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Case 20 - Progressive multifocal leukoencephalopathy

Published online by Cambridge University Press:  18 December 2013

Nafi Aygun
Affiliation:
The Johns Hopkins University
Gaurang Shah
Affiliation:
University of Michigan Health System
Dheeraj Gandhi
Affiliation:
University of Maryland Medical Center
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Summary

Imaging description

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disorder of the brain caused by the JC virus (JCV). PML is most frequently seen in HIV/AIDS patients and patients with hematological malignancies [1]. On MRI, characteristic features of PML include unifocal or multifocal areas of hyperintensity on T2-weighted/FLAIR images, and corresponding areas of hypointensity on T1-weighted images with no associated mass effect or enhancement on post-contrast T1-weighted images [2]. Lesions can be patchy or confluent and involve the subcortical white matter including the U-fibers and the cerebellum (Fig. 20.1). The middle cerebellar peduncle is a common site of involvement (Fig. 20.2).

Diffusion-weighted imaging (DWI) may show variable signal; increased DWI signal corresponds to the sites of active infection [3]. Rarely, multiple sclerosis patients receiving natalizumab (a monoclonal antibody) may develop PML, which may show post-contrast enhancement. The diagnosis of PML is established by demonstrating the JCV DNA in CSF or brain biopsy.

Type
Chapter
Information
Pearls and Pitfalls in Head and Neck and Neuroimaging
Variants and Other Difficult Diagnoses
, pp. 62 - 64
Publisher: Cambridge University Press
Print publication year: 2013

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References

Brew, BJ, Davies, NW, Cinque, P, Clifford, DB, Nath, A. Progressive multifocal leukoencephalopathy and other forms of JC virus disease. Nat Rev Neurol 2010; 6: 667–79.CrossRefGoogle ScholarPubMed
Tan, CS, Koralnik, IJ. Progressive multifocal leukoencephalopathy and other disorders caused by JC virus: clinical features and pathogenesis. Lancet Neurol 2010; 9: 425–37.CrossRefGoogle ScholarPubMed
Clifford, DB, De Luca, A, Simpson, DM, et al. Natalizumab-associated progressive multifocal leukoencephalopathy in patients with multiple sclerosis: lessons from 28 cases. Lancet Neurol 2010; 9: 438–46.CrossRefGoogle ScholarPubMed

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