RT Journal Article
SR Electronic
T1 Increased Intrathecal B and Plasma Cells in Patients With Anti-IgLON5 Disease
JF Neurology - Neuroimmunology Neuroinflammation
JO Neurol Neuroimmunol Neuroinflamm
FD Lippincott Williams & Wilkins
SP 00
DO 10.1212/NXI.0000000000001137
VO 9
IS 2
A1 Christine Strippel
A1 Anna Heidbreder
A1 Andreas Schulte-Mecklenbeck
A1 Lisanne Korn
A1 Tobias Warnecke
A1 Nico Melzer
A1 Heinz Wiendl
A1 Matthias Pawlowski
A1 Catharina C. Gross
A1 Stjepana Kovac
YR 2022
UL http://nn.neurology.org/content/9/2/00.abstract
AB Background and Objectives 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.Methods 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.Results 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.Discussion 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.