Journal
NEUROLOGY
Volume 80, Issue 15, Pages 1430-1438Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1212/WNL.0b013e31828c2fa1
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Funding
- CMSC
- AAN
- Bayer
- BiogenIdec
- PML Consortium
- NIH [U10 NS077384, MH22005, AI25903, R56-NS041198, R01-NS047029, R01-NS074995, K24-NS060950]
- Alzheimer Association
- Biogen Idec
- NeurogesX
- Tibotec
- Pfizer
- University of Kentucky and CMSC/ACTRIMS
- ECTRIMS
- National MS Society
- Bristol-Myers Squibb
- Ono Pharmaceuticals
- Merck Serono
- Hoffmann La Roche
- GlaxoSmithKline
- Perseid Therapeutics
- Vertex Pharmaceutical
- Johnson Johnson
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Objective: To establish criteria for the diagnosis of progressive multifocal leukoencephalopathy (PML). Methods: We reviewed available literature to identify various diagnostic criteria employed. Several search strategies employing the terms progressive multifocal leukoencephalopathy with or without JC virus were performed with PubMed, SCOPUS, and EMBASE search engines. The articles were reviewed by a committee of individuals with expertise in the disorder in order to determine the most useful applicable criteria. Results: A consensus statement was developed employing clinical, imaging, pathologic, and virologic evidence in support of the diagnosis of PML. Two separate pathways, histopathologic and clinical, for PML diagnosis are proposed. Diagnostic classification includes certain, probable, possible, and not PML. Conclusion: Definitive diagnosis of PML requires neuropathologic demonstration of the typical histopathologic triad (demyelination, bizarre astrocytes, and enlarged oligodendroglial nuclei) coupled with the techniques to show the presence of JC virus. The presence of clinical and imaging manifestations consistent with the diagnosis and not better explained by other disorders coupled with the demonstration of JC virus by PCR in CSF is also considered diagnostic. Algorithms for establishing the diagnosis have been recommended.
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