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Inflammatory natalizumab-associated PML: baseline characteristics, lesion evolution and relation with PML-IRIS

Wattjes, MP; Wijburg, MT; Eijk, JV; Frequin, S; Uitdehaag, BMJ; Barkhof, F; Warnke, C; (2018) Inflammatory natalizumab-associated PML: baseline characteristics, lesion evolution and relation with PML-IRIS. Journal of Neurology, Neurosurgery & Psychiatry , 89 (5) pp. 535-541. 10.1136/jnnp-2017-316886. Green open access

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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.

Type: Article
Title: Inflammatory natalizumab-associated PML: baseline characteristics, lesion evolution and relation with PML-IRIS
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1136/jnnp-2017-316886
Publisher version: http://dx.doi.org/10.1136/jnnp-2017-316886
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Magnetic resonance imaging, multiple sclerosis, natalizumab, progressive multifocal leukoencephalopathy
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Brain Repair and Rehabilitation
URI: https://discovery.ucl.ac.uk/id/eprint/10039071
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