People who suffer from the sleep disorder narcolepsy typically have a skewed understanding of the condition. It’s characterized by extreme daily sleepiness that lasts for days at a time, hindering performance in school, work, and social contexts and increasing the likelihood of major accidents and injuries.
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 when 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 awake and sleep results in the predominant symptom of the disorder, drowsiness throughout the day (EDS).
Rapid eye movement (REM) sleep occurs at the end of a normal night’s sleep, typically an hour or more after going to bed. Narcolepsy is characterized by an abnormal sleep pattern in which rapid eye movement (REM) sleep occurs at an abnormally early time (often, several minutes after falling asleep).
People with narcolepsy often experience rapid eye movement (REM) sleep because of brain abnormalities that alter typical sleep patterns. Symptoms of narcolepsy, such as excessive daytime sleepiness, are also brought on by these disturbances.
There are two subtypes 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
Cataplexy, characterized by an abrupt decrease in muscular tone, has been linked to NT1. “Narcolepsy with cataplexy” was the previous name for NT1.
Patients diagnosed with NT1 do not necessarily suffer from cataplexy. Low levels of hypocretin-1, a substance in the body that regulates alertness, are also diagnostic of NT1.
In many persons with low hypocretin-1 levels, cataplexy develops over time even if it is not apparent at diagnosis.
Narcolepsy Type 2
This condition was once classified as “narcolepsy without cataplexy.” Symptomatically, those with NT2 are quite close to those with NT1, although they do not experience cataplexy or hypocretin-1 deficiency.
Those initially diagnosed with NT2 can have their condition downgraded to NT1 if they show signs of cataplexy or have abnormally low levels of hypocretin-1. It is estimated that around 10% of cases have this shift in diagnosis.
What Causes Narcolepsy Symptoms?
Loss of brain cells that produce orexins, a kind of neurotransmitter, has been linked to narcolepsy symptoms. Two of orexins’ various functions are to reinforce awake and prevent rapid eye movement (REM) sleep2. The ability to sleep well at night and wake up feeling refreshed is known as “wakefulness consolidation,” and it’s crucial for staying awake for lengthy stretches of the day.
Maintaining healthy sleep-wake patterns requires a sufficient number of orexin-producing neurons, which the body lacks. There is less of a “recovery” period during sleep, thus people need to nap more frequently during the day. People with narcolepsy frequently have an overwhelming need to sleep at inconvenient times.
When someone has narcolepsy, their body goes through rapid changes between sleep and wakefulness. When compared to people without narcolepsy, those who suffer from it have a significantly faster sleep cycle overall and an even quicker transition into REM sleep. Patients with narcolepsy experience frequent nighttime awakenings accompanied by symptoms including cataplexy, sleep paralysis, and hallucinations because they are stuck in aberrant intermediate states between sleep and wakefulness.
The loss of orexin-producing neurons has been linked to narcolepsy, however this is not the case for everyone with the disorder. However, only type 1 narcoleptics are known to experience symptoms due to a lack of orexin, while type 2 narcoleptics are not thought to have a deficiency in this neurotransmitter.
Type 1 narcoleptics have 85–95% fewer neurons3 that generate orexins compared to healthy controls. Type 2 narcoleptics typically have normal orexin levels and milder narcolepsy symptoms. Patients with type 2 narcolepsy have mysterious symptoms, the origins of which are not well understood.
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. While daytime sleepiness is a universal symptom of narcolepsy, the other symptoms are far less common. Only about 10–15% of narcoleptics report having all four symptoms4.
Excessive Daytime Sleepiness
The earliest sign of narcolepsy is typically EDS, or excessive daytime drowsiness. Even if a person with narcolepsy wakes up feeling refreshed, they may quickly fall asleep again. It doesn’t matter how much sleep you get at night; feeling sleepy during the day is a real thing, and it tends to get worse when you’re doing things like watching TV or sitting in a classroom all day. Intense mental activity may increase a person’s sense of alertness.
Patients with narcolepsy frequently report experiencing both excessive daytime drowsiness and sudden episodes of sleepiness they refer to as “sleep attacks.” Sleep attacks are characterized by sudden onsets of acute drowsiness and an almost overwhelming need to sleep. Naps can last anything from a few seconds to several minutes6, and can happen at any time. When they emerge from their power naps, they are usually energized and ready to take on the day.
People with narcolepsy may engage in actions during attentional lapses or brief periods of sleep of which they are not aware and of which they have little memory afterward. It is possible to fall asleep while engaging in a routine activity like eating, conversing, or typing. During these brief sleep attacks, their performance typically drops, with incomprehensible scribbling being a common example.
Temporary paralysis of the muscles occurs during sleep paralysis, which can occur either as one wakes up from sleep or as one falls asleep. In sleep paralysis, a person’s muscles become stiff and they are unable to move or talk, yet they are otherwise alert. 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 narcolepsy7.
