Misconceptions abound about the sleep disorder narcolepsy. The condition is characterized by excessive daytime sleepiness that can lead to negative consequences in academic, occupational, and social settings as well as an increased risk of accidental death.
Hundreds of thousands of children and adults in the United States suffer with narcolepsy, making it one of the most common neurological illnesses.
Patients and their loved ones can be more equipped to deal with the effects of narcolepsy if they have a firm grasp on the disorder’s classifications, symptoms, causes, diagnosis, and treatment options.
What Is Narcolepsy?
Disorders like narcolepsy cause problems with the body’s natural sleep-wake cycle. The inability of the brain to control wakefulness and sleep1 is the major cause of the condition’s principal symptom, excessive daytime sleepiness (EDS).
Rapid eye movement (REM) sleep is the final stage of normal sleep, typically occurring an hour or more after falling asleep. The onset of rapid eye movement (REM) sleep is abnormally early in people with narcolepsy; it can occur as soon as a few minutes after falling asleep.
People with narcolepsy often experience rapid eye movement (REM) sleep because of brain abnormalities that alter typical sleep patterns. Daytime sleepiness and other narcolepsy symptoms are also brought on by these interruptions.
There are two distinct forms of narcolepsy recognized by the International Classification of Sleep Disorders, Third Edition2 (ICSD-3): narcolepsy type 1 (NT1) and narcolepsy type 2. (NT2).
Narcolepsy Type 1
The condition known as “cataplexy,” in which a person suddenly loses muscle tone, has been linked to NT1. A previous name for NT1 was “narcolepsy with cataplexy.”
Patients diagnosed with NT1 may or may not have cataplexy. Low levels of hypocretin-1, a substance in the body that regulates alertness, can also be indicative of NT1.
Many persons with hypocretin-1 deficiency experience cataplexy3 at some point after their diagnosis, even if it wasn’t present at the time of evaluation.
Narcolepsy Type 2
Previous labels for NT2 included “narcolepsy without cataplexy” and “narcolepsy without hypersomnia.” However, NT2 patients do not experience cataplexy or have abnormally low levels of hypocretin-1, despite sharing many of the same symptoms as NT1 patients.
Cataplexy and/or low hypocretin-1 levels allow a previously diagnosed NT2 individual to be reclassified as NT1. In roughly 10% of cases, doctors re-evaluate their initial diagnosis.
What Causes Narcolepsy Symptoms?
Loss of brain cells that produce orexins, a kind of neurotransmitter, has been linked to the onset of narcolepsy symptoms. One of the various functions of orexins is to prevent rapid eye movement (REM) sleep from occurring, which can lead to sleepiness. As a result of this consolidation, people are able to stay awake for extended periods of time during the day.
Orexin-producing neurons are crucial for regular sleep and wake times. Reduced sleep consolidation results in daytime fatigue and the need for more frequent napping. People with narcolepsy often have an overwhelming need to sleep at the most inconvenient moments.
Because of narcolepsy, the body cycles through sleep and wakefulness very quickly. People with narcolepsy have a substantially quicker sleep onset and REM sleep transition compared to the general population. Narcoleptic patients experience frequent nighttime awakenings and a variety of symptoms associated with being in an aberrant intermediate state between sleep and wakefulness, such as cataplexy, sleep paralysis, and hallucinations.
The loss of orexin-producing neurons has been linked to narcolepsy, however this is not the case for everyone with the disorder. Type 1 narcolepsy is caused by a lack of orexin, while type 2 narcolepsy is not linked to a lack of this neurotransmitter.
The number of orexin-producing neurons3 is reduced by 85%-95% in patients with narcolepsy type 1, compared to those without the disorder. Most people with a diagnosis of narcolepsy type 2 have normal orexin levels and exhibit milder narcolepsy symptoms. It is unclear what triggers symptoms of narcolepsy type 2 in those who suffer from it.
Symptoms of Narcolepsy
Excessive daytime sleepiness, sleep paralysis, hallucinations, and cataplexy are the four main symptoms of narcolepsy, which are often referred to as a tetrad. Excessive daytime sleepiness is a hallmark of narcolepsy, while the other symptoms are more uncommon. Rarely do more than 10%-15% of narcoleptics report all four symptoms.
