Document Detail


Progressive multifocal leukoencephalopathy in individuals with minimal or occult immunosuppression.
MedLine Citation:
PMID:  19828476     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is a deadly demyelinating disease of the brain, caused by reactivation of the polyomavirus JC (JCV). PML has classically been described in individuals with profound cellular immunosuppression such as patients with AIDS, haematological malignancies, organ transplant recipients or those treated with immunosuppressive or immunomodulatory medications for autoimmune diseases. METHODS AND CASE REPORTS: The authors describe five HIV seronegative patients with minimal or occult immunosuppression who developed PML including two patients with alcoholic cirrhosis, one with untreated dermatomyositis and two with idiopathic CD4(+) T cell lymphocytopenia. The authors performed a review of the literature to find similar cases.
RESULTS: The authors found an additional 33 cases in the literature. Of a total of 38 cases, seven (18.4%) had hepatic cirrhosis, five (13.2%) had renal failure, including one with concomitant hepatic cirrhosis, two (5.2%) were pregnant women, two (5.2%) had concomitant dementia, one (2.6%) had dermatomyositis, and 22 (57.9%) had no specific underlying diagnosis. Among these 22, five (22.7%) had low CD4(+) T cell counts (0.080-0.294x10(9)/l) and were diagnosed as having idiopathic CD4(+) lymphocytopenia, and one had a borderline CD4(+) T cell count of 0.308x10(9)/l. The outcome was fatal in 27/38 (71.1%) cases within 1.5-120 months (median 8 months) from onset of symptoms, and 3/4 cases who harboured JCV-specific T cells in their peripheral blood had inactive disease with stable neurological deficits after 6-26 months of follow-up.
DISCUSSION: These results indicate that PML can occur in patients with minimal or occult immunosuppression, and one can revisit the generally accepted notion that profound cellular immunosuppression is a prerequisite for the development of PML.
Authors:
Sarah Gheuens; Gerald Pierone; Patrick Peeters; Igor J Koralnik
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, N.I.H., Extramural; Review     Date:  2009-10-14
Journal Detail:
Title:  Journal of neurology, neurosurgery, and psychiatry     Volume:  81     ISSN:  1468-330X     ISO Abbreviation:  J. Neurol. Neurosurg. Psychiatr.     Publication Date:  2010 Mar 
Date Detail:
Created Date:  2010-02-26     Completed Date:  2010-03-22     Revised Date:  2013-05-31    
Medline Journal Info:
Nlm Unique ID:  2985191R     Medline TA:  J Neurol Neurosurg Psychiatry     Country:  England    
Other Details:
Languages:  eng     Pagination:  247-54     Citation Subset:  IM    
Affiliation:
Division of Viral Pathogenesis, Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA 02215, USA.
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MeSH Terms
Descriptor/Qualifier:
Adult
Brain / pathology
CD4-Positive T-Lymphocytes / immunology
Female
Follow-Up Studies
HIV Seronegativity
Humans
Immune Tolerance / immunology*
Leukoencephalopathy, Progressive Multifocal / diagnosis*,  immunology*,  mortality
Lymphopenia / diagnosis,  immunology
Magnetic Resonance Imaging
Male
Middle Aged
Neurologic Examination
Opportunistic Infections / diagnosis*,  immunology*
Pregnancy
Risk Factors
Survival Rate
Grant Support
ID/Acronym/Agency:
047029//PHS HHS; K24 NS 060950/NS/NINDS NIH HHS; K24 NS060950-03/NS/NINDS NIH HHS; R01 NS 041198/NS/NINDS NIH HHS; R01 NS041198-09/NS/NINDS NIH HHS; R01 NS047029-06/NS/NINDS NIH HHS; T32 CA09031-32/CA/NCI NIH HHS
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