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Accepted version

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2024-11-01

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Please use this identifier to cite or link to this item: https://hdl.handle.net/2445/218676

Clinical presentations and antibody mechanisms in anti-IgLON5 disease

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https://doi.org/10.1016/j.neurol.2024.07.004

Abstract

Anti-IgLON5 disease is a rare neurological disease, identified just ten years ago, where autoimmunity and neurodegeneration converge. The heterogeneity of symptoms, sometimes mimicking pure neurodegenerative diseases or motor neuron diseases, in addition to lack of awareness, represents a diagnostic challenge. Biomarkers of neuronal damage in combination with in vivo visualization of tau deposition using positron emission tomography (PET) scanning could represent a major advance in monitoring disease progression. Recent studies with more autopsies available have helped refine the knowledge of the pathological features of the disease and strengthen the autoimmune hypothesis of the disease. Although the pathogenesis of anti-IgLON5 disease remains unclear, the irreversible antibody-mediated decrease of IgLON5 clusters from the cell surface and alterations produced in the cytoskeleton, as well as the behavioural abnormalities and signs of neuroinflammation and neurodegeneration observed in the brains of animals infused with antibodies from patients by passive transfer, which have recently been published, support the autoimmune hypothesis of the disease. This review aims to summarize these important aspects and recent advances in the pathophysiology of anti-IgLON5 disease.

Subject

Malalties autoimmunitàries, Malalties neurodegeneratives, Autoanticossos, Models animals en la investigació, Encefalitis, Marcadors bioquímics

Subject (English)

Autoimmune diseases, Neurodegenerative Diseases, Autoantibodies, Animal models in research, Encephalitis, Biochemical markers

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Articles publicats en revistes (Medicina)
Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer)

Citation

GAIG VENTURA, Carles and SABATER BAUDET, Lidia. Clinical presentations and antibody mechanisms in anti-IgLON5 disease. Revue Neurologique. 2024. Vol. 180, num. 9, pags. 940-949. ISSN 0035-3787. [consulted: 8 of August of 2026]. Available at: https://hdl.handle.net/2445/218676

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