EARLY treatment with intravenous immunoglobulins (IVIG) significantly improves long-term outcomes and survival in patients with anti-IgLON5 disease, according to findings from a large international cohort study.
Anti-IgLON5 disease is a rare, progressive autoimmune encephalopathy that frequently results in severe disability or death. In this retrospective study spanning a decade and involving 107 patients from Germany, Spain, and the Netherlands, researchers evaluated whether the timing of immunotherapy could alter the disease trajectory. The study included patients with confirmed anti-IgLON5 antibodies and a median follow-up of more than five years.
Only 23% of patients received immunotherapy within the first year of disease onset. Among these, 36% were treated with IVIG and 52% with rituximab. Despite similar baseline disability scores, early IVIG treatment was the only approach independently associated with both reduced long-term disability and improved survival. Patients who received IVIG within the first year had significantly lower disability scores at follow-up and no reported deaths, compared with a 37.5% mortality rate among patients who received other early immunotherapies.
Overall, 41% of patients died during the follow-up period, with nearly two-thirds of these deaths attributed directly to anti-IgLON5 disease. Multivariate analysis identified early immunotherapy—specifically early IVIG—as the only modifiable factor linked to improved outcomes.
These findings underscore the importance of timely diagnosis and initiation of immunotherapy, particularly IVIG, in this devastating neurological condition. While the study design was retrospective and subject to limitations, it offers critical insights into treatment strategies that may meaningfully alter the course of anti-IgLON5 disease.
Larger prospective trials are needed to confirm these results, but for now, the evidence suggests that earlier use of IVIG could be key to improving both survival and quality of life in affected patients.
Reference:
Grüte T et al. Early Treatment With Intravenous Immunoglobulins and Outcomes of Patients With Anti-IgLON5 Disease. JAMA Neurol. 2025.
