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Progressive multifocal leukoencephalopathy: a challenging diagnosis established at autopsy
Progressive multifocal leukoencephalopathy (PML) is a feared entity that occurs most frequently in conditions of extreme immunodeficiency. The diagnosis is often made long after the onset of symptoms due to the physicians’ unfamiliarity, and the unavailability of diagnostic tests in some medical cen...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
São Paulo, SP: Universidade de São Paulo, Hospital Universitário
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6394363/ https://www.ncbi.nlm.nih.gov/pubmed/30863734 http://dx.doi.org/10.4322/acr.2018.063 |
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author | Lopes, Cesar Castello Branco Crivillari, Murillo Prado, José Carlos Mann Ferreira, Cristiane Rubia dos Santos, Pedro José Takayasu, Vilma Laborda, Lorena Silva |
author_facet | Lopes, Cesar Castello Branco Crivillari, Murillo Prado, José Carlos Mann Ferreira, Cristiane Rubia dos Santos, Pedro José Takayasu, Vilma Laborda, Lorena Silva |
author_sort | Lopes, Cesar Castello Branco |
collection | PubMed |
description | Progressive multifocal leukoencephalopathy (PML) is a feared entity that occurs most frequently in conditions of extreme immunodeficiency. The diagnosis is often made long after the onset of symptoms due to the physicians’ unfamiliarity, and the unavailability of diagnostic tests in some medical centers. Although the incidence of PML is decreasing among HIV patients with the advent of highly active antiretroviral therapy (HAART), in Brazil this entity is the fourth highest neurological complication among these patients. The authors present the case of a middle-aged man who tested positive for HIV concomitantly with the presentation of hyposensitivity in the face and the right side of the body, accompanied by mild weakness in the left upper limb. The clinical features worsened rapidly within a couple of weeks. The diagnostic work-up pointed to the working diagnosis of PML after brain magnetic resonance imaging; however, the detection of the John Cunningham virus (JCV) in the cerebral spinal fluid was negative. HAART was started but the patient died after 7 weeks of hospitalization. The autopsy revealed extensive multifocal patchy areas of demyelination in the white matter where the microscopy depicted demyelination, oligodendrocytes alterations, bizarre atypical astrocytes, and perivascular lymphocytic infiltration. The immunohistochemistry was positive for anti-SV40, and the polymerase chain reaction of the brain paraffin-embedded tissue was positive for JCV. The authors highlight the challenges for diagnosing PML, as well as the devastating outcome of PML among HIV patients. |
format | Online Article Text |
id | pubmed-6394363 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | São Paulo, SP: Universidade de São Paulo, Hospital Universitário |
record_format | MEDLINE/PubMed |
spelling | pubmed-63943632019-03-12 Progressive multifocal leukoencephalopathy: a challenging diagnosis established at autopsy Lopes, Cesar Castello Branco Crivillari, Murillo Prado, José Carlos Mann Ferreira, Cristiane Rubia dos Santos, Pedro José Takayasu, Vilma Laborda, Lorena Silva Autops Case Rep Article / Autopsy Case Report Progressive multifocal leukoencephalopathy (PML) is a feared entity that occurs most frequently in conditions of extreme immunodeficiency. The diagnosis is often made long after the onset of symptoms due to the physicians’ unfamiliarity, and the unavailability of diagnostic tests in some medical centers. Although the incidence of PML is decreasing among HIV patients with the advent of highly active antiretroviral therapy (HAART), in Brazil this entity is the fourth highest neurological complication among these patients. The authors present the case of a middle-aged man who tested positive for HIV concomitantly with the presentation of hyposensitivity in the face and the right side of the body, accompanied by mild weakness in the left upper limb. The clinical features worsened rapidly within a couple of weeks. The diagnostic work-up pointed to the working diagnosis of PML after brain magnetic resonance imaging; however, the detection of the John Cunningham virus (JCV) in the cerebral spinal fluid was negative. HAART was started but the patient died after 7 weeks of hospitalization. The autopsy revealed extensive multifocal patchy areas of demyelination in the white matter where the microscopy depicted demyelination, oligodendrocytes alterations, bizarre atypical astrocytes, and perivascular lymphocytic infiltration. The immunohistochemistry was positive for anti-SV40, and the polymerase chain reaction of the brain paraffin-embedded tissue was positive for JCV. The authors highlight the challenges for diagnosing PML, as well as the devastating outcome of PML among HIV patients. São Paulo, SP: Universidade de São Paulo, Hospital Universitário 2019-01-14 /pmc/articles/PMC6394363/ /pubmed/30863734 http://dx.doi.org/10.4322/acr.2018.063 Text en Autopsy and Case Reports. ISSN 2236-1960. Copyright © 2019. http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium provided the article is properly cited. |
spellingShingle | Article / Autopsy Case Report Lopes, Cesar Castello Branco Crivillari, Murillo Prado, José Carlos Mann Ferreira, Cristiane Rubia dos Santos, Pedro José Takayasu, Vilma Laborda, Lorena Silva Progressive multifocal leukoencephalopathy: a challenging diagnosis established at autopsy |
title | Progressive multifocal leukoencephalopathy: a challenging diagnosis established at autopsy |
title_full | Progressive multifocal leukoencephalopathy: a challenging diagnosis established at autopsy |
title_fullStr | Progressive multifocal leukoencephalopathy: a challenging diagnosis established at autopsy |
title_full_unstemmed | Progressive multifocal leukoencephalopathy: a challenging diagnosis established at autopsy |
title_short | Progressive multifocal leukoencephalopathy: a challenging diagnosis established at autopsy |
title_sort | progressive multifocal leukoencephalopathy: a challenging diagnosis established at autopsy |
topic | Article / Autopsy Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6394363/ https://www.ncbi.nlm.nih.gov/pubmed/30863734 http://dx.doi.org/10.4322/acr.2018.063 |
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