Researchers at the German Center for Neurodegenerative Diseases (DZNE) and Charité – Universitätsmedizin Berlin have elucidated the basic mechanisms of rare anti-IgLON5 encephalitis. The disease leads to a malfunction of the immune system, which mistakenly attacks cells in the brain, causing brain inflammation and nerve damage.

In anti-IgLON5 encephalitis , misdirected antibodies attack the protein IgLON5 on the surface of nerve cells. The new study shows that these antibodies cause IgLON5 to adhere to other molecules on the cell surface. As a result, nerve cells become overactive, which triggers a chain reaction. At the end of this reaction there are pathological tau deposits and the destruction of nerve cells.

Prof. Susanne Wegmann, research group leader at the DZNE and Charité, explained that the study proves a causal relationship between IgLON5 antibodies and tau pathology for the first time. This mechanism has parallels to Alzheimer’s disease, in which neuronal hyperactivity also leads to tau deposits.

Anti-IgLON5 encephalitis was first described in 2014. It manifests itself through sleep disorders, cognitive loss and movement problems. Without treatment, the disease can lead to severe disability and early death. Previous therapies such as immunosuppressants have shown limited effect.

The new findings could provide starting points for future therapies that specifically alleviate neuronal overactivity. The study was published in the journal Science Advances . It is based on studies on cell cultures, mice and antibodies from affected persons.

Original Paper:

IgLON5 autoimmune antibodies activate tau via neuronal hyperactivity | Science Advances

Editor: X-Press Journalistenbüro GbR

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