-induced nephropathy and progressive multifocal leukoencephalopathy, respectively. Here we briefly examine the biological and physical characteristics and the lifecycle, namely receptor(s) interaction, mode of entry, intracellular trafficking, viral transcription and replication, and progeny assembly of these two human Polyomaviruses. We also provide an overview of the clinical manifestation of Polyomavirus-induced disorders in affected individuals and discuss the potential involvement of BKV and JCV in human cancer.

BK Virus↗

PubMed1984

Viral infections of the nervous system.

Neurotropic viruses cause a number of important infectious syndromes including encephalitis, myelitis, meningitis, and radiculopathy. In this review, the biology of conventional and unconventional viruses is examined. The host immune response to viruses is discussed, and patterns of viral pathogenesis are explained. The clinical features, laboratory findings, management of important viral infections, such as herpes simplex encephalitis and epidemic encephalitis, are presented. Post-infection syndromes, such as the Guillain-Barré syndrome, and chronic viral infections, such as those causing progressive multifocal leukoencephalopathy and subacute sclerosing panencephalitis, are discussed. Current knowledge concerning the nature of unconventional virus-like agents of the spongiform encephalopathies, including kuru and Creutzfeldt-Jakob disease, is summarized. Finally, viral infections of immunocompromised patients and the possible role of viruses in the newly described acquired immunodeficiency syndrome (AIDS) are examined.

Acquired Immunodeficiency Syndrome↗

PubMed1993

[A unilateral large demyelinating lesion in the cerebral white matter with slowly progressive hemiplegia and intrathecal JC virus antibody production].

A 59-year-old male patient presented in 1991 with left-sided hemiplegia and hemihypesthesia and left homonymous hemianopsia, which had developed for the preceding 12 months. The cerebrospinal fluid (CSF) level of protein was mildly elevated, oligoclonal IgG band was initially negative, but finally positive and myelin basic protein was absent. Magnetic resonance imaging and X-ray computed tomography (CT) of the brain revealed a unilateral demarcated lesion extending around the posterior horn and triagular part of the right lateral ventricle, apparently capped by remarkably gadolinium enhancement at its front, which had no mass effect, and several small isolated plaques were also observed in other areas of the brain. All lesions appeared as high intensity areas on T2 weighted images and hypodense areas on CT. The JC virus hemagglutination-inhibition antibody titers were 1:32 in serum and 1:8 in CSF; the antibody titer ratio and index both suggested an intrathecal antibody production. Pathological examination of brain biopsy specimen taken from the large lesion demonstrated profound demyelination with preservation of axons, a large number of macrophages and spotted perivascular lymphocyte cuffings. Based on this experience, we discussed the features in this case which differentiated it from multiple sclerosis, leukodystrophy and typical progressive multifocal leukoencephalopathy (PML). Diffuse-disseminated sclerosis or transitional sclerosis was most likely so far as the clinical picture, imaging and histology were concerned. However, we implicated the possible involvement of JC virus infection to the etio-pathogenesis of the demyelinating lesions in view of the intrathecal JC virus antibody production in CSF observed in this case.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies, Viral↗

PubMedDate not supplied

Brain biopsy in Indiana AIDS patients.

The neuropathologic findings of brain biopsies in a series of Indiana AIDS patients were reviewed. All subjects displayed focal lesions on radiographic studies. The three most common diagnoses were toxoplasmosis, progressive multifocal leukoencephalopathy and primary central nervous symptoms (CNS) lymphoma. These findings were consistent with previous studies elsewhere. Biopsy appears to affect the course of treatment only if CNS lymphoma is discovered. Because the value of currently available therapy for this disease in AIDS patients is controversial, there is no clear consensus regarding the indications for biopsy in this population.

AIDS Dementia Complex↗

PubMed1998

[Advances in the diagnosis and treatment of infections caused by herpesvirus and JC virus].

