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Stereotactic brain biopsy for diagnosis of progressive multifocal leukoencephalopathy.

We describe three patients in whom the diagnosis of progressive multifocal leukoencephalopathy (PML) was made by stereotactic biopsy. All three patients had a rapidly progressive clinical course pathologically characterized by demyelination, Alzheimer I astrocytes, and basophilic intranuclear inclusions in oligodendrocytes. In each case, the diagnosis was confirmed with immunohistochemical staining of oligodendrocytes for the JC virus and in one case by electron microscopy. Determination of the adequacy of tissue received at the time of frozen section is critical and can direct the appropriate…

Progressive Multifocal Leukoencephalopathy Associated With Idiopathic CD8+ Lymphocytopenia.

Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system (CNS) caused by the polyoma … . Although the first cerebrospinal fluid analysis was negative, the second one was positive for the JC virus. Furthermore, due to radiological and clinical progression, mirtazapine was started and the patient underwent a course of intravenous immunoglobulin, with no response. In parallel, causes of immunosuppression were investigated, which led to the diag …

[Progressive Multifocal Leukoencephalopathy].

Progressive multifocal leukoencephalopathy (PML) is a central nervous system infection caused by reactivation of the JC polyomavirus (JCV). Its diagnosis relies on clinical symptoms, brain magnetic resonance imaging findings, detection of JCV in the cerebrospinal fluid, exclusion of other diagnoses, and histopathological examination. In PML patients with human immunodeficiency virus (HIV) infection, antiretroviral therapy is fundamental. In patients with non-HIV PML, various treatments including combination therapy with mefloquine and mirtazapine, as well as new antiviral drugs, have been atte

Cerebellar form of progressive multifocal leukoencephalopathy in a patient with chronic renal failure.

Progressive multifocal leukoencephalopathy (PML) developed in a 64-year-old woman who had undergone hemodialysis treatment due to chronic renal failure (CRF) for 6 yr. Her initial symptom was ataxia, and computed tomographic (CT) scan and magnetic resonance imaging (MRI) suggested a demyelinating disease of the cerebellum. Her cell-mediated immunity was impaired. At autopsy, the cerebellar disease was confirmed as PML by ultrastructural and immunohistochemical studies. Moreover, the JC type of papova virus infection was verified by Southern blot analysis.

Childhood case of progressive multifocal leukoencephalopathy with improved clinical outcome.

A 6-year-old boy who had been in remission from acute lymphoblastic leukemia for 2.5 years presented with seizures, hemiparesis, visual loss, and white- and gray-matter lesions on cranial magnetic resonance imaging. The diagnosis of progressive multifocal leukoencephalopathy was established on the detection of JC virus DNA by polymerase chain reaction in brain tissue. The patient was administered several anticonvulsants, amantadine, acyclovir, and ganciclovir. He showed partial recovery. This case illustrates the possibility of long-term survival in progressive multifocal leukoencephalopathy w…

Neuroimaging of AIDS. I. Viral infections.

Viral infections of the brain and spinal cord cause significant morbidity and mortality in patients afflicted with AIDS. Debate continues over the specific mechanisms and pathways of how HIV-1 manifests itself in the brain and spinal cord. Attempts to predict which seropositive patients develop neurocognitive deficits caused by HIV-1 and how soon these deficits will occur in the course of disease have had limited success. The neuropathologic changes of HIV-1 must be distinguished from other viral infections, such as cytomegalovirus, JC papovavirus (progressive multifocal leukoencephalopathy),

Brain tumors and polyomaviruses.

Polyomaviruses, including JC virus (JCV), BK virus (BKV), and simian virus 40 (SV40) have attracted much attention in the past decade due to their repeated isolation from various human tumors, including those originating from the central nervous system (CNS). JCV and BKV are considered to be ubiquitous human pathogens that become reactivated under impaired physiological conditions such as immunosuppression. Productive replication of JCV and BKV induces diseases such as progressive multifocal leukoencephalopathy in the brain and hemorrhagic or nonhemorrhagic cystitis and nephritis in the kidney…

HIV-associated PML: changing epidemiology and clinical approach.

Despite the availability of highly active antiretroviral therapy (HAART) for the treatment of human immunodeficiency virus (HIV) infection, … rebellar degeneration has been described in association with HIV infection in patients positive for JC virus, the papovavirus responsible for PML. The standard of care for HIV-associated PML is HAART to achieve immunologic recovery and optimal HIV virologic control. The prognosis of PML has improved greatly since the advent of HAART.

[Progressive multifocal leukoencephalopathy].

Progressive multifocal leukoencephalopathy (PML) is the only polyomavirus infection in human caused by JC virus. Clinically PML is diagnosed by neurological signs, supported by MRI and PET, but its pathologic diagnosis at brain biopsy remains to be a confirmative method, although the PCR method, using csf, gives a predictive value. Virological study of the regulatory region of viral genome has suggested that PML-inducing viral type may be derived from the urine-excreted virus (archetype) by mechanisms involving nucleotide deletion and duplication. Viral neurotropism has been shown to be closel…

Successful treatment of non-HIV progressive multifocal leukoencephalopathy: case report and literature review.

BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is a subacute onset demyelinating disease caused by JC virus and characterized by multifocal involvement of the subcortical white matter and cerebellar hemispheres or peduncles on magnetic resonance imaging (MRI). However, non-HIV PML patients with brain lesions limited to the cerebellum and brainstem have not been well characterized. METHODS: We report a 68-year-old man with systemic lupus erythematosus under treatment with immunosuppressants who developed non-HIV PML with brain lesions limited to the cerebellum and brainstem and s…

[A case of progressive multifocal leukoencephalopathy associated with systemic lupus erythematosus].

