Document Detail


Atypical progressive multifocal leukoencephalopathy associated with an unusual JC polyomavirus mutation.
MedLine Citation:
PMID:  19667225     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: To report the clinical and radiologic features in a patient with myelofibrosis who developed atypical progressive multifocal leukoencephalopathy. DESIGN: Case report. SETTING: Tertiary referral center. Patient A 72-year-old man with myelofibrosis and mild leukopenia experienced progressive limb weakness and dysarthria. RESULTS: Imaging revealed almost complete sparing of the white matter with isolated involvement of the brainstem and deep gray matter. Postmortem examination led to definitive diagnosis of progressive multifocal leukoencephalopathy and demonstrated an unusual miliary pattern of disease rather than the typical confluent involvement. Genetic analysis revealed a mutation in the transcription control region of the JC polyomavirus, prompting speculation about the pathogenesis of progressive multifocal leukoencephalopathy. CONCLUSIONS: Leukopenia may render patients effectively immunosuppressed. The differential diagnosis should include progressive multifocal leukoencephalopathy even in patients with atypical clinical and radiologic features.
Authors:
Emma C Tallantyre; Simon M L Paine; Colin P Sharp; James S Lowe; Bruno Gran
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Archives of neurology     Volume:  66     ISSN:  1538-3687     ISO Abbreviation:  Arch. Neurol.     Publication Date:  2009 Aug 
Date Detail:
Created Date:  2009-08-11     Completed Date:  2009-08-20     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0372436     Medline TA:  Arch Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1021-4     Citation Subset:  AIM; IM    
Affiliation:
Division of Clinical Neurology, University of Nottingham, Nottingham, England.
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MeSH Terms
Descriptor/Qualifier:
Aged
Brain / pathology
Brain Stem / pathology
Codon / genetics
DNA Mutational Analysis*
DNA, Viral / genetics*
Genome, Viral / genetics*
Humans
JC Virus / genetics*
Leukoencephalopathy, Progressive Multifocal / diagnosis,  virology*
Magnetic Resonance Imaging
Male
Microscopy, Electron, Transmission
Neurologic Examination
Polymerase Chain Reaction
Transcription, Genetic / genetics*
Virion / genetics,  ultrastructure
Chemical
Reg. No./Substance:
0/Codon; 0/DNA, Viral

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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