During the last two years important advances in the diagnosis and treatment of cytomegalovirus (CMV) disease have occurred. Several studies have suggested that biologic markers of CMV viremia (PCR, branched DNA and pp65 antigenemia) might be useful both to stratify the risk of developing CMV disease and to follow the response of CMV retinitis to therapy. It has been shown that patients who are plasma CMV PCR positive have a risk of developing CMV disease three times higher than patients who are plasma CMV PCR negative. In addition, for each log10 increase of the CMV viral load there is a 3-fold higher risk of developing CMV disease. Currently, therapeutic options for induction treatment of CMV retinitis (CMVR) are: i.v. ganciclovir (GCV), i.v. foscarnet, i.v. cidofovir or GCV intraocular implant combined with oral GCV. For maintenance therapy options are: i.v. GCV (3, 5 or 7 days per week), oral GCV (only for peripheral retinitis), i.v. foscarnet (daily), i.v. cidofovir (biweekly) and GCV intraocular implant (replaced every 6-8 months) combined with oral GCV. There is currently enough evidence to allow the diagnosis of progressive multifocal leukoencephalopathy (PML) based on the finding of JC virus DNA in CSF by PCR. There are still no drugs with proven clinical efficacy against JC virus but the possibility that HAART treatments might improve the control of this disease appear promising.

AIDS-Related Opportunistic Infections↗

PubMed1999

Chorea in patients with AIDS.

OBJECTIVE: To describe differing etiologies and possible anatomoclinical correlates of choreic movements in a series of AIDS patients. METHODS: We analyzed the clinical records and neuroimaging data of 5 consecutive AIDS patients who developed choreic movements at our center from January, 1994 to December, 1996. RESULTS: There were 2 cases of focal choreic dyskinesias, 1 of right hemichorea, and 2 of generalized chorea. Onset was acute and febrile in 1 case, and subacute in the other 4. In 1 patient the chorea was the AIDS onset symptom; in another choreic movements were the first neurological symptom following AIDS diagnosis; in 2 patients AIDS had a neurological onset other than chorea; and in the fifth patient buccofacial dyskinesias appeared following the development of bacterial encephalitis. CONCLUSION: Chorea was associated with cerebral toxoplasmosis in 2 patients, progressive multifocal leukoencephalopathy in 1, subacute HIV encephalopathy in another, and was probably iatrogenic in the last. Chorea is not unusual in AIDS, however the causes are variable and careful neuroradiological and clinical evaluation is required to identify them. AIDS-related disease should be considered in young patients presenting with chorea without a family history of movement disorders.

Acquired Immunodeficiency Syndrome↗

PubMed1993

Stereotactic biopsy in cerebral lesions of AIDS.

The experiences with stereotactic biopsies in 121 patients with AIDS compared to 142 non-AIDS patients are presented. In the AIDS group most of the tumors (38) were lymphomas (34). Other frequent diagnoses have been progressive multifocal leukoencephalopathy (20) and toxoplasmosis (16)--although most AIDS patients already had been treated for toxoplasmosis, and those who responded to it did not undergo biopsy. Initially among the AIDS patients there was a tendency of delayed intracranial bleeding (4 cases, 3 of them fatal). After initiation of a prophylactic coagulopathy protocol no other such complications have occurred in the following 70 biopsies.

Acquired Immunodeficiency Syndrome↗

PubMed1997

Postmortem MR imaging of the brains of patients with AIDS.

Forty-two postmortem formalin-fixed brains of known patients with AIDS were examined with T2-weighted MR imaging before brain cutting. The gross and microscopic brain findings were correlated with the T2 signal changes in the postmortem MR imaging. The brains included examples of progressive multifocal leukoencephalopathy with involvement of the central brain and cerebellum. The authors also encountered the coexistence of progressive multifocal leukoencephalopathy and HIV encephalitis, and the T2 signal changes for each were compared. The T2 signal changes of leptomeningeal and perivascular space cryptococcal infection and CMV ependymitis are documented. Several expressions of primary cerebral lymphoma, including large nodules, choroid plexus infiltration, and diffuse microscopic sites of tumor also are assessed.

AIDS-Related Opportunistic Infections↗

PubMed2004

[Correlation between clinical apparatus-based differential diagnosis and neuropathological diagnosis in patients with AIDS].

