Inflammatory natalizumab-associated PML: baseline characteristics, lesion evolution and relation with PML-IRIS. Issue 5 (15th November 2017)

• Record Type: Journal Article
• Authors: Wattjes, Mike P; Wijburg, Martijn T; van Eijk, Jeroen; Frequin, Stephan; Uitdehaag, Bernard M J; Barkhof, Frederik; Warnke, Clemens; Killestein, Joep
• Other Names: author non-byline.; Van oosten Bob W author non-byline.; Polman Chris H author non-byline.; Siepman Dorine A author non-byline.; Hintzen Rogier author non-byline.; Mostert Jop author non-byline.; Moll Wibe author non-byline.; Van golde Alex El author non-byline.; Frequin Stephan Tfm author non-byline.; Bouma Paul Ad author non-byline.; Quivron Bénédicte author non-byline.; Braeckeveldt Jean author non-byline.; Munster Erik Van author non-byline.; Eijk Jeroen Van author non-byline.; Heersema Thea author non-byline.; Graaf Jaap De author non-byline.
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 89:Issue 5(2018)
• Page Start: 535
• Page End: 541
• Publication Date: 2017-11-15
• Subjects: multiple sclerosis -- magnetic resonance imaging -- progressive multifocal leukoencephalopathy -- natalizumab; Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp-2017-316886
• Languages: English
• ISSNs: 0022-3050

Abstract

Abstract : Background and objective: Natalizumab-associated progressive multifocal leukoencephalopathy (NTZ-PML) patients may show imaging signs suggestive of inflammation at diagnosis ('inflammatory PML'), reminiscent of PML-immune reconstitution inflammatory syndrome (PML-IRIS). We investigated the imaging characteristics of inflammatory NTZ-PML lesions and PML-IRIS to determine differentiating and overlapping features. Methods: We scored the presence, localisation and pattern of imaging characteristics of inflammation on brain MRI scans of inflammatory NTZ-PML patients. The imaging characteristics were followed up until the occurrence of PML-IRIS. Results: Ten out of the 44 NTZ-PML patients included showed signs suggestive of inflammation at the time of diagnosis. The inflammation pattern at diagnosis was similar to the pattern seen at PML-IRIS, with contrast enhancement representing the most frequent sign of inflammation (90% at diagnosis, 100% at PML-IRIS). However, the severity of inflammation differed, with absence of swelling and low frequency of perilesional oedema (10%) at diagnosis, as compared with the PML-IRIS stage (40%). Conclusion: Patterns of inflammation at the time of PML diagnosis and at the PML-IRIS stage overlap but differ in their severity of inflammation. This supports histopathological evidence that the inflammation seen at both stages of the same disease shares a similar underlying pathophysiology, representing the immune response to the JC virus to a variable extend.