Early Immunotherapy Critical for Treating Anti-IgLON5 Disease, New Study Finds

Anti-IgLON5 disease is a rare autoimmune neurological disorder characterized by a variety of symptoms such as restless sleep, movement disorders, swallowing difficulties, and speech problems. Its complex presentation often makes diagnosis challenging. If left untreated, anti-IgLON5 disease can progress rapidly, leading to severe disabilities and a high risk of mortality within a few years.

Recent research emphasizes the importance of initiating immunotherapy as early as possible to improve outcomes. An international study involving 107 patients across Germany, the Netherlands, and Spain demonstrated that early treatment significantly impacts disease progression. The study highlights that intravenous immunoglobulins, a treatment derived from donated blood proteins, show the most promising results when administered promptly.

The research, led by Professor Ilya Ayzenberg and Associate Professor Thomas Grüter from Ruhr University Bochum, was published in JAMA Neurology. It reveals that immunotherapy during the initial inflammatory phase of the disease—usually occurring in the early stages—can still influence the disease course. As the disease progresses, inflammation diminishes but is replaced by nerve cell and fiber degeneration, at which point immunotherapy becomes largely ineffective.

Patients who received immunotherapy within the first year of symptom onset, particularly with immunoglobulin infusions, showed better outcomes compared to those treated later. The study found that only about a third of the patients were diagnosed within a year of symptom onset, indicating a critical need for early detection. The researchers urge neurologists to consider anti-IgLON5 disease early in the diagnostic process and to perform blood tests for specific antibodies to facilitate timely treatment.

Delayed diagnosis and treatment often result in severe impairments or death, with approximately 40% of patients in the study passing away within five years—mainly due to the disease itself. The findings underscore the need for increased awareness and improved diagnostic protocols. Researchers also advocate for enhanced participation in disease registries, such as Germany’s GENERATE network, to facilitate further research and better understanding of this rare condition.

In summary, starting immunotherapy early, especially with intravenous immunoglobulins, can considerably alter the disease trajectory of anti-IgLON5 disorder. This emphasizes the critical importance of early diagnosis and intervention for better patient outcomes.

Source: https://medicalxpress.com/news/2025-08-immunotherapy-anti-iglon5-disease-early.html