The Sleep Disorder That Makes People Hallucinate Their Own Death

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Every night for the last 12 years, Anne has slept by the phone, waiting for calls. A few minutes after half past two in the morning, perhaps, the phone will ring.

Sometimes the caller is a nuisance, which is why Anne prefers to keep her last name private. But more often than not, somebody is calling for a few words of reassurance. They ask Anne a straightforward question: Am I still alive?

Anne tells them that, yes, they are alive, and then they hang up, released from their confusion, at least for tonight.

The callers have narcolepsy, a debilitating malady best known for its more public symptoms. People who live with narcolepsy are prone to sudden attacks of sleep, often at inconvenient times, such as when they are in the shower or at a staff meeting. They also suffer from extraordinary sleepiness during the day and a condition known as cataplexy, the real hallmark of the condition, in which some of a person’s muscles suddenly lose their strength and that person may just fall to the ground, lose grip on something they are holding, or perhaps have their facial muscles fall into an appearance over which they have no control. These situations may last minutes or seconds depending on the individual. They can be triggered by emotional surges, such as laughing at a joke or becoming angry.

But there is a more private symptom of narcolepsy that is even more frightening: the hallucinations.

One of the features of narcolepsy is the ability to slip straight from wakefulness into REM (rapid eye movement) sleep. For most people, there are four distinct stages of sleep that precede REM sleep, and each of these is characterized by a different pattern of brain waves. These four stages culminate in “deep sleep,” a period in which the brain is least active. In this state, sleep regulates a number of tasks that need to be accomplished after the wear and tear of another day.  You don’t dream in deep sleep. Your mind gets out of the road so that your body can look after itself.

About an hour and a half after falling asleep, a profound change comes over the nature of sleep, marking the arrival of REM sleep. In this state, the frenetic activity of your eyes behind closed eyelids indicates that the brain is tired of being stuck on the sideline and wants to start playing again; more than that, it wants to captain the team. In deep sleep, your brain is still while your body is quiet and stable with slow regular heart rate and low blood pressure. In REM sleep, however, your body is physiologically active, and your brain gets restless. Most dreams, although not all, take place in REM sleep.

Narcoplepsy allows the rest of us to understand what can happen when the cycle of sleep gets disrupted and for some reason REM sleep decides to come before the other stages. People with narcolepsy may well be in the REM stage within minutes of falling asleep.

The immediate onset of REM can bring immediate dreams. The problem is that without the slow process that usually leads to REM, a person with narcolepsy can have genuine problems telling if their dreams are real or not. They may wake up in the morning and head off to appointments they have only dreamed about, or start looking desperately for the keys of cars they have only dreamt they own. To make matters worse, the dreams are often nasty.

That is why they might ring Anne in the early hours of the morning. They have just dreamed that they are dead. They need an outsider to tell them this is not true.

Anne is a nurse who first encountered narcolepsy when she was training in 1956; one day she found a fellow nurse propped up against a wall, able to hear but not move. She later found herself married to a schoolteacher who has narcolepsy, a condition that isn’t life-threatening but that has treatment rather than a cure. It is a long-haul illness. Anne’s husband waited for ages before he found a doctor who was able to respond appropriately. In the meantime, he would come straight home from school and fall asleep. He would then need another nap later in the evening to wake himself up enough in order get himself to bed. Once he was in bed, he also had PLMD (periodic limb movement disorder), which meant that his night’s sleep usually cost Anne a few bruises. Some of the couple’s children also have narcolepsy; a genetic factor has often been observed, but the condition is not inevitable. It is possible for one identical twin to have narcolepsy and the other not.

Part of dealing with all this has meant, for Anne, being available to help others. “A woman rang me at some ungodly hour, just after half past two,” she says. “There was an angel in her room so she needed to know if she was dead yet. I told her she wasn’t and she said that was fine, but she sounded slightly disappointed. She said that it had been very pleasant flying around the room.”

Anne does not answer the phone between dawn and noon.

* * *

Narcolepsy is rare, although the incidence of it varies from country to country. It is more common than leukemia and affects a similar percentage of people as Parkinson’s disease, at least one in two thousand. But like many sleep disorders, narcolepsy has a long history of not being taken seriously.

It was first marked in the scientific record in 1881 by a French doctor, John-Baptiste-Edouard Gélineau. Born in 1828, Gélineau became a naval doctor and had some risqué adventures in the Indian Ocean, before settling down to private practice in a rural community, where he had time and space to indulge his interest in natural history. He made his money when, in 1871, he developed a heady brew of bromide, antimony, and picrotoxin, which he marketed as a cure for epilepsy and sold in tablet form. The success of the product probably says more about the desperation, at the time, of families living with epilepsy than it does about the efficacy of the pills. The history of sleep medicine is likewise full of wonder cures. People will pay almost anything for a decent night’s sleep.

Gélineau went on to establish a neurological clinic in Paris. One day, a 38-year-old approached him with a bewildering problem. The man was a vital member of the local community; he sold wine barrels. But this active businessman was prone to sudden episodes of sleep in any situation. He also had a proclivity to fall down for no apparent reason, a condition Gélineau called astasia but which we know as cataplexy.

