Background: Progressive multifocal leukoencephalopathy is a fatal demyelinating condition due to reactivation of latent JC virus in cerebral white matter. Its prevalence is 1in 200,000 and extremely rare in immunocompetent individuals. It can mimic subacute stroke, brain tumours and other demyelinating conditions which have different outcomes. PML typically occurs in an immunocompromised patient, where the archetype JC virus gains pathogenic potential and initiate oligodendroglial inflammation. Currently, the immune reconstitution is considered as the treatment of choice; however, paradoxical worsening with IRIS PML is a significant challenge. There is limited evidence available on how to manage PML in immune competent patients. Direct antiviral agents have no convincing evidence to-date. There is anecdotal evidence that IL-2, filgrastim, and vaccination may be helpful. Case presentation: A 74-year-old man presented with right-sided weakness and dysphasia. He was initially managed as having subacute stroke based on imaging and clinical findings. He subsequently deteriorated, triggering to revisit of the original diagnosis and repeat imaging. He underwent extensive workup, including lumbar puncture and JC viral testing. He was commenced on Mirtazapine to prevent JC viral spread; however, he later passed away. A subsequent post-mortem brain biopsy confirmed the progressive leukoencephalopathy. Conclusion: Even though extremely rare, progressive multifocal leukoencephalopathy should be considered in the differential diagnosis of progressive neurological conditions. It is essential to rule out other treatable conditions as progressive multifocal leukoencephalopathy has a fatal outcome invariably.
| Published in | American Journal of Psychiatry and Neuroscience (Volume 9, Issue 1) |
| DOI | 10.11648/j.ajpn.20210901.13 |
| Page(s) | 15-18 |
| Creative Commons |
This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
| Copyright |
Copyright © The Author(s), 2021. Published by Science Publishing Group |
Progressive Multifocal Leukoencephalopathy, JC Virus, Immunocompetent
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APA Style
Nisahan Balasingam, Zhi Yang Siow, Athula Kosgallana, Tom Robertson. (2021). An Unusual Case of Progressive Multifocal Leukoencephalopathy in an Immunocompetent Patient Masquerading as a Stroke. American Journal of Psychiatry and Neuroscience, 9(1), 15-18. https://doi.org/10.11648/j.ajpn.20210901.13
ACS Style
Nisahan Balasingam; Zhi Yang Siow; Athula Kosgallana; Tom Robertson. An Unusual Case of Progressive Multifocal Leukoencephalopathy in an Immunocompetent Patient Masquerading as a Stroke. Am. J. Psychiatry Neurosci. 2021, 9(1), 15-18. doi: 10.11648/j.ajpn.20210901.13
AMA Style
Nisahan Balasingam, Zhi Yang Siow, Athula Kosgallana, Tom Robertson. An Unusual Case of Progressive Multifocal Leukoencephalopathy in an Immunocompetent Patient Masquerading as a Stroke. Am J Psychiatry Neurosci. 2021;9(1):15-18. doi: 10.11648/j.ajpn.20210901.13
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Download@article{10.11648/j.ajpn.20210901.13,
author = {Nisahan Balasingam and Zhi Yang Siow and Athula Kosgallana and Tom Robertson},
title = {An Unusual Case of Progressive Multifocal Leukoencephalopathy in an Immunocompetent Patient Masquerading as a Stroke},
journal = {American Journal of Psychiatry and Neuroscience},
volume = {9},
number = {1},
pages = {15-18},
doi = {10.11648/j.ajpn.20210901.13},
url = {https://doi.org/10.11648/j.ajpn.20210901.13},
eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajpn.20210901.13},
abstract = {Background: Progressive multifocal leukoencephalopathy is a fatal demyelinating condition due to reactivation of latent JC virus in cerebral white matter. Its prevalence is 1in 200,000 and extremely rare in immunocompetent individuals. It can mimic subacute stroke, brain tumours and other demyelinating conditions which have different outcomes. PML typically occurs in an immunocompromised patient, where the archetype JC virus gains pathogenic potential and initiate oligodendroglial inflammation. Currently, the immune reconstitution is considered as the treatment of choice; however, paradoxical worsening with IRIS PML is a significant challenge. There is limited evidence available on how to manage PML in immune competent patients. Direct antiviral agents have no convincing evidence to-date. There is anecdotal evidence that IL-2, filgrastim, and vaccination may be helpful. Case presentation: A 74-year-old man presented with right-sided weakness and dysphasia. He was initially managed as having subacute stroke based on imaging and clinical findings. He subsequently deteriorated, triggering to revisit of the original diagnosis and repeat imaging. He underwent extensive workup, including lumbar puncture and JC viral testing. He was commenced on Mirtazapine to prevent JC viral spread; however, he later passed away. A subsequent post-mortem brain biopsy confirmed the progressive leukoencephalopathy. Conclusion: Even though extremely rare, progressive multifocal leukoencephalopathy should be considered in the differential diagnosis of progressive neurological conditions. It is essential to rule out other treatable conditions as progressive multifocal leukoencephalopathy has a fatal outcome invariably.},
year = {2021}
}
TY - JOUR T1 - An Unusual Case of Progressive Multifocal Leukoencephalopathy in an Immunocompetent Patient Masquerading as a Stroke AU - Nisahan Balasingam AU - Zhi Yang Siow AU - Athula Kosgallana AU - Tom Robertson Y1 - 2021/03/22 PY - 2021 N1 - https://doi.org/10.11648/j.ajpn.20210901.13 DO - 10.11648/j.ajpn.20210901.13 T2 - American Journal of Psychiatry and Neuroscience JF - American Journal of Psychiatry and Neuroscience JO - American Journal of Psychiatry and Neuroscience SP - 15 EP - 18 PB - Science Publishing Group SN - 2330-426X UR - https://doi.org/10.11648/j.ajpn.20210901.13 AB - Background: Progressive multifocal leukoencephalopathy is a fatal demyelinating condition due to reactivation of latent JC virus in cerebral white matter. Its prevalence is 1in 200,000 and extremely rare in immunocompetent individuals. It can mimic subacute stroke, brain tumours and other demyelinating conditions which have different outcomes. PML typically occurs in an immunocompromised patient, where the archetype JC virus gains pathogenic potential and initiate oligodendroglial inflammation. Currently, the immune reconstitution is considered as the treatment of choice; however, paradoxical worsening with IRIS PML is a significant challenge. There is limited evidence available on how to manage PML in immune competent patients. Direct antiviral agents have no convincing evidence to-date. There is anecdotal evidence that IL-2, filgrastim, and vaccination may be helpful. Case presentation: A 74-year-old man presented with right-sided weakness and dysphasia. He was initially managed as having subacute stroke based on imaging and clinical findings. He subsequently deteriorated, triggering to revisit of the original diagnosis and repeat imaging. He underwent extensive workup, including lumbar puncture and JC viral testing. He was commenced on Mirtazapine to prevent JC viral spread; however, he later passed away. A subsequent post-mortem brain biopsy confirmed the progressive leukoencephalopathy. Conclusion: Even though extremely rare, progressive multifocal leukoencephalopathy should be considered in the differential diagnosis of progressive neurological conditions. It is essential to rule out other treatable conditions as progressive multifocal leukoencephalopathy has a fatal outcome invariably. VL - 9 IS - 1 ER -
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