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Posterior fossa progressive multifocal leukoencephalopathy: first presentation of an unknown autoimmune disease

We present a case of a 57-year-old man who presented with progressive cerebellar dysarthria and cerebellar ataxia. Additional investigations confirmed the diagnosis of progressive multifocal leukoencephalopathy (PML) in the posterior fossa. This is a demyelinating disease of the central nervous syst...

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Autores principales: Scholten, Paulette, Kralt, Peter, Jacobs, Bram
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BMJ Publishing Group 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5652366/
https://www.ncbi.nlm.nih.gov/pubmed/29025774
http://dx.doi.org/10.1136/bcr-2017-220990
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author Scholten, Paulette
Kralt, Peter
Jacobs, Bram
author_facet Scholten, Paulette
Kralt, Peter
Jacobs, Bram
author_sort Scholten, Paulette
collection PubMed
description We present a case of a 57-year-old man who presented with progressive cerebellar dysarthria and cerebellar ataxia. Additional investigations confirmed the diagnosis of progressive multifocal leukoencephalopathy (PML) in the posterior fossa. This is a demyelinating disease of the central nervous system, caused by an opportunistic infection with John Cunningham virus. PML has previously been considered a lethal condition, but because of careful monitoring of patients with HIV and of patients using immunosuppressive drugs it is discovered in earlier stages and prognosis can be improved. Our patient had no known immune-compromising state, but further work-up revealed that the PML was most likely the first presentation of a previous untreated autoimmune disorder: sarcoidosis.
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spelling pubmed-56523662017-10-25 Posterior fossa progressive multifocal leukoencephalopathy: first presentation of an unknown autoimmune disease Scholten, Paulette Kralt, Peter Jacobs, Bram BMJ Case Rep Unusual Presentation of More Common Disease/Injury We present a case of a 57-year-old man who presented with progressive cerebellar dysarthria and cerebellar ataxia. Additional investigations confirmed the diagnosis of progressive multifocal leukoencephalopathy (PML) in the posterior fossa. This is a demyelinating disease of the central nervous system, caused by an opportunistic infection with John Cunningham virus. PML has previously been considered a lethal condition, but because of careful monitoring of patients with HIV and of patients using immunosuppressive drugs it is discovered in earlier stages and prognosis can be improved. Our patient had no known immune-compromising state, but further work-up revealed that the PML was most likely the first presentation of a previous untreated autoimmune disorder: sarcoidosis. BMJ Publishing Group 2017-10-11 /pmc/articles/PMC5652366/ /pubmed/29025774 http://dx.doi.org/10.1136/bcr-2017-220990 Text en © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted. This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
spellingShingle Unusual Presentation of More Common Disease/Injury
Scholten, Paulette
Kralt, Peter
Jacobs, Bram
Posterior fossa progressive multifocal leukoencephalopathy: first presentation of an unknown autoimmune disease
title Posterior fossa progressive multifocal leukoencephalopathy: first presentation of an unknown autoimmune disease
title_full Posterior fossa progressive multifocal leukoencephalopathy: first presentation of an unknown autoimmune disease
title_fullStr Posterior fossa progressive multifocal leukoencephalopathy: first presentation of an unknown autoimmune disease
title_full_unstemmed Posterior fossa progressive multifocal leukoencephalopathy: first presentation of an unknown autoimmune disease
title_short Posterior fossa progressive multifocal leukoencephalopathy: first presentation of an unknown autoimmune disease
title_sort posterior fossa progressive multifocal leukoencephalopathy: first presentation of an unknown autoimmune disease
topic Unusual Presentation of More Common Disease/Injury
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5652366/
https://www.ncbi.nlm.nih.gov/pubmed/29025774
http://dx.doi.org/10.1136/bcr-2017-220990
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