In Vivo Measurement of Tau Depositions in Anti-IgLON5 Disease Using [18F]PI-2620 PET

Hendrik Theis, Gérard N. Bischof, Norbert Brüggemann, Justina Dargvainiene, Alexander Drzezga, Thomas Grüter, Jan Lewerenz, Frank Leypoldt, Bernd Neumaier, Klaus Peter Wandinger, Ilya Ayzenberg, Thilo Van Eimeren*

*Corresponding author for this work

Abstract

Objectives: Anti-IgLON5 disease is a recently discovered neurologic disorder combining autoimmunity and neurodegeneration. Core manifestations include sleep disorders, bulbar symptoms, gait abnormalities and cognitive dysfunction, but other presentations have been reported. Hallmarks are autoantibodies targeting the neuronal surface protein IgLON5, a strong human leukocyte antigen system Class II association, and brainstem and hypothalamus-dominant tau deposits. The purpose of this cohort study was to visualize tau deposition in vivo with the second-generation tau-PET tracer.MethodsA cohort of 4 patients with anti-IgLON5 disease underwent a dynamic PET scan with [18F]PI-2620. One patient received a follow-up scan. Z-deviation maps and a 2-sample t test in comparison with healthy controls (n = 10) were performed. Antibody titers, neurofilament light chain, and disease duration were correlated with brainstem binding potentials.

Results: Patients demonstrated increased [18F]PI2620 tau binding potentials in the pons, dorsal medulla, and cerebellum. The longitudinal scan after 28 months showed an increase of tracer uptake in the medulla despite immunotherapy. Higher antibody titers and neurofilament light chain correlated with higher tracer retention.

Discussion: The results indicate that tau depositions in anti-IgLON5 disease can be visualized with [18F]PI-2620 and might correlate with the extent of disease. For validation, a larger longitudinal study is necessary.

Original languageEnglish
JournalNeurology
Volume101
Issue number22
Pages (from-to)E2325-E2330
ISSN0028-3878
DOIs
Publication statusPublished - 28.11.2023

Research Areas and Centers

  • Academic Focus: Center for Brain, Behavior and Metabolism (CBBM)

DFG Research Classification Scheme

  • 206-07 Clinical Neurology Neurosurgery and Neuroradiology
  • 206-05 Experimental Models for Investigating Diseases of the Nervous System

Cite this