We describe a case of systemic lupus erythematosus (SLE) complicated with multifocal leukoencephalopathy (PML). A 57-year old woman, who had … emyelinating lesions with the presence of JC viral antigen. Polymerase chain reaction also revealed JC virus DNA in the cerebrospinal fluid. Her condition gradually progressed, and she died a year later due to pneumonia. Although acquired immunodeficiency syndrome is currently the most common disease associated with PML, patients with autoimmune diseases r …

Progressive multifocal leukoencephalopathy in an HIV patient was diagnosed by 3 times lumbar punctures and 2 times brain biopsies.

Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease of the central nervous system caused by JC virus (JCV) and is difficult to diagnose. We report on a male HIV-positive patient with PML finally diagnosed by 3 times lumbar punctures and 2 times brain biopsies. Negative results of JCV-PCR in cerebrospinal fluid (CSF) do not rule out the diagnosis of PML when clinical manifestations and neuroimaging features suspected PML. It is necessary to obtain new CSF and make repeat tests and even perform brain biopsy.

Progressive multifocal leukoencephalopathy developed in incomplete Heerfordt syndrome, a rare manifestation of sarcoidosis, without steroid therapy responding to cidofovir.

Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system caused by the JC virus; the mortality rate is high and it is usually refractory to treatment. In non-HIV patients, PML occurs as a late consequence of hematologic malignancies or during prolonged immunosuppression for transplantation or autoimmune disease. We describe a 34-year-old PML patient with incomplete Heerfordt syndrome, a rare type of sarcoidosis, who had not received any immunosuppressants, including steroids, at the onset and who was clinically and radiologically responsi…

Virus-specific T-cell therapies for patients with primary immune deficiency.

Viral infections are common and are potentially life-threatening in patients with moderate to severe primary immunodeficiency disorders. Bec … nt published studies have successfully targeted additional pathogens, including HHV6, BK virus, and JC virus. Though most studies have used VSTs derived from allogenic stem cell donors, the use of banked VSTs derived from partially HLA-matched donors has shown efficacy in multicenter settings. Hence, this approach could shorten the time for patients to rec …

Detection of polyomaviral DNA in clinical samples from immunocompromised patients: correlation with clinical disease.

Clinical samples from immunocompromised patients were screened for polyomaviral sequences by nested polymerase chain reaction (PCR) to evaluate the association of these viral infections with progressive multifocal leukoencephalopathy (PML). JC virus (JCV) DNA was detected in 19 of 23 CSF samples and all four brain samples from patients with PML. Neither BK virus (BKV) nor simian virus 40 (SV40) DNA were detected in these samples. No evidence was found to support the hypothesis that polyomaviral DNA is present in the central nervous system of immunosuppressed patients without PML (CSF n = 67, b…

History and current concepts in the pathogenesis of PML.

The JC virus (JCV), first described in 1971, is responsible for initiation of progressive multifocal leukoencephalopathy (PML), a disease characterized by demyelinating plaques and a classic triad of symptoms consisting of cognitive impairment, visual deficits, and motor dysfunction. To establish a diagnosis of PML, evidence of the presence of JCV DNA in pathologic tissue is necessary. The host range for productive infection of JCV is controlled by factors in the cell nucleus that bind to the viral promoter, initiating transcription of mRNA for the coordinated synthesis of viral proteins. Olig…

Comprehensive proteomic analysis of JC polyomavirus-infected human astrocytes and their extracellular vesicles.

Progressive multifocal leukoencephalopathy is a crimpling demyelinating disease of the central nervous system caused by JC polyomavirus (JCPyV). Much about JCPyV propagation in the brain remains obscure because of a lack of proper animal models to study the virus in the context of the disease, thus hampering efforts toward the development of new antiviral strategies. Here, having established a robust and representative model of JCPyV infection in human-induced pluripotent stem cell-derived astrocytes, we are able to fully characterize the effect of JCPyV on the biology of the cells and show th

Прогрессивная многофукальная лейкоэнцефалопатия на диметилфумарате с сохраняющимся количеством лимфоцитов, но глубоким истощением Т-клеток.

: Прогрессивная многофукальная лейкоэнцефалопатия (ПМЛ) у многочисленных склерозов (МС) пациентов, получающих диметилфумарат (ДМФ), связана с иатрогенной лимфопенией, преобладающей на CD8+T-клетках. ЦЕЛИ И МЕТОДЫ: Мы сообщаем о необычайном случае ДМВ в 66-летнем пациенте MS с сохраненным количеством лимфоцитов (nadir: 810/мм3) и нормальным количеством CD8+T-клеток. Результаты: Было обнаружено массированное перевыражение ингибиторного рецептора Программируемой Клеточной Смерти 1 (PD-1) на CD8+ и эффектом памяти Т-клеток вместе с поврежденным антиJC вирусом (JCV) специфической реакцией Т-клеток,

Совпадение в аннотации оригинала
BACKGROUND: Progressive multifocal leukoencephalopathy (PML) among multiple sclerosis (MS) patients receiving dimethyl fumarate (DMF) is ass … r Programmed Cell Death 1 (PD-1) on CD8+ and memory effector T-cells together with an impaired anti-JC virus (JCV) specific T-cells response were found, compatible with exhaustion. Following DMF withdrawal, PML progressively regressed, PD-1 was downregulated, and a functional anti-JCV response was established. CONCLUSION: T-cells exhaustion may favor PML …