Excessive Daytime Sleepiness
First and foremost, people with narcolepsy generally experience extreme daytime sleepiness (EDS). Individuals with narcolepsy may feel refreshed upon waking, only to become fatigued again shortly after. No matter how much sleep you get at night, you’ll still feel sleepy during boring or uninteresting activities like watching TV or sitting in class. Focused activity has been shown to increase feelings of alertness.
Patients with narcolepsy typically report experiencing both excessive daytime tiredness and periodic episodes of sleep paralysis known as “sleep attacks.” It’s hard to fight off the overwhelming want to sleep that comes on suddenly during a sleep attack. Short naps can last anywhere from a few seconds to several minutes6, and can happen at any time. After one of these short naps, they may feel revitalized and ready to take on the day.
The symptoms of narcolepsy include the involuntary performance of tasks during moments of distraction or sleep, followed by a lack of recollection of those tasks. This might happen while they are engaged in a routine task, such as eating, chatting, or typing, and they fall asleep while their hands are still moving. During these brief sleep attacks, their performance typically drops, with incomprehensible scribbling being a common example.
Brief immobility upon awakening or slumbering is known as sleep paralysis. During sleep paralysis, the affected person is awake but unable to move or speak. Many people experience paralysis during their sleep, but after waking up, their normal motor and speech functions return. Sleep paralysis occurs in around 25% of patients with narcolepsy.
REM sleep typically begins 60-90 minutes after falling asleep8 in those who do not suffer from narcolepsy. Rapid Eye Movement (REM) sleep is characterized by heightened brain activity and the prevalence of vivid dreams. A transient paralysis of muscle function known as atonia is also present during REM sleep. It is common knowledge that atonia, which inhibits a person from acting out their dreams, subsides upon waking.
Narcoleptics generally experience REM sleep within 15 minutes of falling asleep, and REM sleep phenomena may spill over into awake. Sleep paralysis occurs when a person wakes up yet continues to exhibit atonia. Some people may suffer hallucinations if the type of dreaming typical of REM sleep occurs while they are awake.
Narcoleptic hallucinations are a real danger to those who suffer from narcolepsy. The onset of these hallucinations is most commonly associated with the onset of sleep, however they can also occur upon awakening. Most hallucinations are visual, such as stumbling onto an unknown person in your bedroom, but they can also be multimodal, including more than one sense at a time.
One-third of persons with narcolepsy experience hypnagogic hallucinations, which occur just before the individual is ready to enter a deep sleep state. Similarly to sleep paralysis, which is thought to be an intrusion of REM sleep into consciousness, hallucinations are thought to be a manifestation of REM sleep itself.
Cataplexy is the rapid and unexpected loss of muscular tone that occurs while a person is awake. In contrast to illnesses like fainting and seizure disorders, patients with cataplexy retain full awareness throughout the episode. The intense feelings of joy, shock, rage, or excitement can all set off a cataplexy attack. Cataplexy is a symptom exclusive to patients with narcolepsy type 1.
The paralysis of muscle activity that characterizes sleep paralysis is identical to that which characterizes cataplexy, however it typically manifests itself only during rapid eye movement sleep. Cataplexy, in contrast to sleep paralysis, occurs after a person has completely awakened.
In mild cases of cataplexy, only a few muscle groups, such the eyelids, may become weak. The complete lack of voluntary muscle control that occurs during more severe episodes of cataplexy can linger for many minutes. Although alarming, these experiences are usually harmless so long as the victim is in a secure setting.
Disrupted Nighttime Sleep
While insomnia has long been known to be a symptom of narcolepsy, new evidence reveals that the typical tetrad is not the only problem sufferers of the disorder face. Nighttime sleep disruption, which affects 30–95% of patients11, may be a sign of narcolepsy in and of itself or the result of some other sleep problem. Insomnia, sleep apnea, REM sleep behavior abnormalities, and periodic limb movement disorder are all co-occurring sleep problems in patients with narcolepsy.
Somewhat surprisingly, many people with narcolepsy sleep the same number of hours that persons without this illness do while having severely disordered sleep patterns. People with narcolepsy generally sleep in fragments throughout the day and night, rather than getting a full eight hours of sleep at night like the rest of us.
