Severe sleep-related hypoventilation in antibody-positive and antibody-negative autoimmune encephalitis: an emergency

BACKGROUND AND OBJECTIVES: Although sleep disorders of neurological origin have been documented extensively in cases of autoimmune encephalitis, the impact and clinical significance of sleep disordered breathing remain underexplored. We aimed at determining the prevalence of sleep disordered breathing in autoimmune encephalitis. METHODS: A cohort study of consecutive patients with autoimmune encephalitis referred to a tertiary centre for sleep pathology was conducted over a period of 4.5 years. Patients underwent an overnight video-polysomnography with capnography. We investigated the presence of sleep-related breathing disorders, including sleep-related hypoventilation, sleep apnoea-hypopnoea syndrome, central hyperventilation, irregular breathing and excessive sighing. RESULTS: 46 participants with autoimmune encephalitis were included, with a median (interquartile range) age of 60 (40-69) years, of whom three were previously treated with noninvasive ventilation after acute respiratory failure and three were previously treated with positive airway pressure. A total of 26 (56.5%) out of 46 participants had sleep disordered breathing, including 19 (41.3%) out of 46 participants with moderate to severe apnoea-hypopnoea syndrome. Capnography revealed sleep-related hypoventilation in 12 (36%) out of 33 cases, including four (80%) out of five anti-IgLON5, one (25%) out of four anti-glutamic acid decarboxylase-65, one (25%) out of four anti-CASPR2, and six (42.8%) out of 14 antibody-negative cases. Nocturnal stridor was observed in two (40%) out of five anti-IgLON5 cases. Overall, 20 (43%) out of 46 subjects required respiratory support, including positive airway pressure (n=13) and noninvasive ventilation (n=7). CONCLUSION: More than one-third of participants with autoimmune encephalitis required nocturnal mechanical ventilation. Particular attention should be paid to sleep-related hypoventilation (assessed by nocturnal capnography) in patients with autoimmune encephalitis to guide timely respiratory support and reduce morbidity and mortality.