Kleine-Levin Syndrome sufferers can sleep 20 hours a day for months at a time, which is why it’s commonly known as “Sleeping Beauty Syndrome.” But unlike the well-known Disney character, those afflicted with KLS are forced to confront the repercussions of lost time.
Sleeping Beauty was born in 1812, the brainchild of Jacob and Wilhelm Grimm. Her story is well known: A beautiful princess, cursed by a vindictive fairy, falls into a 100-year sleep before she is woken by the kiss of a traveling prince. In the years since, the story of Brier-Rose, as they called her, has been turned into a Disney movie and a ballet scored by Tchaikovsky, not to mention an army of commercial goods. But it wasn’t until the 21st century that her name became associated with Kleine-Levin Syndrome (KLS), a rare sleep disorder.
In 2014, a young woman in Britain who slept 22 hours a day was declared “the real sleeping beauty” by theDaily Mail. “Woman has rare condition,” read the subtitle, “which means she is only awake for two hours a day.” The year before, CBS reported on a Pennsylvania teenager who slept for 64 days in a row, waking only to eat. These and other victims told stories about missing birthdays and holidays and presidential elections, about being unable to attend high school and college or move away from home.
Like famous fictional sleepers — Sleeping Beauty, Rip Van Winkle — KLS patients go to bed in the midst of one reality and wake to another. But these stories sidestep the grief and loss that comes with the theft of time. Why has the escape into sleep inspired fantastical fiction? What does reality mean for people and characters who dream for days, weeks, even years at a time?
The Sleep Virus
Kleine-Levin Syndrome is a disorder characterized by an excessive need for sleep. During KLS episodes — which can last for days, weeks or even months — patients may sleep up to 20 hours per day, waking only to eat or use the bathroom.
While awake, they show changes in behavior and personality; they are often confused and lethargic, irritable, depressed and childlike. Apathy is common — they have little desire to engage with the outside world. They may hallucinate, eat excessively and even display uninhibited hypersexuality. Many experience intense sensitivity to light and noise. The KLS Foundation also lists “reduced understanding of the world” as a symptom.
What do we know about the cause of KLS? “The truth is, not a lot,” said Mali Einen, clinical research coordinator at Stanford’s Center for Narcolepsy. “There’s no definitive test. The thinking is that it’s probably viral or immune-related, sort of like one catches a virus...that doesn’t clear completely. That’s why it recurs.”
According to a 2005 review, published in Brain, KLS is most often preceded by an infection, head trauma or alcohol consumption. Roughly 50 percent of the patients reviewed were beset by flu-like symptoms disease days before their first episode. But because the patient pool is so small — the disorder is estimated to affect one in a million worldwide — large studies are lacking, but the Brain review notes the median age of onset to be 15-years-old. And though KLS can affect children and adults of both genders, 70 percent of those diagnosed are adolescent males. On average, the syndrome lasts eight years, with episodes spread about three months apart.
It’s tempting to make a joke here about KLS patients as being the most adolescent-male of adolescent males — sex-crazed, gluttonously hungry and sleeping, quite literally, at all hours of the day — but KLS is no laughing matter. Dr. Isabelle Arnulf, professor of neurology at Pitié-Salpêtrière University Hospital, president of the French Society for Sleep Research and Sleep Medicine and one of the world’s leading KLS researchers, wrote that early episodes can last 20 to 24 hours a day. But “as time passes, they [sleep] 11 to 12 hours and are lying all day long in their room without sleeping, apathetic, in the dark, exhausted, slow and unable to understand well the world around them, and are not motivated to go out.”
Einen described patients as cognitively impaired in these rare moments of wakefulness during an episode. Indeed, their brains often slow. The Brain review describes abnormal speech, even muteness. One patient described “[struggling] to follow a thought.” Another felt “everything was going too fast.”
“They’re not going to engage in conversation,” said Einen. “Some people revert back to being childlike.” It’s as though they lack a filter, she explained. “The way a 16-year-old boy would talk to other 16-year-old boys might not be the way he would talk to his mom.”
She emphasized that the hypersexuality that can be a symptom of KLS is not threatening to others, though it can be humiliating for patients. They might make inappropriate sexual comments or masturbate where others are present. Most have little to no memory of what happened during an episode.
