SUBSCRIBEFAQs
The following statements are for informational purposes only; should not be taken as medical diagnosis, treatment or advice; and are not a substitute for examination and care provided by licensed physicians.
- What is Narcolepsy?
- Is Narcolepsy a psychological or mental disorder?
- What causes narcolepsy?
- Is Narcolepsy Inherited?
- Can Narcolepsy be caused by head injury?
- Does Narcolepsy affect learning?
- How common is Narcolepsy?
- Is Narcolepsy limited to certain groups of people?
- At what age do people get Narcolepsy?
- Can Narcolepsy be cured?
- What are the symptoms of Narcolepsy?
- Is cataplexy dangerous?
- Do I have Narcolepsy?
- How is Narcolepsy diagnosed?
- How is narcolepsy treated?
- What are the long-term problems of narcolepsy?
- What is the Narcolepsy Network?
Question: What is narcolepsy?
Answer: Narcolepsy is a serious and chronic life time neurologic disorder that can affect all areas of a person’s life. Depending on the severity of symptoms, it can tremendously strain relationships with family and friends, affect education and employment, make driving and public outings hazardous, and even turn small daily tasks into major chores.
Question: Is narcolepsy a psychological or mental disorder?
Answer: Narcolepsy is a neurologically-based sleep disorder involving the dissociation of sleep states. Psychological problems can result from the individual’s inability to cope with the symptoms and their family’s misunderstanding of the disorder. It is very difficult for a person with narcolepsy and those around him/her to understand that sleepiness and sleep attacks are uncontrollable. Failure to accept this fact may seriously impact self-esteem and/or personal relationships. Health care counseling for persons with narcolepsy and their families can help alleviate these secondary problems. Educating the public, especially school, health and human resource personnel, can help lessen or even prevent many of these problems.
Question: What causes Narcolepsy?
Answer: Much has been learned since 1998, when researchers discovered that, in most cases of narcolepsy with cataplexy, the two brain chemicals called hypocretins (orexins) are missing. These neurotransmitters are involved in the regulation of the sleep/wake cycle as well as other bodily functions (e.g., blood pressure and metabolism). Further research has shown that the majority of hypocretin-producing cells, located in the hypothalamus, have been destroyed in the brains of those who develop narcolepsy with cataplexy. Scientists have confirmed that narcolepsy with cataplexy is caused by this loss of hypocretins. The cause(s) of narcolepsy without cataplexy are unknown. Further research is needed to determine why hypocretin cells are destroyed and to identify the exact trigger(s) of both forms of narcolepsy.
Question: Is Narcolepsy Inherited?
Answer: In the general population of the U.S. one person out of about 2,000 develops narcolepsy. There appears to be some genetic predisposition to developing narcolepsy with cataplexy, the most common form. About one quarter of the general population in the U.S. carries the HLA-DQB1* o602 genetic marker but only one person out of about 500 of these people will develop this form of narcolepsy.
Question: Can Narcolepsy be caused by head injury?
Answer: There is some evidence that head trauma, an illness or other cause of physiological stress on the brain may also cause a person to develop what is now known as Secondary Narcolepsy. It can have all of the features of Hypocretin deficient Narcolepsy with the possible exception of Cataplexy.
Question: Does narcolepsy affect learning?
Answer: Although narcolepsy does not affect intelligence, learning cannot help but be affected by the symptoms. Study, concentration, memory, and attention span may be periodically impaired by sleep. Children with narcolepsy should be identified at the earliest possible age to prevent a pattern of failure from developing, thus fostering low self-esteem. Adjustments in study habits may be continually necessary. This can best be accomplished with the cooperation of school personnel.
Question: How common is Narcolepsy?
Answer: It is estimated that there are over 200,000 persons with Narcolepsy in the United States only about 25% or 50,000 of them have been diagnosed. On average it takes over seven years from onset of symptoms until a diagnosis is established.
Question: Is Narcolepsy limited to certain groups of people?
Answer: Incidence of Narcolepsy can vary by ethnic group. The highest occurrence is found among the Japanese at one in about 600 and the lowest rate is among Israeli Jews at one in about 500,000.
Narcolepsy affects both men and women equally.
Question: At what age do people get Narcolepsy?
Answer: Although any person can develop Narcolepsy at any age, the typical onset is in the second to third decade (between 10 – 30 years of age) of life.
