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J. Neurol. Neurosurg. Psychiatr. · May 2018
Inflammatory natalizumab-associated PML: baseline characteristics, lesion evolution and relation with PML-IRIS.
- Mike P Wattjes, Martijn T Wijburg, van EijkJeroenJDepartment of Neurology, Jeroen Bosch Ziekenhuis, s'Hertogenbosch, The Netherlands., Stephan Frequin, UitdehaagBernard M JBMJDepartment of Neurology, Neuroscience Amsterdam, VUmc MS Center Amsterdam, VU University Medical Center, Amsterdam, The Netherlands., Frederik Barkhof, Clemens Warnke, Joep Killestein, and Dutch-Belgian Natalizumab-Associated PML Study Group.
- Department of Diagnostic and Interventional Neuroradiology, Hannover Medical School, Hannover, Germany.
- J. Neurol. Neurosurg. Psychiatr. 2018 May 1; 89 (5): 535-541.
Background And ObjectiveNatalizumab-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.MethodsWe 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.ResultsTen 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%).ConclusionPatterns 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.© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.
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