Ventilation and tracheostomy insertion in anti-IgLON5 disease: A systematic review of cases

Abstract

Anti-IgLON5 is a rare condition with a diverse clinical spectrum. Mortality is high with patients developing respiratory compromise secondary to central hypoventilation or upper airway obstruction. Patients often progress to requiring ventilatory support and tracheostomy. There is a lack of knowledge regarding the clinical course of airway compromise in anti-IgLON5 disease and which factors predict future tracheostomy placement, understanding this is key to enabling shared decision making with patients. We conducted a systematic review in accordance with PRISMA reporting guidelines including all case reports and series relating to anti-IgLON5 disease published up to May 2024. 281 reports were identified, 74 reports containing 93 individual cases were included. 79.6 % described bulbar, airway, or ventilatory compromise. 19 required mechanical ventilation of which 11 progressed to require tracheostomy. Of those who did not undergo tracheostomy, 5 died, and 2 were reintubated. A total of 18 patients underwent tracheostomy; there were no examples of successful tracheostomy removal. 50 % of patients with stridor and 80 % of patients with vocal cord palsy required tracheostomy. Immunomodulatory treatment did not facilitate successful tracheostomy removal or sustained resolution of vocal cord palsy although treatment was started prior to tracheostomy insertion in the minority of cases. This will inform shared decision making with patients, acknowledging the limitations of this study, and illustrates the need for further prospective studies examining the response to immunotherapy in anti-IgLON5 disease.