REM sleep typically occurs between 60 and 90 minutes after falling asleep8 in healthy, non-narcoleptic adults. REM sleep is characterized by heightened brain activity and the experience of more realistic dreams. Atonia, or momentary paralysis of muscles, also occurs during REM sleep. Atonia, the inability to act out one’s dreams while asleep, typically diminishes upon waking.
It is common for people with narcolepsy to experience REM sleep within 15 minutes of falling asleep, and REM sleep phenomena may even creep into non-REM states of consciousness. Sleep paralysis occurs when atonia continues after waking. Some people may suffer hallucinations if the type of dreaming typical of REM sleep occurs while they are awake.
Some persons with narcolepsy report terrifying hallucinations. These hallucinations typically arise just as a person is drifting off to sleep, but they can also occur upon awakening. Although most hallucinations are visual, such as seeing a person or thing in the bedroom, they can also be multimodal, including more than one sense at a time.
About a third of narcoleptics9 have hypnagogic hallucinations. Hallucinations, like sleep paralysis, are thought to be an intrusion of REM sleep phenomena into waking life.
Cataplexy is characterized by an abrupt loss of muscular tone that occurs while the victim is conscious. In contrast to illnesses like fainting and seizure disorders, patients with cataplexy retain full awareness throughout the episode. Strong emotions10, such as laughing, surprise, rage, and enthusiasm, have been linked to cataplexy. Cataplexy is a symptom exclusive to patients who have narcolepsy type 1.
Sleep paralysis is a paralysis of muscle action that happens during REM sleep and is related to cataplexy. While sleep paralysis typically occurs at the threshold between sleep and wakefulness, cataplexy can strike at any time after awakening.
In mild cases of cataplexy, only a few muscle groups, such the eyelids, may become weak. Cataplexy is characterized by a sudden and complete lack of voluntary muscle control, which can linger for several minutes in more severe cases. These episodes can be terrifying, but they are usually harmless so long as the person is in a safe place.
Disrupted Nighttime Sleep
New research reveals that narcoleptics frequently experience evening sleep disruptions in addition to the traditional tetrad of narcolepsy symptoms long recognized by medical professionals. 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.
Despite having severely altered sleep cycles, many persons with narcolepsy nonetheless sleep the same number of hours per night as those who do not suffer from the illness. Individuals with narcolepsy tend to accumulate their sleep time via fragmented naps throughout the day and night, rather than from one long stretch of sleep at night like most people.
Narcolepsy Symptoms in Children
Excessive daytime drowsiness is a common presenting symptom of narcolepsy in young people. In addition to the return of daytime naps common in infants and toddlers, this heightened weariness may cause people to sleep for longer than usual (a condition known as hypersomnia).
Long sleep durations may improve as narcolepsy progresses because of the greater nocturnal sleep disruption caused by more vivid dreams and more frequent evening awakenings. In spite of 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 various illnesses. 12. Narcoleptics are at a higher risk for developing cardiovascular and metabolic disorders.
These symptoms tend to be more prevalent among narcoleptics, which may be due to the many functions orexins serve in the body. Loss of orexin-producing neurons has been linked to sleep disruption, weight gain, high blood pressure at night, and atherosclerosis, all of which increase the risk of cardiovascular disease.
ADHD, anxiety, eating disorders, depression, and schizophrenia are only some of the psychiatric illnesses that have been linked to narcolepsy13. Researchers speculate that the impairment and changes in lifestyle brought on by narcolepsy symptoms may contribute to the onset of psychiatric illnesses, or that the etiology of narcolepsy and psychiatric diseases are comparable. More study is required to understand the complex interaction between narcolepsy and psychiatric diseases, despite the fact that its influence on mental health is severe, with up to 57% of patients with narcolepsy also having depression.
What Are the Effects of Narcolepsy?
Negative effects of narcolepsy symptoms on a person’s health and well-being are not to be underestimated. Sleep paralysis, sleepiness, and cataplexy pose a serious risk to one’s life in potentially hazardous situations like driving or operating heavy machinery. Those who suffer from narcolepsy have an increased risk of automobile accidents of threefold to fourfold, according to one study11.
There is evidence that narcolepsy can cause problems in the academic and professional spheres. Poor performance can be attributed to sleepiness and lapses in focus, which may be misunderstood as behavioral issues in youngsters.
The social isolation experienced by many people with narcolepsy is a direct result of the shame they feel about their disease. Without help, this can exacerbate mental health issues and have far-reaching consequences for a person’s academic performance, professional prospects, and interpersonal connections.
Obesity, cardiovascular concerns like hypertension, and psychological difficulties like depression, anxiety, and ADD/ADHD are all more common among those with narcolepsy (ADHD).
Careful evaluation by a physician well-versed in narcolepsy is necessary for a correct diagnosis. Narcolepsy can be misdiagnosed for a long time because of its rarity and the fact that its symptoms can easily be attributable to other conditions.