“They’ve lost that chunk of time,” Einen said. “It’s gone; they don’t remember it.” Maybe it’s better they don’t.
Sleeping Through History
Characters like Sleeping Beauty and Rip Van Winkle are trapped in sleep, but they’re also elevated by it, made mythic.
Dreamed up by Washington Irving in 1819, Van Winkle is symbolic of the years before and after the Revolutionary War. Irving’s protagonist was a descendent of Dutch colonists and a loyal subject of King George who falls asleep after a night of mountaintop revelry. When he wakes, the American Revolution has come and gone. Van Winkle has lost not just time, but his own identity. He has woken up in a world at once familiar and foreign. “Does nobody know poor Rip Van Winkle!” he cries out.
Like Van Winkle, KLS patients miss out on their personal lives as well as cultural moments. During episodes, patients are not able to take care of themselves, attend school or go to work. In between episodes, patients have no sign of dysfunction and can resume normal life.
Or can they? If patients have gaps in memory and have missed critical moments in their lives, both personal and cultural, to what extent is the resumption of normal live truly possible?
“I think the part that’s difficult,” said Einen, “is that when they come out of [an episode], they do feel the loss of that gap. They’re not aware of the passing of time; they have to be told. It’s almost like they’ve been in a state of pseudo-hibernation. Life has moved on for other people, and now they have to pick up.” Their KLS may eventually disappear, “the sense of loss is overwhelming.”
Because of the preceding flu-like symptoms that accompany many cases, as well as factors like the young age of onset and recurrence, doctors at the Center for Narcolepsy at Stanford believe KLS may be an autoimmune disease. There’s no proven psychological component, but Einen noted that episodes can be brought on by stress, as in narcolepsy — a divorce in the family, or a period of exhausting travel.
Stress as a trigger would not contradict an autoimmune hypothesis; on the contrary, the immune system fights stress. A research group at Stanford led by Dr. Emmanuel Mignot is exploring the role of the human leukocyte antigen system, which plays a role in encoding proteins that regulate the immune system. If KLS does indeed have an autoimmune basis, existing drugs that reduce autoimmunity could be tested on KLS patients.
For now, there is no standard treatment. Lithium has been shown to work in some patients, but most simply have to wait until it goes away — sleep it off, so to speak. Some patients try to fight the drowsiness and pursue normal activities, but few are successful. The Brain review describes a need for sleep so overpowering that one male teenager was found sleeping beneath a neighbor’s porch. Another left his school classroom, laid down in the hallway and fell asleep. A third patient was found asleep on a public sidewalk.
After hypersomnia, or excessive sleep, the next most common symptom is “derealization.” Writing in Brain, the study’s authors described this as “a feeling of unreality (surroundings seemed wrong, distorted or unreal, as in a dream) or of disconnected thinking during episodes was… felt [by patients] to be the most specific symptom of the syndrome.”
Some experienced hallucinations, paranoia and delusions. They feared being robbed, filmed or poisoned, while others described their surroundings as nightmarish and distorted. One patient’s own voice appeared “strange to himself.”
Beauty Has Its Price
Disney’s version of Sleeping Beauty is a whitewash. Few fans of the 1959 film know the more frightening original story.
The writer Giambattista Basile is credited with the first version, “Sun, Moon, and Talia” (1634), which also happens to be the most disturbing. A princess named Talia falls into a deep sleep after a piece of flax gives her a splinter; this has been predicted by land’s wise men and astrologers. When a traveling king, hunting nearby, comes across Talia, he rapes her. She becomes pregnant with twins, one of whom sucks the splinter from her finger and wakes her. When the king returns to Talia, he finds his “mistress” with two children, Sun and Moon.
His wife, the queen, is enraged and orders the palace cook to turn the three into food to be eaten by her husband. When the cook refuses, the queen lures Talia to the castle and orders her burned alive. As Talia takes off her clothes, “with every item...she uttered a loud scream.”
Hearing Talia’s shrieks, the king rages at the queen, his wife — “You renegade bitch” — and orders that she, not Talia, be cast into the fire. He then marries Talia, who “enjoyed a long life with her husband and her children.”
