Unveiled central hypoventilation after tracheotomy in anti-IgLON5 disease: a case report

Abstract

Anti-IgLON5 disease is a recently described entity that has been associated with neurological symptoms and sleep disturbances including sleep breathing disorders. Sleep stridor as well as obstructive and less often central sleep apnea have been reported but rarely needing ventilation on tracheotomy. We report the case of a patient in whom obstructive sleep apnea with secondary development of dysphagia and recurrent aspiration pneumonia led to the diagnosis of anti-IgLON 5 disease. Acute respiratory failure due to laryngospasm required intubation and eventually tracheotomy. Yet hypoventilation persisted, and polysomnography demonstrated central sleep apnea alternating with sleep-related tachypnea. Nocturnal ventilation was thus reintroduced. The association of obstructive sleep apnea with dysphagia is a potential red flag for anti-IgLON5 disease, which remains an overlooked diagnosis. Breathing disorders can be complex in this context, with a mixed obstructive and central pattern whose central component can be unveiled after tracheotomy. This highlights the importance of closely monitoring sleep and respiration even after tracheotomy.

Citation: Tankéré P, Le Cam P, Folliet L, et al. Unveiled central hypoventilation after tracheotomy in anti-IgLON5 disease: a case report. J Clin Sleep Med. 2023;19(9):1701-1704.

Keywords: anti-IgLON5 disease; central hypoventilation; dysphagia; sleep stridor; tracheotomy.

Figure 1. Hypnogram and typical respiratory patterns. The hypnogram (top) shows highly fragmented sleep. Three 60-second time windows are presented, showing three respiratory patterns: tachypnea in N3 (left), normal respiratory rate in Wake (middle), and central apnea (cessation of airflow > 10 seconds with no respiratory effort observed on chest and abdomen belts) associated with desaturations (right).