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Volume 96, Issue 1

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To sleep, perchance

It was 50 years ago this September that Chicago professor Nathaniel Kleitman and doctoral student Eugene Aserinsky published a Science paper announcing their discovery of rapid eye movement (REM) sleep and its association with dreaming. Yet sleep, which occupies one-third of our lives, remains a mystery to be unraveled, even as physicians attempt to treat the conditions that impede it.

During one recent “sleep reading” at the Hospitals’ sleep-disorders clinic five physicians and two post-M.D. fellows gathered at 9 a.m. in the observation room, where eight flat, wide computer screens reside under eight black-and-white video monitors. On one video monitor flickered a still-life of a deserted rumpled bed, the sheets and blanket thrown aside, the pillow left at an angle, still indented from the previous night’s sleep—or lack thereof.

Each evening eight new patients enter the clinic, get wires and electrodes hooked up to their chests, abdomens, eyes, noses, and chins, and go (or try to go) to sleep. As the night wears on, jagged lines rise and fall across the computer screens, recording patients’ brain waves, breathing, heart rate, and eye and other body movements. When morning comes, the patients are unhooked from their wiring. Each Thursday the graduate fellows and instructors huddle around the week’s worth of polysomnograms, the doctors quizzing each other on what the lines might mean. Diagnoses can be hotly debated. “This is not a central decision crowd,” says Michael Kohrman, associate professor of pediatrics and neurology. “That’s what makes it fun.”

This Thursday’s first case is an 80-year-old woman complaining of fatigue. Using the polysomnogram software, the doctors “zoom” in and out, viewing the patient’s sleep lines for the entire night or shorter intervals. Jean Spire, clinic director and a professor in Neurology, Surgery, and the Committee on Computational Neuroscience, examines the woman’s sleep patterns at five-minute cycles. “This looks like nothing we’ve ever seen before,” he says excitedly. “She has central apnea. She completely stops breathing every 1.25 minutes.” He glances up at Mohan Kandari, a graduate fellow. “Did she look ill?” Kandari thumbs through a large bound notebook to read the night nurse’s observations. “No. She seemed healthy.”

“It’s central hypoventilation syndrome,” agrees Stephen Sheldon, a neurologist and pediatrician at Northwestern University. Also called Ondine’s Curse, the uncommon syndrome is found usually in children but rarely develops later in life, as seems to be the case here; its name comes from the mythological water sprite condemned to stay awake in order to breathe. In Ondine’s patients the central nervous systems fail to control breathing during sleep; patients fall asleep, their breathing stops, and they wake again. “Put her on Ritalin and give her a mass ventilation mask,” Sheldon suggests.

Though much has changed in sleep medicine over the past half century, Ritalin has been used to treat sleep disorders since the 1960s, when it took the place of amphetamines. Mass ventilation refers to another type of treatment, continuous positive airway pressure (CPAP), in which a mask is worn over the nose during sleep while compressed air is gently forced through the nose to keep the airway open.

The sleep doctors page the patient’s attending physician to discuss her medical history and give their diagnosis, then they wheel around the room discussing the day’s other cases, mostly sufferers of common forms of sleep apnea, when breathing stops temporarily during sleep, and hypopnea, in which breathing slows. Fifteen minutes later Melinda Henderson, a second-year pulmonary resident, appears. The 80-year-old “presented with congestive heart failure,” she tells the physicians. “She’s been difficult to treat but has stabilized, in part because she was drinking eight cans of beer a day a year ago and now she’s down to one can a day. She has significant dementia.” That fact, the doctors acknowledge, may mean that CPAP can’t be used—the patient might not be able to pull the rip cord should something go wrong in the middle of the night.

“Our tentative diagnosis is Ondine’s Curse,” Spires says. “Do you know of it?” Henderson raises her eyebrows in appreciation. “I know a little bit about it,” she replies. “It’s pretty rare, isn’t it? That must be really exciting for you to see.” The fellows’ eyes sparkle, clearly proud to have helped diagnose a rarity. Then it’s back to the other part of their work—trying to find a way for another patient to get a good night’s sleep.—Sharla A. Stewart


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