Narcolepsy Symptoms in Children
Excessive daytime sleepiness is generally the first symptom of narcolepsy in children and teenagers. Due to this heightened fatigue, people may sleep for longer than usual (a condition known as hypersomnia) or return to the frequent daytime naps characteristic of infants and toddlers.
Long sleep durations may improve when narcolepsy worsens because of the increased frequency and intensity of dreaming and awakenings during the night. Despite the fact that narcolepsy is an illness that lasts a person’s entire life, its symptoms do not often worsen with age.
Other Health Conditions Associated with Narcolepsy
Multiple studies have shown that people with narcolepsy have a higher chance of developing a variety of health problems. High blood pressure, high cholesterol, obesity, and diabetes are all illnesses that are more common in people with narcolepsy.
Due to the hormone’s many functions, orexins may be a contributing factor in narcoleptics’ propensity to experience these symptoms. Problems with controlling sleep-wake cycles aren’t the only thing that can be affected by a lack of orexin-producing neurons; overnight blood pressure, weight gain, and plaque formation in the arteries are also on the list of potential heart disease risk factors.
ADHD, anxiety, eating disorders, depression, and schizophrenia are only some of the psychiatric illnesses that have been linked to narcolepsy. Researchers speculate that narcolepsy and psychiatric diseases share same causes, or that the impairment and changes in lifestyle brought on by narcolepsy symptoms contribute to the emergence of psychiatric disorders. Despite the fact that narcolepsy has a profound effect on mental health, with up to 57% of narcoleptics also suffering from depression, more study is required to fully comprehend the intertwined nature of narcolepsy and psychiatric diseases.
What Are the Effects of Narcolepsy?
Negative effects of narcolepsy symptoms on a person’s health and well-being are not to be taken lightly. Sleep paralysis, sleepiness, and cataplexy pose a serious risk to one’s life in potentially hazardous situations like driving or operating heavy machinery. People with narcolepsy are three to four times more likely to get in a car crash, according to studies.
Both academic and occupational performance may be negatively impacted by narcolepsy. In youngsters, especially, drowsiness and lapses in focus may be misunderstood and taken as behavioral issues, which has negative consequences for performance.
Many people with narcolepsy experience social isolation because they are ashamed of having the disorder. Without help, this can exacerbate mental health issues and have far-reaching consequences for a person’s life in all spheres of functioning.
Obesity, cardiovascular disorders like high blood pressure, and psychological difficulties like depression, anxiety, and attention-deficit/hyperactivity disorder (ADHD) are all more common in people with narcolepsy (ADHD).
When seeking a diagnosis for narcolepsy, it’s important to see a doctor who has experience with the condition. Oftentimes, narcolepsy is misdiagnosed for a long time before it is finally identified as the underlying reason of the patient’s sleep problems.
Examining the patient’s symptoms and health background is the first step in making a diagnosis. The doctor can learn more about the patient’s sleep patterns and the extent of their EDS if they take this step. When treating a patient, especially a child, it is common practice to involve the patient’s loved ones in order to provide a fuller picture of the patient’s symptoms.
Examinations of sleep patterns and EDS may be performed. The Epworth Sleepiness Scale (ESS) is a diagnostic tool that relies on the patient’s own impressions of their condition. In some cases, a thorough examination called polysomnography (PSG) is required. This sleep research takes place over the course of a single night in a specialized facility.
The Multiple Sleep Latency Test (MSLT) can be used to objectively quantify drowsiness the day following the PSG test. The MSLT procedure involves the patient attempting to fall asleep at five separate times while still wearing the PSG monitoring devices. A common characteristic of narcoleptic patients undergoing MSLT is an early onset of REM sleep.
Cerebrospinal fluid (CSF) removal and hypocretin concentration analysis is another possible diagnostic procedure. A spinal tap or lumbar puncture is the medical procedure used to achieve this. The difference between NT1 and NT2 can be seen in the absence of hypocretin.
Diagnostic Criteria for Narcolepsy
Sleep disorders are diagnosed by doctors using a set of accepted criteria. Correctly diagnosing NT1, NT2, hypersomnias, and other diseases that can lead to EDS is facilitated by using a standardized approach.
Both NT1 and NT2 criteria call for severe EDS that has persisted for at least three months. Symptoms of cataplexy and a rapid eye movement (REM) sleep latency on the MSLT are diagnostic of NT1, as are low levels of hypocretin in the cerebrospinal fluid (CSF). Patient MSLT findings must be similar for NT2, although cataplexy or low hypocretin levels are not acceptable.
