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Ondine's curse

Ondine's curse, central alveolar hypoventilation syndrome, or primary alveolar hypoventilation, is a respiratory disorder that is fatal if untreated. Persons afflicted with Ondine's curse are unable to breathe without conscious intervention; if they fall asleep, they will die. Its name is a reference to the myth of Ondine, who cursed her adulterous husband that he would stop breathing and die if he ever fell asleep. more...

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This disorder is associated with a malfunction of the nerves that control involuntary body functions (autonomic nervous system) and abnormal development of early embryonic cells that form the spinal cord (neural crest). The abnormal neural crest development can lead to other abnormalities such as absent or impaired bowel function (Hirschsprung's disease). Most affected individuals have an abnormality in PHOX2B or other genes.

Most people with congenital Ondine's curse do not survive infancy, though they can be kept alive with a ventilator. It was first described in 1962 by Severinghaus and Mitchell in three patients following surgery to the upper cervical spinal cord and brainstem.

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A false alarm: no … it's more freudian - theories on panic attacks
From Psychology Today, 9/1/93

Panic attacks. The anxiety and panic are real. And terrifying. But they've been a mystery to researchers and clinicians who've been trying for years to pinpoint the cause. Now, the psychiatrist who put the disorder on the map says he's found the culprit and it's strictly biological. It's not a matter of past experience. It's a case of faulty wiring - a defect in the way our brains warn against suffocation.

Along with assorted other alarms, the human brain has a warning system that fires whenever the amount of carbon dioxide in the blood becomes too high - a sure sign of oxygen deprivation. In some people, the system is overly sensitive and fires spontaneously at the slightest increase in blood [CO.sub.2], says Donald Klein, M.D., professor of psychiatry at Columbia University.

The false alarm then sets off a cascade of events that culminate in panic attack. Just before the attack, the victim is overwhelmed by feelings of suffocation and tries to compensate by breathing deeply. But it's too late, and heavy breathing can't alleviate the feeling of suffocation.

"The brain then says |let's get out of here,'" observes Klein. "People having panic attacks often run to the window and throw it open. They are trying to get some air in."

Proof that a suffocation monitor exists, he says, lies in infants who are born without one, those with the defect known as Ondine's curse (sleep-induced apnea). Suffocation is particularly adversive to the human brain, and most infants cry instinctively whenever their nose are held - a minor panic reaction. But these children calmly suffocate.

Klein dismisses intense fear as a cause of panic. The outstanding thing about panic is that it is not motivated by fear," he says. "That's just a mistake." He points to the many people who get panic attacks during sleep or deep relaxation - states where oxygen deprivation is the norm but little anxiety exists. Besides, if fear were behind panic, sufferers should show increased levels of adrenaline, and they don't.

Sure, says a team of Pittsburgh and New York psychiatrists, some biological predisposition does exist. But panic attacks don't happen in the absence of certain kinds of early experience and psychic conflict.

Resurrecting the theories of Sigmund Freud, they insist that both biology and early experience conspire to cause panic.

In their neo-Freudian model, innate temperament, psychodynamics, parental behavior, and objective and subjective experience all play a role.

People with the disorder are born with a neurophysiological irritability that shows itself as early fearfulness. As children, they typically fear new or unfamiliar situations. Their parents, perhaps arudous or prone to panic themselves, fail to case the way they fit with the world and wind up exacerbating the child's fearful nature.

The child feels threatened and suffocated by their parents' behavior but at the same time becomes overly dependent on them. In adolescence, the child becomes very complacent and eager to please, but at the same time resentful of authority.

Now the steep slide towards the first panic attack begins. At some point in early adulthood, something happens, usually involving a powerful other or figure of authority, to make the future sufferer extremely angry or upset. Already predisposed toward fear, they become frightened at their intense negative emotions. Anger leads to a physical response - heavy heartbeat, sweating, anxiety - and this triggers a fight-or-flight reaction in the brain: a panic attack.

The first attack begins a vicious cycle. Fear of another panic attack leads to more psychological vulnerability, which leads to more fear that may result in another panic attack. In some sufferers, the attacks may occur over a brief period of time and then never reappear. But in others, the endless chain of fear and panic may go on for years, virtually destroying their ability to function normally, according to Katherine Shear, M.D., professor of psychiatry at the University of Pittsburgh, and colleagues.

Their bold new theory, reported in the American Journal of Psychiatry (Vol. 150, No. 6), is the outgrowth of interviews with nine patients suffering from panic disorder, published reports of psychological characteristics of panic patients, and data from animal and infant research on temperament. All patients describe themselves as shy and nervous as children, and their parents as suffocating, critical, and demanding. All reported later problems with overdependency and fear of authority. And all described stressful incidents just before the onset of their first panic attack.

COPYRIGHT 1993 Sussex Publishers, Inc.
COPYRIGHT 2004 Gale Group

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