• Miljenka Planjar-Prvan, Pavao Krmpotić, Ilija Jergović, and Ivan Bielen.
• Klinika za neurologiju, Klinicka bolnica Sveti Duh, Psihijatrijska bolnica Vrapde, Zagreb, Hrvatska. miljenka.planjar@gmail.com
• Acta Med Croatica. 2010 Oct 1;64(4):297-301.

AbstractOndine's curse syndrome primarily refers to cases with congenital central alveolar hypoventilation, but the term can also be used for acquired cases and implies central sleep apnea that occurs as a manifestation or complication of focal lesion in the area of the dorsolateral segment of medulla oblongata. It occurs rarely, but can lead to fatal outcome. Based on our own case report, the aim of this article is to review its clinical symptoms, and appropriate diagnostic and therapeutic procedures. We present a patient who had symptoms of vascular lesion of the dorsolateral segment of the medulla, which was verified by magnetic resonance imaging. On day 12 of his hospital stay, in the early morning, rapid development of coma was observed, which was an expression of serious respiratory failure with dominant hypercapnia. In the beginning, urgent intubation and mechanical ventilation were necessary, while in the later course of the disease breathing was assisted by noninvasive methods of Bilevel Positive Airway Pressure (BiPAP) and Continuous Positive Airway Pressure (CPAP). Throughout the night, polygraph recording confirmed the diagnosis of the central sleep apnea syndrome. The course of the disease was favorable, with a very slow but constant improvement of respiratory function. According to literature data, the disease course is not always favorable. There are published cases where it was concluded that ventilatory support was no longer needed but after a long period of normal breathing hypoventilation and death occurred suddenly during sleep. The treatment of central hypoventilation consists of ventilatory support, but there were also attempts of medicamentous treatment with the common aim of raising alertness and reactibility of the automatic breathing center. It is important to emphasize that patients with the risk of central sleep apnea should not be supplied with oxygen without arterial blood gas monitoring because of the possibility of delaying the right diagnosis. The use of oxygen in patients who already have hypercapnia due to hypoventilation could further intensify hyporeactivity of the breathing center and lead to respiratory arrest.

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