Brainstem involvement has been recently rediscovered as a frequent feature of both AQP4-immunoglobulin G (IgG) and myelin oligodendrocyte glycoprotein (MOG)-IgG-positive NMO [1, 9, 10], and respiratory insufficiency due to brainstem or upper cervical cord lesions is nowadays the most common cause of death from NMO [11]. Here, AQP4 is linked to neuromyelitis optica.