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Central sleep apnea (CSA) is characterized by a lack of drive to inspire for at least 10 sec. In the CSA-syndrome accompanying arousals and desaturations of the arterial blood cause sleep disturbances and sympathetic nerve activations which lead to excessive daytime sleepiness and increase the risk for cardiovascular morbidity. There are six manifestations of CSA: a rare primary or idiopathic form, often in hypocapnic patients with an increased hypercapnic ventilatory drive; Cheyne-Stokes respiration, characterised by periodic CSA and a crescendo/decrescendo breathing pattern, often in patients with severe cardiac or neurological diseases; high altitude-induced periodic breathing (above 4000 m), CSA due to medical or neurological conditions; CSA due to drug or substance use; and primary sleep apnea of infancy. Besides the consequent treatment of the underlying medical conditions therapeutic options include the use of drugs, e. g. acetacolamide or oxygen, as well as non-invasive ventilation, e. g. continuous positive airway pressure (CPAP) or adaptive servo-ventilation.