Increased Intrathecal B and Plasma Cells in Patients With Anti-IgLON5 Disease
A Case Series
release_rev_51a9a566-2ee5-4ce2-8080-92fb9da2d201
by
Christine Strippel, Anna Heidbreder, Andreas Schulte-Mecklenbeck, Lisanne Korn, Tobias Warnecke, Nico Melzer, Heinz Wiendl, Matthias Pawlowski, Catharina C. Gross, Stjepana Kovac
2022 p00
Abstract
<jats:sec><jats:title>Background and Objectives</jats:title>Despite detection of autoantibodies, anti-IgLON5 disease was historically considered a tau-associated neurodegenerative disease, with limited treatment options and detrimental consequences for the patients. Observations in increasing case numbers hint toward underlying inflammatory mechanisms that, early detection provided, open a valuable window of opportunity for therapeutic intervention. We aimed to further substantiate this view by studying the CSF of patients with anti-IgLON5.</jats:sec><jats:sec><jats:title>Methods</jats:title>We identified 11 patients with anti-IgLON5 from our database and compared clinical, MRI, and CSF findings with a cohort of 20 patients with progressive supranuclear palsy (PSP) (as a noninflammatory tauopathy) and 22 patients with functional neurologic disorder.</jats:sec><jats:sec><jats:title>Results</jats:title>Patients with anti-IgLON5 show inflammatory changes in routine CSF analysis, an increase in B-lymphocyte frequency, and the presence of plasma cells in comparison to the PSP-control group and functional neurologic disease controls. Patients with intrathecal plasma cells showed a clinical response to rituximab.</jats:sec><jats:sec><jats:title>Discussion</jats:title>Our findings indicate the importance of inflammatory mechanisms, in particular in early and acute anti-IgLON5 cases, which may support the use of immune-suppressive treatments in these cases. The main limitation of the study is the small number of cases due to the rarity of the disease.</jats:sec>
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