Gélineau’s observations were astute. He noted that astasia was different from epilepsy in that an epileptic seizure tended to make muscles tighten and contract. Astasia had the opposite effect; the muscles turned to jelly, and furthermore, the attacks seemed to switch off as suddenly as they switched on. He rightly observed that these attacks seemed to follow occasions of strong emotion which, for a French wine barrel merchant, were not infrequent. Gélineau was also correct in his deduction that the problem was somehow located in the brain. In 1880, he coined the term narcolepsy to describe the whole complex.

But the medical mainstream wasn’t especially interested in the findings of a maverick, and it wasn’t until the discovery of REM in 1953 that his work was given credit.

Meanwhile, Gélineau had found there were other ways to help people sleep. In 1900, he returned to Bordeaux to make wine.

* * *

Elizabeth Hickey became aware of the symptoms of narcolepsy following a bout of glandular fever when she was 16. But her struggles with the condition go back even further. At school, she had trouble concentrating and was often in trouble with teachers, who liked her to sit near the front of the class so that they could keep an eye on her. This old-fashioned approach to discipline may have some valid basis: by creating mild stress for the pupil, a teacher may be helping them release enough adrenaline to stay awake.

Elizabeth’s behavior might now be described as attention-deficit/hyperactivity disorder (ADHD); she believes her brain was doing gymnastics to compensate for poor sleep and to keep her awake during the day.

Elizabeth is middle-aged now. Though she still has narcolepsy, the drug that enables her to function is Ritalin—the same drug that can arouse controversy for its use in treating children with ADHD. There’s a theory that ADHD affects poor sleepers who get through the day by having numerous very short “micro-sleeps,” which may last only seconds but which are long enough to rupture concentration and reduce the world to fragments. There are others who’d say that poor sleep is a symptom rather than a cause of ADHD.

Elizabeth showed all the classic signs of narcolepsy. At the age of 11, she surprised herself by being able to swim across a pool. She was delighted by her achievement and the emotion triggered a cataplectic episode as a result of which she lost the ability to move her limbs, and but for the vigilance of a friend who fished her out, she would have drowned.  Years later, she found herself as the mother of twins and struggling to cope with profound exhaustion. She was so sleepy all the time that she found it hard to function, but she was inclined to attribute this to the demands of having two babies in the house. One day, some friends arrived unexpectedly on her doorstep. The sheer delight of seeing them once again activated her cataplexy and she fell to the floor.

Cataplexy is just one of a suite of symptoms. As a girl, Elizabeth had an extraordinary number of imaginary friends, each of which was absolutely real to her, and now wonders if this was a result of narcoleptic hallucination. Even as an adult, Elizabeth suffered from hallucinations; she can well recall driving with her children in the backseat and experiencing terrifying mirages of oncoming trucks. She has experienced sleep paralysis and, with that, the most frightening dreams.

“I dreamt that things were eating me up. That I was being interfered with,” she says. “It was horrible. The dreams would come with a real, physical pain.”

Elizabeth’s suffering was intensified by the inability of anyone, including herself, to recognize the problem for what it was. It took  years of groping in the dark, until, at the age of forty-one, another doctor was able to scratch the word narcolepsy into her file.

Narcolepsy is, admittedly, not always an easy diagnosis to make and not one to reach in a hurry. One of the tools in diagnosis is the multiple sleep latency test (MSLT), which needs to be performed in a sleep lab during the day. In this test, the patient is wired up to an electroencephalograph (EEG) and asked to fall asleep, a number of times, during a period in which the person would otherwise be up and about. The test can see how readily the patient falls asleep, a phenomenon that is called sleep latency, a simple but effective measure of sleepiness and hence an important clue to sleep deprivation. The EEG can also see if REM sleep jumps the queue and pushes its way into the initial stages of sleep, causing no end of trouble.
But the MSLT is fallible, partly because it takes place in such odd conditions for sleep. It is possible to do well on the test and still have narcolepsy. It is equally possible to have early-onset REM and not have narcolepsy.

Current thinking is inclined to attribute narcolepsy to low levels of hormones called orexins (also called hypocretins), which are produced by a part of the brain called the hypothalamus, an amazing little gadget that is a bit like the brain’s brain. It regulates sleep, appetite, and body temperature, three aspects of our lives that are closely related. Disturb one and the chances are you will disturb the other. Orexin levels are often measured by a spinal tap, an intrusive procedure that can have slight risks of its own. Certain drugs that can help with narcolepsy, such as modafinil, have been found to stir the orexins. But Elizabeth uses large amounts of Ritalin and has long argued that the maximum recommended doses of this drug are nowhere near sufficient for her needs. “If I don’t take enough,” she says, “I may as well not take any.”

Ritalin is not a substance to be toyed with, and Elizabeth is well aware that there is a list of drugs, including some antidepressants, with which it has a dysfunctional relationship. Nevertheless, although Ritalin is often used to keep people awake, for her it means she can get into the depths of sleep without having to wade through shoals of hallucination. At the end of the day, she finds it is important she gets to bed before the effects of the drug have worn off. Otherwise, she can end up spending several hours in the kitchen, pottering around with no idea what she is doing or why. This is called automatic behavior, a form of waking sleepfulness that can beset anybody but is part of a range of sleeping disorders, especially narcolepsy.

At this stage, only further medication will get Elizabeth to bed. She needs to wake up enough in order to sleep. By now, though, at least she knows how to get there — and self-doubt no longer plagues her as intensely as her nightmares.

Adapted from Michael McGirr’s SNOOZE: The Lost Art of Sleep.

The Sleep Disorder That Makes People Dream Their Own Death