| Atypical progressive multifocal leukoencephalopathy associated with an unusual JC polyomavirus mutation. | |
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MedLine Citation:
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PMID: 19667225 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Emma C Tallantyre; Simon M L Paine; Colin P Sharp; James S Lowe; Bruno Gran |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Archives of neurology Volume: 66 ISSN: 1538-3687 ISO Abbreviation: Arch. Neurol. Publication Date: 2009 Aug |
Date Detail:
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Created Date: 2009-08-11 Completed Date: 2009-08-20 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0372436 Medline TA: Arch Neurol Country: United States |
Other Details:
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Languages: eng Pagination: 1021-4 Citation Subset: AIM; IM |
Affiliation:
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Division of Clinical Neurology, University of Nottingham, Nottingham, England. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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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:
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0/Codon; 0/DNA, Viral |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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