Anti-IgLON5 disease, a rare autoimmune disease of the nervous system, poses a diagnostic challenge due to its diverse symptoms. Typical are sleep disorders, movement, swallowing and speech disorders, which, if left untreated, lead to severe impairments and often death within a few years. An international observational study with 107 patients, also conducted by researchers at St. Josef Hospital, Klinikum der Ruhr-Universität Bochum, shows that early immunotherapy, especially with intravenous immunoglobulins, is the most effective treatment option. The results were published in JAMA Neurology on August 4, 2025.

The disease, which was first described in 2014, occurs mainly in older age and is characterized by inflammatory processes in the brain, which in later stages transition into a neurodegenerative phase with the breakdown of nerve cells. In this late phase, immunotherapy usually loses its effectiveness. The study, which evaluated data from Germany, the Netherlands and Spain, showed that, on average, the diagnosis was not made until two years after the onset of the first symptoms, although early diagnosis is crucial. Only a third of the 107 patients received a diagnosis within a year, which explains the similarity of symptoms to other neurological diseases such as atypical Parkinson’s disease, bulbar myasthenia or motor neuron disease.

The study compared two immunotherapies: rituximab, which targets antibody-producing B cells, and immunoglobulins, which are derived from donor blood. Surprisingly, the less specific immunoglobulin therapy showed better results. About 23 percent of patients received immunotherapy in the first year, 52 percent later. The researchers emphasize that early treatment can significantly slow down disease progression. Without or with too late therapy, 40 percent of patients died within an average of five years, with the disease being the cause of death in at least two-thirds of the cases.

The researchers appeal to neurologists to think about the anti-IgLON5 disease at an early stage if they have symptoms and to carry out antibody tests in the blood. They are also calling for greater participation in registries such as the German GENERATE network in order to advance research on this rare disease.

Original Paper:

Early Treatment With Intravenous Immunoglobulins and Outcomes of Patients With Anti-IgLON5 Disease | Neurology | JAMA Neurology | JAMA Network

Editor: X-Press Journalistenbüro GbR

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