NT2 can be difficult to identify because its symptoms are similar to those of other sleep disorders. This is why a thorough evaluation of the patient’s symptoms and test results is essential for the doctor to rule out alternative diagnoses. Short, restorative naps and evening sleep disruptions can distinguish narcolepsy from other hypersomnias but aren’t sufficient for a diagnosis of NT2.
Neither type 1 nor type 2 narcolepsy can be cured at this time. Improved patient safety, less symptoms, and increased quality of life are all targets of narcolepsy treatment.
People with narcolepsy often see little to no fluctuation in their condition over time. A patient’s condition may improve with age, and in extremely rare instances, symptoms may disappear altogether. Researchers have not yet pinned down a reason for the disease’s varying manifestations in patients.
Similar approaches can be taken to treating NT1 and NT2, with the exception that NT2 patients will not need to worry about possibly needing to take drugs for cataplexy.
Symptoms can be considerably mitigated with a mix of medicinal and behavioral treatments, but they cannot be eliminated entirely. The severity of EDS typically remains unchanged after treatment. A physician is the only one who can create a treatment plan that is unique to each patient and their condition.
Behavioral Approaches to Treatment
People with narcolepsy can benefit from behavioral methods, a form of therapy that does not involve medication, in a number of ways.
- Budgeting in time for short naps during the day can help minimize EDS because naps of 20 minutes or less are restorative for those with narcolepsy. Nap time may need to be accommodated in the classroom or in the office.
- People with narcolepsy can benefit from adopting healthy sleep practices in order to improve their nightly sleep quality. Maintaining good sleep hygiene entails limiting the use of electronic devices in the hours leading up to night, keeping your sleeping space dark and quiet, and sticking to a regular bedtime and wake-up routine.
- Avoiding sedatives like alcohol is important for people with narcolepsy because it can make their daytime sleepiness much worse.
- A person with narcolepsy should discuss safe driving practices with their doctor. One way to increase safety is to get some sleep before getting behind the wheel. Another is to avoid long, boring rides.
- People with narcolepsy are more likely to become overweight, thus maintaining a healthy weight through proper nutrition is crucial to their well-being.
- Regular exercise has been shown to reduce the risk of obesity and enhance sleep quality.
- Seeking help from mental health specialists and support groups can help someone with narcolepsy avoid the negative effects of isolation, despair, and anxiety on their mental health.
Although behavioral treatments for narcolepsy are often effective, most patients also use medication to manage their condition.
Treatment for narcolepsy typically results in lessening of symptoms, however some drugs have undesirable side effects. These medications are available only with a doctor’s prescription and should be taken cautiously and in accordance with any and all guidelines supplied by your healthcare provider and pharmacist.
Medication for narcolepsy is typically prescribed for:
- The first-line treatment for EDS is the wakefulness-inducing medications modafinil and armodafinil, which are chemically identical.
- Methylphenidate, an amphetamine, can help lessen the symptoms of EDS.
- The FDA finally green-lit solriamfetol in 2019, and it has similar effects on EDS as modafinil21, so it’s a good bet.
- Sodium oxybate is an effective treatment for EDS, cataplexy, and insomnia, while the effects on EDS22 may not be seen for a few weeks.
- Pitolisant, a wakefulness-promoting medicine with a beneficial effect on cataplexy, was approved by the FDA in 2019.
Unfortunately, not all patients benefit from medicine, and some people may have more troublesome reactions to pharmaceuticals than others. By collaborating closely with the physician, the optimal drug and dose can be determined.
Narcolepsy Treatment and Children
While narcolepsy treatment for children is comparable to that for adults, special care may be needed in selecting drugs and determining appropriate dosages. The American Academy of Pediatrics advises having a child’s heart checked before beginning stimulant drug treatment.
Narcolepsy Treatment and Pregnancy
Most medications used to treat narcolepsy have not been adequately studied for their safety in pregnant, trying-to-conceive, or nursing women. Most medical professionals, according to a poll, advise discontinuing narcolepsy treatment before becoming pregnant or breastfeeding. Symptom management after medication withdrawal often necessitates new or altered behavioral strategies.