Ondine's Curse

Mythology buffs are familiar with the classic tale of Ondine's curse. While it is a fictional mythical story, a real medical ailment was actually named after Ondine's curse due to the characteristic similarities between the curse portrayed in the fictitious story and the actual illness. One of the most rare types of stroke can produce brain deficits that cause symptoms of the disorder known as Ondine's curse.

 

The Myth

In the ancient mythical story, a young nymph named Ondine falls in love and marries. Upon discovering that her beloved has been unfaithful to her, she uses her supernatural powers to set a curse on him. Ondine’s curse is an unusual spell that robs her disloyal husband of rest. Her husband is doomed to a life in which he retains the ability to breath- but only while he is awake and conscious! Subsequently, afflicted with Ondine's curse- her victim cannot breath if he falls asleep - and therefore must choose between sleeping and remaining alive.

Symptoms 

The dramatic fictional tale has a real life counterpart in a rare disorder that is medically described as hypoventilation syndrome. Hypoventilation means a decrease in breathing. Ventilation is the normal mechanism our body uses to breath in oxygen and breath out carbon dioxide through the lungs. Patients who are afflicted with hypoventilation syndrome are not able to maintain regular ventilation during sleep and are at risk of completely stopping their breathing cycle during sleep.

The cessation of regular breathing seen in hypoventilation syndrome is more severe than the breathing cessation noted in the more prevalent condition of sleep apnea. Interruption of breathing can cause a decrease in the supply of oxygen to the body as well as excess buildup of carbon dioxide in the body.

Oxygen is necessary to sustain life, so the oxygen deficit may be so severe that it can cause fatigue, damage to the body’s organs or even death. The buildup of carbon dioxide is damaging to the body's functioning. This rare, but serious disorder can be present from birth due to a genetic anomaly, may be caused by congenital developmental abnormalities or may be caused by brain damage to the area of the brain that controls breathing.

When hypoventilation syndrome is caused by an injury in the brain, it is called central hypoventilation syndrome because the brain is the central commander of breathing.

Stroke

When a stroke causes Ondine's curse, symptoms include lack of breathing while sleeping. This results in abnormally low oxygen levels. Under normal circumstances, breathing is maintained in large part due to a reflex response to the body’s oxygen and carbon dioxide levels. However, those with central hypoventilation syndrome, do not posses the normal ability to respond to oxygen and carbon dioxide levels in the body.

What Part of the Brain is Responsible for Ondine’s Curse?

The area of the brain affected in this disorder is located in part of the brain called the brainstem. The lateral medulla, a region found in the lower section of the brainstem is where the reflex center for breathing is found. The lateral medulla adjusts the rate and depth of breathing to respond to the body's needs. When it is damaged, people can still breath voluntarily, meaning on purpose. This lack of reflex breathing causes the breathing problems in central hypoventilation syndrome to become exaggerated during sleep.

Treatment

Ondine’s curse was considered a fatal diagnosis in the past. However, with increased awareness of the physiology behind central hypoventilation syndrome along with improved technology, treatment for Ondine's curse has become possible. Medical management includes oxygen administration and mechanical breathing techniques.

Sources

Tremoureux L, Raux M, Hudson AL, Ranohavimparany A, Straus C, Similowski T, Does the supplementary motor area keep patients with Ondine's curse syndrome breathing while awake? PLoS One, January 2014

Mendoza M, Latorre JG, Pearls and oy-sters: reversible Ondine's curse in a case of lateral medullary infarction, Neurology, January 2013

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