Progressive Multifocal Leukoencephalopathy in a HIV Negative, Immunocompetent Patient

Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease most common in immunodeficient patients. It occurs due to reactivation of the John Cunningham Virus (JCV) and carries a poor prognosis, with a median life expectancy of 6 months. We report a case of a 66-year-old man wi...

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Main Author: T. Nanda
Format: Article
Language:English
Published: Wiley 2016-01-01
Series:Case Reports in Neurological Medicine
Online Access:http://dx.doi.org/10.1155/2016/7050613
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author T. Nanda
author_facet T. Nanda
author_sort T. Nanda
collection DOAJ
description Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease most common in immunodeficient patients. It occurs due to reactivation of the John Cunningham Virus (JCV) and carries a poor prognosis, with a median life expectancy of 6 months. We report a case of a 66-year-old man with a history of HCV related cirrhosis (HCV) and hepatocellular carcinoma (HCC) who was found to have PML in the setting of a negative viral load in the CSF and a CD4+ >200. He initially presented with two weeks of mild confusion and word-finding difficulty concerning for hepatic encephalopathy. An MRI was notable for extensive T2/FLAIR hyperintensity signal in the left temporal lobe. Brain biopsy was positive for JCV. PML is rare in immunocompetent individuals, especially in the setting of a negative viral load. It is possible, however, that transient states of immunosuppression may have been responsible in this case. Although viral load was reported as negative, virus may still have been detected but was below the quantifiable threshold. It is important for clinicians to note that a negative result does not necessarily exclude the possibility of PML, and care should be taken to review lab values on viral load in closer detail.
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spelling doaj-art-d78e16e13eaf4e0a9b09950caebe01892025-08-20T02:20:09ZengWileyCase Reports in Neurological Medicine2090-66682090-66762016-01-01201610.1155/2016/70506137050613Progressive Multifocal Leukoencephalopathy in a HIV Negative, Immunocompetent PatientT. Nanda0Columbia College of Physicians and Surgeons, Columbia University Medical Center, 161 Fort Washington Avenue, New York, NY 10032, USAProgressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease most common in immunodeficient patients. It occurs due to reactivation of the John Cunningham Virus (JCV) and carries a poor prognosis, with a median life expectancy of 6 months. We report a case of a 66-year-old man with a history of HCV related cirrhosis (HCV) and hepatocellular carcinoma (HCC) who was found to have PML in the setting of a negative viral load in the CSF and a CD4+ >200. He initially presented with two weeks of mild confusion and word-finding difficulty concerning for hepatic encephalopathy. An MRI was notable for extensive T2/FLAIR hyperintensity signal in the left temporal lobe. Brain biopsy was positive for JCV. PML is rare in immunocompetent individuals, especially in the setting of a negative viral load. It is possible, however, that transient states of immunosuppression may have been responsible in this case. Although viral load was reported as negative, virus may still have been detected but was below the quantifiable threshold. It is important for clinicians to note that a negative result does not necessarily exclude the possibility of PML, and care should be taken to review lab values on viral load in closer detail.http://dx.doi.org/10.1155/2016/7050613
spellingShingle T. Nanda
Progressive Multifocal Leukoencephalopathy in a HIV Negative, Immunocompetent Patient
Case Reports in Neurological Medicine
title Progressive Multifocal Leukoencephalopathy in a HIV Negative, Immunocompetent Patient
title_full Progressive Multifocal Leukoencephalopathy in a HIV Negative, Immunocompetent Patient
title_fullStr Progressive Multifocal Leukoencephalopathy in a HIV Negative, Immunocompetent Patient
title_full_unstemmed Progressive Multifocal Leukoencephalopathy in a HIV Negative, Immunocompetent Patient
title_short Progressive Multifocal Leukoencephalopathy in a HIV Negative, Immunocompetent Patient
title_sort progressive multifocal leukoencephalopathy in a hiv negative immunocompetent patient
url http://dx.doi.org/10.1155/2016/7050613
work_keys_str_mv AT tnanda progressivemultifocalleukoencephalopathyinahivnegativeimmunocompetentpatient