ReviewNeurology(R) neuroimmunology & neuroinflammation2025
Anti-IgLON5 Disease 10 Years Later: What We Know and What We Do Not Know.
Review in Neurology(R) neuroimmunology & neuroinflammation, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
22 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Anti-IgLON5 Disease: A Systematic Review and Meta-Analysis.European journal of neurology · 2025Pooled it
- Neuronal Antibodies in Patients With Atypical Parkinsonism.Neurology(R) neuroimmunology & neuroinflammation · 2026Observational
- Article
- Prognostic Determinants of Presentation and Outcome in Anti-IgLON5 Disease.Neurology(R) neuroimmunology & neuroinflammation · 2026Observational
- The Intersection of Autoimmunity and Sleep: Anti-IgLON5 as the Key Player in a Case Series.Cureus · 2026Article
- Autoimmune Encephalitis in Acute Care-Pathology, Diagnosis, and Management.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026Review
- Human IgLON5 Antibodies Reduce Neuronal IgLON5 Clusters and Cause Motor Dysfunction in Mice.Neurology(R) neuroimmunology & neuroinflammation · 2026Article
- Brainstem pathology in anti-IgLON5 disease: new insights into early events and tau progression.Brain : a journal of neurology · 2026Article
- Novel methods for neural IgG characterization and validation in autoimmune neurologic disorders.Journal of immunological methods · 2026Review
- Efgartigimod as an Add-on Therapy for Anti-IgLON5 Disease: A Case Report.Neurology and therapy · 2026Article
- Clinical utility of human leukocyte antigen genotyping and immunoglobulin G4 autoantibody testing in autoimmune neurological diseases: A focused minireview.World journal of biological chemistry · 2026Review
- A patient with anti-IgLON5 disease associated with cerebral hemorrhage, multiple ischemic strokes, and kidney failure: case report.Journal of neurology · 2026Article
- Movement Disorders in Antibody-Associated Neurologic Diseases: A Nationwide Study.Neurology(R) neuroimmunology & neuroinflammation · 2026Observational
- Testing for neural antibodies in autoimmune encephalitis: who, what, where, when, why, and how.Therapeutic advances in neurological disorders · 2026Review
- Anti-IgLON5 disease presenting with myokymia and favourable response to FcRN-antagonist combined with oral glucocorticoids: case report.Frontiers in immunology · 2026Article
- Facio-Brachial Dystonic Seizures as an Atypical Presentation of Anti-IgLON5 Disease.Journal of central nervous system disease · 2026Article
- Progressive Supranuclear Palsy With Post-traumatic Frontal Lobe Damage Mimicking Anti-IgLON5 Antibody Disease.Cureus · 2025Article
- Serum Neurofilament Light Chain Correlates With Clinical Severity and Predicts Mortality in Anti-IgLON5 Disease.Neurology(R) neuroimmunology & neuroinflammation · 2025Article
- No anti-IgLON5 antibody in carefully diagnosed PSP patients with "atypical" or variant clinical features.Parkinsonism & related disorders · 2025Article
- Anti-IgLON5-Related Movement Disorders: A Series of Three Cases from a Tertiary Centre in India.Journal of movement disorders · 2025Article
Corrections and comments
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Authors and funding
7 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Anti-IgLON5 disease was identified 10 years ago, thanks to the discovery of IgLON5 antibodies and the joint effort of specialists in sleep medicine, neuroimmunology, and neuropathology. Without this collaboration, it would have been impossible to untangle fundamental aspects of this disease. After the seminal description in 2014, today there is growing evidence that most patients present a chronic progressive course with gait instability, abnormal movements, bulbar dysfunction, and a sleep disorder characterized by nonrapid eye movement and REM parasomnias, and obstructive sleep apnea with stridor. Unlike other autoimmune encephalitides, the response to immunotherapy is suboptimal. Neuropathologic studies in patients with a prolonged clinical course showed a novel 3-repeat and 4-repeat neuronal tauopathy mainly involving the hypothalamus and tegmentum of the brainstem. The absence of tau deposits in the brain of patients who died early, the demonstration that IgLON5 antibodies cause an irreversible decrease in cell-surface levels of IgLON5, and a disorganization of the neuronal cytoskeleton suggest that the disease is primarily autoimmune and the tauopathy a secondary event. After a decade, we now know the disease much better, but important issues still need to be addressed. We have to gather more information on the natural course of the disease, develop better treatments, and identify robust predictors of outcome. More basic research is needed on the physiology of IgLON5, how antibodies disrupt its function, and the downstream effects leading to neurodegeneration. Finally, better designed passive transfer and active immunization models are needed to confirm the pathogenic effect of IgLON5 antibodies.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.