Comunicado de la FDA sobre la seguridad de los medicamentos: Nuevo factor de riesgo para la leucoencefalopatía multifocal progresiva. Abstract. PASQUALOTTO, Alessandro Comarú; MATTOS, Alice J.Z. de and ROCHA, Marineide Melo. Leucoencefalopatia multifocal progressiva confirmada por. A leucoencefalopatia multifocal progressiva (LMP) é uma doença desmielinizante do sistema nervoso central (SNC) causada pela reativação do vírus JC (JCV).

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Progressive multifocal leukoencephalopathy in non-HIV patient

Leucoencffalopatia report the case of a year-old man with an indolent non-Hodgkin lymphoma stage IV diagnosed 15 months earlier. Since diagnosis, he had remained asymptomatic and in clinical follow-up, under no specific therapy.

During a follow-up visit, he reported speech and visual difficulties. Cerebrospinal fluid analysis was normal and multofocal tests for herpes virus, syphilis, toxoplasmosis and HIV were all negative. Stereotactic biopsy was performed and the anatomopathological examination revealed areas of intense demyelination with presence of large oligodendrocytes Immunohistochemistry staining on brain biopsy demonstrated JC virus DNA, characterizing progressive multifocal leukoencephalopathy PML.

Despite the support therapy, the patient evolved with clinical and radiological deterioration Figure 3.

It is caused by the reactivation of the JC virus genus: The virus remains latent mainly in the kidneys and lymphoid organs and is reactivated and spreads to the brain, almost exclusively in the setting of advanced immunosuppression.

Once reactivated in the CNS, the virus infects and destroys oligodendrocytes, which are responsible for the formation and maintenance of myelin leucoencefaoopatia. However, other causes of immunosuppression such as organ transplantation, chemotherapy, immunotherapies with monoclonal antibodies, autoimmune diseases and lymphoma, may also be associated with PML.

The clinical picture of PML varies according to the patterns of demyelination. Patients may experience changes in cognition, languagebehavior and personality.


Progressive multifocal leukoencephalopathy

Memory loss, visual deficits such as hemianopsia and cortical blindness, sensory-motor alterations, and generalized neurological decline, may also occur. The presence of seizures, vertigo, headache and aphasia is less frequent. Cerebellar symptoms may appear in the case of lesion in the posterior fossa. Rarely, there is involvement of the spinal cord.

In general, demyelination occurs in a multifocal and asymmetric manner. The lesions typically start at the cortical junction between the gray and white matter, with concentric dissemination.

They can be variable in size and may coalesce. There is a predilection for the parieto-occipital lobes. The areas are small in the beginning, increasing progressively, with a tendency for confluence. It is commonly multifocal and has an asymmetric pattern of demyelinationuni- or bilaterally, without mass effect, and invariably without contrast enhancement. The subcortical lesions tend to involve U-fibers. Any lobe of the brain may be affected, but there is preference for the frontal and parieto-occipital regions.

There may be involvement of deep gray structures, by affection of myelin fibers that pass through basal ganglia. PML can be differentiated from these diseases by its slower temporal evolution, fewer systemic manifestations, absence of fever, preservation of conscience and lack of mass effect, perilesional edema or contrast enhancement on imaging tests.

Herpes virus encephalitis should be considered in the initial phase, but white matter involvement is frequently temporal, bilateral and asymmetrical, with greater contrast enhancement than that found in PML. PML lesions are more often multifocal and asymmetric, with greater predilection for the subcortical white matter and are associated with more pronounced signal change on T1-weighted images, while in HIV-associated demyelination, lesions are often isointense on T1, and may not be visible.

Progressive multifocal leukoencephalopathy – Wikipedia

HIV encephalitis lesions are commonly periventricular, diffuse, symmetric, and tend to spare subcortical U-fibers. Clinically, HIV-associated demyelination presents as global cognitive disorder and dementia, while in PML motor, sensory or cognitive focal deficits predominate.


The prognosis for PML is reserved and there is no specific therapy. Improvement in immunosuppression seems to be beneficial and can leucoencefalopstia to stabilization or regression of the disease in follow-up on imaging exams. National Center for Biotechnology InformationU.

Journal List Dement Neuropsychol v. Author information Article notes Copyright and License information Disclaimer. Gislaine Cristina Lopes Machado Porto.

Received Mar 5; Accepted May This is an open-access article distributed under the terms of the Creative Commons Attribution License.

Open in a separate window. T1-weighted image post-contrast reveals cortical-subcortical hypointensity in left temporal lobe, without enhancement white arrow. T2-weighted image reveals asymmetrical areas of hyperintensity, more marked in the left temporal region white arrows. FLAIR image reveals multifocal and asymmetric progression of PML, with involvement of the left temporal-occipital lobes white arrow and of the subcortical region of the right temporal lobe U-fibers black arrow.

Previous biopsy area thin white arrow. Progressive multifocal leukoencephalopathy and other disorders caused by JC virus clinical features and pathogenesis. Inflammatory progressive multifocal leukoencephalopathy in human immunodeficiency virus-negative patients. Incidence of progressive multifocal leukoencephalopathy in patients without HIV.

Progressive multifocal leukoencephalopathy after rituximab therapy in HIV-negative patients a report of 57 cases from the Research on Adverse Drug Events and Reports project.

Progressive multifocal leukoencephalopathy a review of the neuroimaging features and differential diagnosis. Progressive multifocal leukoencephalopathy value of diffusion-weighted and contrast-enhanced magnetic resonance imaging for diagnosis and treatment control.

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