Management and outcome of CSF-JC virus PCR-negative PML in a natalizumab-treated patient with MS
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Abstract
Objective: To describe the diagnosis and management of a 49-year-old woman with multiple sclerosis (MS) developing a progressive hemiparesis and expanding MRI lesion suspicious of progressive multifocal leukoencephalopathy (PML) 19 months after starting natalizumab.
Results: Polyomavirus JC (JCV)–specific qPCR in CSF was repeatedly negative, but JCV-specific antibodies indicated intrathecal production. Brain biopsy tissue taken 17 weeks after natalizumab discontinuation and plasmapheresis was positive for JCV DNA with characteristic rearrangements of the noncoding control region, but histology and immunohistochemistry were not informative except for pathologic features compatible with immune reconstitution inflammatory syndrome. A total of 22 months later, the clinical status had returned close to baseline level paralleled by marked improvement of neuroradiologic abnormalities.
Conclusions: This case illustrates diagnostic challenges in the context of incomplete suppression of immune surveillance and the potential of recovery of PML associated with efficient immune function restitution.
Glossary
- GLOSSARY
- AI=
- antibody index;
- EDSS=
- Expanded Disability Status Scale;
- IgG=
- immunoglobulin G;
- IRIS=
- immune reconstitution inflammatory syndrome;
- IVIg=
- IV immunoglobulin;
- JCV=
- polyomavirus JC;
- MS=
- multiple sclerosis;
- NTZ=
- natalizumab;
- PML=
- progressive multifocal leukoencephalopathy;
- rr-NCCR=
- rearranged noncoding control region;
- RRMS=
- relapsing-remitting multiple sclerosis
Footnotes
Study funding: Assays performed at the NINDS/NIH are funded through the Division of Intramural Research to the Laboratory of Molecular Medicine and Neuroscience.
Editorial, page 2006
-
Supplemental data at www.neurology.org
- Received March 31, 2011.
- Accepted June 7, 2011.
- Copyright © 2011 by AAN Enterprises, Inc.
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