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.
journal_name
Lupusjournal_title
Lupusauthors
Nived O,Bengtsson AA,Jönsen A,Sturfelt Gdoi
10.1177/0961203308089445subject
Has Abstractpub_date
2008-11-01 00:00:00pages
1036-41issue
11eissn
0961-2033issn
1477-0962pii
17/11/1036journal_volume
17pub_type
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