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Progressive multifocal leukoencephalopathy - the importance of early diagnosis illustrated in four cases.

Nived, Ola LU ; Bengtsson, Anders LU ; Jönsen, Andreas LU and Sturfelt, Gunnar LU (2008) In Lupus 17(11). p.1036-1041
Abstract
Progressive multifocal leukoencephalopathy (PML) is a rare, deadly demyelinating disease of the central nervous system, which is caused by a reactivation of the DNA polyomavirus JC and occurs in immunosuppressed individuals. So far, only 25 cases have been described in patients with SLE and none survived without antiviral therapy and only two cases in RA. We present four additional cases from a defined area, three in SLE, of which one survived without antiviral therapy, and one case in RA, also surviving after reduction of immunosuppressive treatment. In three of these cases, diagnosis could only be confirmed by stereotactical brain biopsy, including the two surviving cases. Thus, this article illustrates the difficulty in diagnosing... (More)
Progressive multifocal leukoencephalopathy (PML) is a rare, deadly demyelinating disease of the central nervous system, which is caused by a reactivation of the DNA polyomavirus JC and occurs in immunosuppressed individuals. So far, only 25 cases have been described in patients with SLE and none survived without antiviral therapy and only two cases in RA. We present four additional cases from a defined area, three in SLE, of which one survived without antiviral therapy, and one case in RA, also surviving after reduction of immunosuppressive treatment. In three of these cases, diagnosis could only be confirmed by stereotactical brain biopsy, including the two surviving cases. Thus, this article illustrates the difficulty in diagnosing progressive multifocal leukoencephalopathy, the need for brain biopsy in many cases, the importance of reduced immunosuppression as early as possible and the severe damage progressive multifocal leukoencephalopathy can cause. Furthermore, progressive multifocal leukoencephalopathy might be much more common in SLE than expected with 1 case in 800 patient-years. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Lupus
volume
17
issue
11
pages
1036 - 1041
publisher
SAGE Publications Ltd
external identifiers
  • wos:000260720400013
  • pmid:18852230
  • scopus:61549128409
ISSN
0961-2033
DOI
10.1177/0961203308089445
language
English
LU publication?
yes
id
75febda7-321b-4bff-9e9e-03ebf056380c (old id 1262255)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/18852230?dopt=Abstract
date added to LUP
2008-11-05 13:50:21
date last changed
2017-01-08 05:27:23
@article{75febda7-321b-4bff-9e9e-03ebf056380c,
  abstract     = {Progressive multifocal leukoencephalopathy (PML) is a rare, deadly demyelinating disease of the central nervous system, which is caused by a reactivation of the DNA polyomavirus JC and occurs in immunosuppressed individuals. So far, only 25 cases have been described in patients with SLE and none survived without antiviral therapy and only two cases in RA. We present four additional cases from a defined area, three in SLE, of which one survived without antiviral therapy, and one case in RA, also surviving after reduction of immunosuppressive treatment. In three of these cases, diagnosis could only be confirmed by stereotactical brain biopsy, including the two surviving cases. Thus, this article illustrates the difficulty in diagnosing progressive multifocal leukoencephalopathy, the need for brain biopsy in many cases, the importance of reduced immunosuppression as early as possible and the severe damage progressive multifocal leukoencephalopathy can cause. Furthermore, progressive multifocal leukoencephalopathy might be much more common in SLE than expected with 1 case in 800 patient-years.},
  author       = {Nived, Ola and Bengtsson, Anders and Jönsen, Andreas and Sturfelt, Gunnar},
  issn         = {0961-2033},
  language     = {eng},
  number       = {11},
  pages        = {1036--1041},
  publisher    = {SAGE Publications Ltd},
  series       = {Lupus},
  title        = {Progressive multifocal leukoencephalopathy - the importance of early diagnosis illustrated in four cases.},
  url          = {http://dx.doi.org/10.1177/0961203308089445},
  volume       = {17},
  year         = {2008},
}