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Journal of Neurology
Published in: Journal of Neurology 1/2024

Open Access 20-09-2023 | Natalizumab | Original Communication

Long-term outcome of natalizumab-associated progressive multifocal leukoencephalopathy in Austria: a nationwide retrospective study

Authors: Tobias Moser, Georg Zimmermann, Anna Baumgartner, Thomas Berger, Gabriel Bsteh, Franziska Di Pauli, Christian Enzinger, Elisabeth Fertl, Thomas Heller, Stefan Koppi, Paulus S. Rommer, Georg Safoschnik, Thomas Seifert-Held, Robert Stepansky, Johann Sellner

Published in: Journal of Neurology | Issue 1/2024

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Abstract

Background/objective

The use of natalizumab (NAT) in multiple sclerosis (MS) may be complicated by progressive multifocal leukoencephalopathy (PML), a rare and life-threatening opportunistic brain infection. We aimed to analyze the course of MS after PML recovery together with the long-term outcome of NAT-associated PML (NAT-PML) in Austria.

Methods

Retrospective study based on identification of cases in the nationwide Austrian MS treatment registry (AMSTR) and MS centers with review of patient records. The expanded disability status scale (EDSS) was used to measure neurological disability and outcome.

Results

As of December 2022, we identified 15 NAT-PML cases in Austria; only 20% occurred after 2016, when increased vigilance commenced. Two patients did not survive acute PML, and an additional patient died five years later, yielding a mortality rate of 20%. Seizures occurred exclusively in patients with pronounced EDSS increase. Gadolinium (Gd)-enhancement on brain magnetic resonance imaging (MRI) on PML suspicion was associated with minor changes of post-PML neurological disability. Long-term follow-up of up to 132 months (median 76 months) was available in 11/15. The overall median EDSS increased from 3.5 at pre-PML to 6.5 at the last assessment. Regarding inflammatory MS-related disease activity during the observation period, one single individual experienced an MS relapse and another patient had two Gd-enhancing brain lesions. Three patients converted to progressive MS within three years from PML and the EDSS further increased in 6/11.

Conclusions

The number of NAT-PML cases is decreasing over time. While many patients accumulated severe persistent neurological deficits compared to pre-PML, inflammatory MS-related disease activity after PML recovery was rare.
Appendix
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Metadata
Title
Long-term outcome of natalizumab-associated progressive multifocal leukoencephalopathy in Austria: a nationwide retrospective study
Authors
Tobias Moser
Georg Zimmermann
Anna Baumgartner
Thomas Berger
Gabriel Bsteh
Franziska Di Pauli
Christian Enzinger
Elisabeth Fertl
Thomas Heller
Stefan Koppi
Paulus S. Rommer
Georg Safoschnik
Thomas Seifert-Held
Robert Stepansky
Johann Sellner
Publication date
20-09-2023
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology / Issue 1/2024
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-023-11924-7

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