BACKGROUND: During the course of HIV infection, the majority of patients develop opportunistic cerebral neuro-manifestations. If conventional diagnostic tools are not sufficient, a stereotactic biopsy is often necessary. PATIENTS AND METHODS: In order to evaluate the correctness of the clinical diagnosis of cerebral neuro-manifestations in HIV-infected patients, we compared the results of cerebral biopsy or autopsy with the previous clinical diagnosis. A total of 19 biopsies and 49 autopsies could be analyzed. RESULTS: Except for HIV-associated encephalopathy, we detected a very high conformity between the clinical and the neuropathological diagnoses. We obtained the best sensitivity for progressive multifocal leukoencephalopathy (PML), whereas for cerebral toxoplasmosis the worst sensitivity and specificity was identified. CONCLUSION: We conclude that the diagnosis of PML can be made on clinical grounds alone, whereas the diagnosis of cerebral toxoplasmosis and lymphoma often requires a biopsy, which should be performed early.

AIDS Dementia Complex↗

PubMed1996

[Movement disorders and AIDS].

We describe the clinical characteristics, causes and response to treatment in 6 patients with AIDS who presented with abnormal movement disorders between January 1987 and July 1993 in our hospital, 3 with hemiballismus-hemichorea, 1 with athetosis, 1 myoclonia and 1 with "rubric" tremor. Brain imaging showed lesions in the corpus striatum in all the patients. Suspected diagnoses were cerebral toxoplasmosis in 4, cerebral lymphoma in 1 and progressive multifocal leukoencephalopathy in 1. The toxoplasmosis patients showed improvement (2 cases) or disappearance (2 cases) of movements with antiparasitic therapy. Treatment provided no benefit to the patients with leukoencephalopathy and lymphoma. Hemiballismus-hemichorea was the most common movement disorder in AIDS patients. The underlying cause is usually lesions in the basal ganglia arising from toxoplasmosis. If the lesions are so caused, movements may improve with antiparasitic therapy.

Acquired Immunodeficiency Syndrome↗

PubMedDate not supplied

Neuropathological findings in an autopsy series of Italian subjects with AIDS.

Lesions of the central nervous system were seen in 13/22 autopsies of Italian subjects affected by the acquired immune deficiency syndrome and including 19 intravenous drug addicts. Multinucleated cells similar to those originally described by Sharer et al. [1985] in response to direct central nervous system lesions by the human immune deficiency virus were seen in five subjects. Isolated or multiple opportunistic infections of the brain including cerebral toxoplasmosis (7 cases), progressive multifocal leukoencephalopathy (4), cytomegalovirus encephalitis (3), and tuberculous meningoencephalitis (2) were recognized. Finally, three cases of primitive cerebral lymphoma of large round cells were found. The central nervous system is a target, like the lymphoid tissue, of direct damage for the human immune deficiency virus, and it is frequently affected by opportunistic infections due to the immunological impairment sustained by the acquired immune deficiency syndrome.

Acquired Immunodeficiency Syndrome↗

PubMed1999

Multiplex polymerase chain reaction for the simultaneous detection and typing of polyomavirus JC, BK and SV40 DNA in clinical samples.

A novel multiplex nested PCR (nPCR) method was developed for detecting and differentiating simultaneously the DNA of polyomaviruses JC, BK and SV40 in a single tube. In the first amplification step the same set of primers were used to amplify a conserved DNA region of the large T antigen gene of JCV, BKV and SV40. The second round of multiplex nPCR was carried out using a set of primers designed to render products of different size for each related virus. The thermocycling parameters and concentration of each reaction component were optimised systematically to achieve optimal specificity and sensitivity for the nPCR assay. The sensitivity of the method ranged between one and 10 copies of polyomavirus genome. Cerebrospinal fluid (CSF) was examined from AIDS patients with clinical and neuroradiological evidence of progressive multifocal leukoencephalopathy (PML) and CSF from AIDS patients with other neurological alterations. Urine specimens from bone marrow transplant recipients affected by haemorrhagic cystitis were also tested. The results obtained suggest that the assay is a good tool for supporting the diagnosis of polyomavirus infection and could be used for epidemiological purposes and in other studies in order to define better the role of polyomaviruses in human disease.

Acquired Immunodeficiency Syndrome↗