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Progressive Multifocal Leukoencephalopathy (PML)
Miral D. Jhaveri, MD, MBA
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Progressive multifocal leukoencephalopathy (PML)
              • Classic PML (cPML)
              • Inflammatory PML (iPML)
            • Definitions

              • Subacute opportunistic infection caused by DNA John Cunningham polyomavirus (JC virus, JCV)

            IMAGING

            • General Features

              • Imaging Recommendations

                • CT Findings

                  • MR Findings

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Image Interpretation Pearls

                                      Selected References

                                      1. López Sala P et al: Cortical hypointensity in T2-weighted gradient-echo sequences in patients with progressive multifocal leukoencephalopathy. Radiologia. 62(1):59-66, 2020
                                      2. Bartsch T et al: The spectrum of progressive multifocal leukoencephalopathy: a practical approach. Eur J Neurol. 26(4):566-e41, 2019
                                      3. Thurnher MM et al: Susceptibility-weighted mr imaging hypointense rim in progressive multifocal leukoencephalopathy: the end point of neuroinflammation and a potential outcome predictor. AJNR Am J Neuroradiol. 40(6):994-1000, 2019
                                      4. Grebenciucova E et al: Progressive multifocal leukoencephalopathy. Neurol Clin. 36(4):739-50, 2018
                                      5. Major EO: Progressive Multifocal leukoencephalopathy lesions and jc virus: the limits and value of imaging. JAMA Neurol. 75(7):789-90, 2018
                                      6. Berger JR: Progressive multifocal leukoencephalopathy. Handb Clin Neurol. 123:357-76, 2014
                                      7. Morrison VA: Immunosuppression associated with novel chemotherapy agents and monoclonal antibodies. Clin Infect Dis. 59 Suppl 5:S360-4, 2014
                                      8. Wattjes MP et al: MRI pattern in asymptomatic natalizumab-associated PML. J Neurol Neurosurg Psychiatry. 86(7):793-8, 2014
                                      9. Zanoni BC et al: Update on opportunistic infections in the era of effective antiretroviral therapy. Infect Dis Clin North Am. 28(3):501-18, 2014
                                      10. Shah R et al: Imaging manifestations of progressive multifocal leukoencephalopathy. Clin Radiol. 65(6):431-9, 2010
                                      11. Boster A et al: Progressive multifocal leukoencephalopathy and relapsing-remitting multiple sclerosis: a comparative study. Arch Neurol. 66(5):593-9, 2009
                                      12. Carson KR et al: Monoclonal antibody-associated progressive multifocal leucoencephalopathy in patients treated with rituximab, natalizumab, and efalizumab: a review from the Research on Adverse Drug Events and Reports (RADAR) Project. Lancet Oncol. 10(8):816-24, 2009
                                      13. Boren EJ et al: The emergence of progressive multifocal leukoencephalopathy (PML) in rheumatic diseases. J Autoimmun. 30(1-2):90-8, 2008
                                      14. Bergui M et al: Progressive multifocal leukoencephalopathy: diffusion-weighted imaging and pathological correlations. Neuroradiology. 46(1):22-5, 2004
                                      15. Thurnher MM et al: Initial and follow-up MR imaging findings in AIDS-related progressive multifocal leukoencephalopathy treated with highly active antiretroviral therapy. AJNR Am J Neuroradiol. 22(5):977-84, 2001
                                      16. Whiteman ML et al: Progressive multifocal leukoencephalopathy in 47 HIV-seropositive patients: neuroimaging with clinical and pathologic correlation. Radiology. 187(1):233-40, 1993
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Top Differential Diagnoses

                                            • Clinical Issues

                                              • Diagnostic Checklist

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Progressive multifocal leukoencephalopathy (PML)
                                                  • Classic PML (cPML)
                                                  • Inflammatory PML (iPML)
                                                • Definitions

                                                  • Subacute opportunistic infection caused by DNA John Cunningham polyomavirus (JC virus, JCV)

                                                IMAGING

                                                • General Features

                                                  • Imaging Recommendations

                                                    • CT Findings

                                                      • MR Findings

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Gross Pathologic & Surgical Features

                                                              • Microscopic Features

                                                                CLINICAL ISSUES

                                                                • Presentation

                                                                  • Demographics

                                                                    • Natural History & Prognosis

                                                                      • Treatment

                                                                        DIAGNOSTIC CHECKLIST

                                                                        • Image Interpretation Pearls

                                                                          Selected References

                                                                          1. López Sala P et al: Cortical hypointensity in T2-weighted gradient-echo sequences in patients with progressive multifocal leukoencephalopathy. Radiologia. 62(1):59-66, 2020
                                                                          2. Bartsch T et al: The spectrum of progressive multifocal leukoencephalopathy: a practical approach. Eur J Neurol. 26(4):566-e41, 2019
                                                                          3. Thurnher MM et al: Susceptibility-weighted mr imaging hypointense rim in progressive multifocal leukoencephalopathy: the end point of neuroinflammation and a potential outcome predictor. AJNR Am J Neuroradiol. 40(6):994-1000, 2019
                                                                          4. Grebenciucova E et al: Progressive multifocal leukoencephalopathy. Neurol Clin. 36(4):739-50, 2018
                                                                          5. Major EO: Progressive Multifocal leukoencephalopathy lesions and jc virus: the limits and value of imaging. JAMA Neurol. 75(7):789-90, 2018
                                                                          6. Berger JR: Progressive multifocal leukoencephalopathy. Handb Clin Neurol. 123:357-76, 2014
                                                                          7. Morrison VA: Immunosuppression associated with novel chemotherapy agents and monoclonal antibodies. Clin Infect Dis. 59 Suppl 5:S360-4, 2014
                                                                          8. Wattjes MP et al: MRI pattern in asymptomatic natalizumab-associated PML. J Neurol Neurosurg Psychiatry. 86(7):793-8, 2014
                                                                          9. Zanoni BC et al: Update on opportunistic infections in the era of effective antiretroviral therapy. Infect Dis Clin North Am. 28(3):501-18, 2014
                                                                          10. Shah R et al: Imaging manifestations of progressive multifocal leukoencephalopathy. Clin Radiol. 65(6):431-9, 2010
                                                                          11. Boster A et al: Progressive multifocal leukoencephalopathy and relapsing-remitting multiple sclerosis: a comparative study. Arch Neurol. 66(5):593-9, 2009
                                                                          12. Carson KR et al: Monoclonal antibody-associated progressive multifocal leucoencephalopathy in patients treated with rituximab, natalizumab, and efalizumab: a review from the Research on Adverse Drug Events and Reports (RADAR) Project. Lancet Oncol. 10(8):816-24, 2009
                                                                          13. Boren EJ et al: The emergence of progressive multifocal leukoencephalopathy (PML) in rheumatic diseases. J Autoimmun. 30(1-2):90-8, 2008
                                                                          14. Bergui M et al: Progressive multifocal leukoencephalopathy: diffusion-weighted imaging and pathological correlations. Neuroradiology. 46(1):22-5, 2004
                                                                          15. Thurnher MM et al: Initial and follow-up MR imaging findings in AIDS-related progressive multifocal leukoencephalopathy treated with highly active antiretroviral therapy. AJNR Am J Neuroradiol. 22(5):977-84, 2001
                                                                          16. Whiteman ML et al: Progressive multifocal leukoencephalopathy in 47 HIV-seropositive patients: neuroimaging with clinical and pathologic correlation. Radiology. 187(1):233-40, 1993