Laryngeal Symptoms in Anti-IgLON5 Disease: Case Reports and a Systematic Review of Airway Management and Recommendations

Abstract

Anti-IgLON5 disease, a rare neuroimmunological disorder, frequently manifests with aerodigestive symptoms such as dysphagia, dysarthria, acute respiratory failure, and eventually death. The Anti-IgLON5 disease is under-recongnized in the field of otolaryngology. Therefore, the aim of this systematic review is to summarize the current literature on airway management in Anti-IgLON5 disease, a potentially life-threatening condition, and present two cases that highlight the critical importance of early recognition and intervention for laryngeal symptoms. A comprehensive search of PubMed/MEDLINE for studies related to Anti-IgLON5 and respiratory complications was conducted, focusing on articles published before September 13, 2024. Cases involving airway presentation and management were included. The literature review included 16 articles yielded 28 patients, revealing that 78.5% required tracheotomy due to respiratory failure or laryngeal symptoms. Dysphagia and obstructive sleep apnea were the most common presenting symptoms of the disease. Bilateral vocal cord (VC) paralysis was observed in 67% of cases, though it often developed later in the disease course. The two case reports align with these findings, illustrating that early tracheotomy may be necessary to prevent sudden death, even in patients with initial VC mobility. Anti-IgLON5 disease can lead to severe airway compromise, necessitating early intervention. Regular otolaryngological evaluation and polysomnography are critical in managing these patients, and tracheotomy should be considered a key preventive measure against respiratory failure. Awareness of IgLON5 antibodies as a differential diagnosis in patients presenting with unexplained laryngeal symptoms is essential for timely intervention.

Keywords: Anti-IgLON5—IgLON5—Laryngology—Autoimmunology—Dyspnea.