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Inamullah et al. Neuroimmunol Neuroinflammation 2018;5:3 Neuroimmunology
DOI: 10.20517/2347-8659.2017.61 and Neuroinflammation

Case Report Open Access

Progressive multifocal leukoencephalitis in a patient
with sarcoidosis on hydroxychloroquine with

negative cerebrospinal fluid testing for the John
Cunningham virus

Ovais Inamullah , Casey Farin , Linh Tran , Roger McLendon , Mark Skeen , Joel Morgenlander 1
1
1
2
1
3
1 Neurology Department, Duke University Hospital, Durham, NC 27705, USA.
2 Child Neurology Department, Duke University Hospital, Durham, NC 27705, USA.
3 Pathology Department, Duke University Hospital, Durham, NC 27705, USA.
Correspondence to: Dr. Ovais Inamullah, Neurology Department, Duke University Hospital, 2301 Erwin Rd, Durham, NC 27705, USA.
E-mail: ovi@duke.edu
How to cite this article: Inamullah O, Farin C, Tran L, McLendon R, Skeen M, Morgenlander J. Progressive multifocal leukoencephalitis in
a patient with sarcoidosis on hydroxychloroquine with negative cerebrospinal fluid testing for the John Cunningham virus. Neuroimmunol
Neuroinflammation 2018;5:3. http://dx.doi.org/10.20517/2347-8659.2017.61
Received: 29 Nov 2017 First Decision: 22 Jan 2018 Revised: 24 Jan 2018 Accepted: 29 Jan 2018 Published: 31 Jan 2018

Science Editor: Athanassios P. Kyritsis Copy Editor: Jun-Yao Li Production Editor: Huan-Liang Wu

Abstract

Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system caused
by the John Cunningham (JC) virus typically seen in immuno-compromised patients. Several drugs that suppress that
immune system have already been known to cause PML such as natalizumab and rituximab. We present a patient with
sarcoidosis who develops PML in the rare setting of minimal immunosuppression with only hydroxychloroquine. There was
significant delay in the diagnosis due to negative cerebrospinal fluid testing for JC virus and concern for neuro-sarcoidosis,
but eventually a diagnosis of PML was made via brain biopsy.

Keywords: Progressive multifocal leukoencephalopathy, sarcoidosis, hydroxychloroquine, neuroimmunology

INTRODUCTION
Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous
system caused by the John Cunningham (JC) virus. Approximately 86% of adults have antibodies to the
JC virus, believed to be secondary to an asymptomatic childhood infection . When a patient becomes
[1]

© The Author(s) 2018. Open Access This article is licensed under a Creative Commons Attribution 4.0
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