The story’s gruesomeness is just barely tamped down in the next version, Charles Perrault’s “The Sleeping Beauty in the Wood,” published in 1697. Here, Perrault turns the rapist king into an admiring prince who wakes Sleeping Beauty after she has slept for 100 years. We are kindly reassured that Sleeping Beauty looks “not a bit less charming or beautiful for all that.”
But the prince’s mother, the queen — who is “of the race of the ogres” and may even have “ogreish inclinations” — resents Sleeping Beauty and the two children she’s birthed. Once he becomes king and goes off to war, his mother plans to eat Sleeping Beauty and the children “with a sauce Robert.” The queen is eventually thrown into a bathtub full of toads, vipers, snakes and “all sorts of serpents.” Naturally, the new king “could not but be very sorry, for she was his mother; but he soon comforted himself with his beautiful wife and his pretty children.”
“Little Brier-Rose,” penned by the brothers Grimm in 1812, cuts the ogress, cannibalism and murder of the previous version. In their hands, Sleeping Beauty becomes a tale of sexual awakening, maybe even sexual misadventure:
The princess grew and became a miracle of beauty. One day, when she had just reached her fifteenth year, the king and queen went away, leaving her all alone in the castle. She walked from room to room, following her heart's desire. Finally she came to an old tower. A narrow stairway led up to it. Being curious, she climbed up until she came to a small door. There was a small yellow key in the door. She turned it, and the door sprang open.
She found herself in a small room where an old woman sat spinning flax. She was attracted to the old woman, and joked with her, and said that she too would like to try her hand at spinning. She picked up the spindle, but no sooner did she touch it, than she pricked herself with it and then fell down into a deep sleep…
[A] thorn hedge grew up around the entire castle, growing higher and higher, until nothing at all could be seen of it.
Princes, who had heard about the beautiful Brier-Rose, came and tried to free her, but they could not penetrate the hedge. It was as if the thorns were firmly attached to hands. The princes became stuck in them, and they died miserably. And thus it continued for many long years.
Then one day a prince was traveling through the land. An old man told him about the belief that there was a castle behind the thorn hedge, with a wonderfully beautiful princess asleep inside with all of her attendants. His grandfather had told him that many princes had tried to penetrate the hedge, but that they had gotten stuck in the thorns and had been pricked to death.
"I'm not afraid of that," said the prince. "I shall penetrate the hedge and free the beautiful Brier-Rose."
When the prince approaches the thorny hedge, it turns to flowers that separate, allowing him through. With his kiss, Brier-Rose wakes up and everyone, yes, lives happily every after. Whatever adjustments Sleeping Beauty has to make in this new world — reckoning with the loss of her family; connecting with new in-laws; navigating motherhood and marriage; adjusting to an entirely new century — are ignored.
The Myth of Happy Endings
Both Rip Van Winkle and Sleeping Beauty may have been inspired by an ancient legend called “The Seven Sleepers of Ephesus.”
In this tale — adopted as both Christian and Muslim lore — seven men, noble religious believers, conceal themselves in a cave to escape the persecution of an idol-worshiping emperor. Miraculously, they wake more than 200 years later to find the empire converted to Christian (or Muslim, depending). The sleepers are celebrated for their faith and fortitude.
For the Seven Sleepers, Grimm’s Sleeping Beauty and even, to some extent, Rip Van Winkle, sleep is an escape hatch into an ideal world. The fantasy is that life will be better when you wake up than it was when you went to sleep, that you need not do anything to better the world but wait idly for the better version to present itself. It is the fantasy of inaction rewarded with action: Rip Van Winkle goes to sleep in pre-Revolutionary America and wakes to national independence. Sleeping Beauty goes to sleep as a cursed singleton and wakes as a soon-to-be bride. The Seven Sleepers go to sleep as infidels and wake to a society that values them, a society whose values parallels their own.
It is painful, of course, to find that the world has gone on without you, but in these stories, it’s a luxury, too. Active citizenship isn’t necessary to affect political change. Dating isn’t necessary to find a partner—even a royal one.
But KLS patients enjoy no such fantasy. When they wake, they must fight through grogginess to piece together memories and grapple with disorienting loss, both temporal and experiential.
“I can’t help but think that sense of loss or sadness is something that most 16-year-olds, 14-year-olds, don’t experience,” said Einen. “Young people [with KLS] have to grow up faster.”
They must do the hard work, not just of waking up but of living.