Review of symptoms and medical history is the first step in making a diagnosis. To better diagnose the patient’s EDS, the doctor needs to learn about the patient’s sleeping habits. 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.
Sleep and EDS could be tested for. The patient’s own impression of their symptoms serves as the basis for the Epworth Sleepiness Scale (ESS). Depending on the situation, a thorough examination called polysomnography (PSG), in which sensors track brain and body activity, may be required. A sleep clinic is where you go to spend the night for this kind of investigation.
The Multiple Sleep Latency Test (MSLT) can be used to objectively quantify drowsiness the day following the PSG test. At five distinct times during the MSLT, the patient will be asked to try to fall asleep while still linked to the PSG’s sensors. 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. Hypocretin deficiency is characteristic of NT1 and serves as a diagnostic marker distinguishing it from NT2.
Diagnostic Criteria for Narcolepsy
When diagnosing sleep disorders, doctors use a set of established criteria. The correct diagnosis and distinction of NT1, NT2, hypersomnias, and other disorders that induce EDS is aided by the use of standardized criteria.
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 CSF. A patient with NT2 must also show similar results on the MSLT, but they cannot have cataplexy or low levels of hypocretin.
Diagnosing NT2 can be challenging because its symptoms are similar to those of other sleep disorders. Therefore, the doctor must thoroughly examine the patient’s test results and symptoms to rule out alternative diagnoses. Short, restorative naps and overnight sleep disruptions are diagnostic of NT2, but not narcolepsy.
Type 1 and type 2 narcolepsy are incurable. Improving patient safety, decreasing symptoms, and raising quality of life are all targets of narcolepsy treatment.
A large percentage of persons with narcolepsy experience no significant change in their condition over time. Some patients may have a reduction in their symptoms as they age18, and in extremely rare situations, a complete remission of their symptoms may occur spontaneously19. Why the sickness manifests in different ways for different people is a mystery that has thus far eluded researchers.
The treatments for NT1 and NT2 are very similar, with the exception that there is no possibility of medicine for cataplexy in NT2.
Symptoms can be considerably mitigated with a mix of medicinal and behavioral interventions, but they cannot be eliminated entirely. In most cases, a percentage of EDS symptoms will still be present even after treatment has been administered. Medical professionals are better suited to assess a patient’s needs and develop a treatment plan that addresses those needs.
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.
- Nap time budgeting: Narcoleptics benefit from short naps, so scheduling in time for them during the day can help reduce daytime sleepiness. Nap time may need to be accommodated in the classroom or in the office.
- People with narcolepsy can benefit from practicing proper sleep hygiene in order to improve their nightly sleep. Consistent bedtime and wake times, a quiet, undisturbed bedroom, and limited screen usage in the hours before bed all contribute to good sleep hygiene.
- If you suffer from narcolepsy, it’s best to avoid substances like alcohol and sedatives, which can make daytime sleepiness worse.
- People with narcolepsy should discuss safe driving practices with their doctors. Avoiding long or boring rides and getting some sleep before getting behind the wheel are two ways to boost safety.
- Eating well: Because of the increased likelihood of obesity associated with narcolepsy, eating healthfully is crucial to the wellbeing of those with the disorder.
- Being physically active has been shown to reduce the risk of obesity and may also lead to better sleep at night.
- Seeking help from mental health specialists and support groups can help persons with narcolepsy maintain their mental health and reduce their vulnerability to the negative effects of social isolation, despair, and anxiety.
Most persons with narcolepsy also need medicine to help control at least one symptom, despite the fact that behavioral treatments are often successful.
Medication for narcolepsy typically results in reduced symptoms, however some people may experience unwanted negative effects. These medications are available only with a doctor’s prescription and should be taken cautiously and in accordance with the recommendations of your healthcare provider and pharmacy.
Narcolepsy medications include, but are not limited to,
- These two wakefulness-promoting medications, modafinil and armodafinil, are chemically identical and are often used as initial treatment for EDS.
- Methylphenidate, an amphetamine, has been shown to alleviate symptoms of EDS.
- Solriamfetol, a medication approved by the FDA this year, has shown effects in EDS that are equivalent to modafinil21.
- This drug may take weeks to treat EDS22, but it can help with cataplexy, EDS, and sleep difficulties at night.
- Pitolisant, a wakefulness-promoting medicine that has also been found to have a favorable effect on cataplexy, was approved by the FDA in 2019.
Some people may have more severe adverse drug reactions or problems with drug interactions when using a given prescription. If you and your doctor work together, you can find the right drug and dose with little risk.
Narcolepsy Treatment and Children
While narcolepsy treatment for children is comparable to that for adults, more care may need to be given while deciding on drugs and dosages. The American Academy of Pediatrics23 advises having a child’s heart checked before beginning stimulant 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 medication24 before becoming pregnant or breastfeeding. In order to safely manage symptoms after stopping medication, it may be necessary to make adjustments to one’s current pattern of behavior or to other aspects of one’s lifestyle.
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