Question: Can Narcolepsy be cured?
Answer: Currently no cure for Narcolepsy exists, nor any way to replace the missing Hypocretin. Treatment of Narcolepsy aims to relieve the symptoms. The symptoms of Narcolepsy can vary greatly from one person to another, as can the treatments and their effectiveness.
Question:What are the symptoms of Narcolepsy?
Answer: Narcolepsy has five primary symptoms:
- Excessive Daytime Sleepiness (EDS) is present in all cases, but it is also a frequent symptom in many other medical and sleep disorders. Patients often experience EDS in one of three ways: a constant level of drowsiness, sudden sustained episodes of extreme and undeniable sleepiness, and brief moments of sleep called Micro Sleep. For a person without Narcolepsy to feel this effect, she or he would have to stay awake for 48 hours without even a brief nap and then try to function normally. A score of greater than 10 on the Epworth Sleepiness Scale is indicative of symptomatic sleepiness.
- Cataplexy (C) is present in most cases and is exclusive to Narcolepsy. These are episodes of bilateral, partial or total, loss of strength or tone to all or part of a person’s voluntary muscles. It should not be confused with the paralysis of Stroke, Transient Ischemic Attacks or Head Trauma which usually affect only one side of the body and often result in unconsciousness.
Cataplexy can be as subtle as a brief facial tic, or as dramatic as a full body collapse. Episodes are usually triggered by strong emotions such as surprise, anger, humor, love, joy, etc. These events can last brief moment to a few minutes.
During a full body episode of Cataplexy the person may appear to be asleep. In reality they are usually fully awake and aware of everything that is happening around her or him, but the individual is temporarily unable to respond to any stimulus.
Unless a person having Cataplexy is injured, is in a hazardous situation, or collapses into a twisted, uncomfortable or embarrassing posture, no intervention is usually required. - Sleep Paralysis (SP) appears in about half of all cases of Narcolepsy. Like EDS, it can also be present in other medical and sleep disorders. Sleep Paralysis is the inability to speak or control any of the voluntary muscles of the body at the onset of sleep or wakefulness. This may also include the feeling of inability to breathe. Like cataplexy, it is the brain and body’s inability to separate the sleep-wake boundaries that causes attacks. Episodes can last from a brief moment to several minutes. The experience can be very frightening.
- Hypnagogic (at onset of sleep) Hallucinations or Hypnopompic (at onset of wakefulness) Hallucinations (HH) also are found in about half of all people with narcolepsy. They too can be present in other medical and sleep disorders. These vivid, realistic, and often frightening dreams are experienced at the onset of sleep or wakefulness, but the mind believes that the dreams are reality. Episodes often include visual, tactile, kinetic and auditory phenomena as well as the feeling of the presence of someone or something. These events can be particularly terrifying when they occur along with Sleep Paralysis. Imagine being completely terrified by a horrific nightmare while also completely unable to move.
- Disrupted Nighttime Sleep (DNS) is found in many cases of narcolepsy. It is present in other medical and Sleep Disorders. It is the inability to maintain sleep for more than a few hours at a time. While the arousals from sleep may be total (coming full awake with awareness and memory of being awake), they are often partial (without awareness or memory of awakening). This prevents restful, adequate, and satisfying sleep.
Other symptoms reported by people with Narcolepsy can include:
- Automatic Behavior (AB) – The performance of tasks that are often routine, dull or repetitive without conscious effort or memory.
- Memory Lapses – Difficulty in remembering recent events, actions or words
Question: Is cataplexy dangerous?
Answer: Mild cataplexy, while perhaps embarrassing, is not dangerous. One can often find support for weakened head, neck, or arm muscles, so that others may not even be aware of the momentary loss of control. However, severe cataplexy, resulting in immediate and sudden body collapse, may cause injury. Companions should be told in advance what to expect and how to help. They should always check for the person’s safety and comfort, immediately relieving any unnatural bending of limbs or unusual body positions, assuring complete relaxation and then allowing him or her to recover naturally. Cataplexy for some can be so instantaneous that there is no time to prepare for safety, and injury may occur. Obviously, potentially life threatening situations should be avoided unless cataplexy is fully controlled.
Question: Do I have Narcolepsy?
Answer: Because of much misunderstanding and misinformation, many people think that any prolonged Excessive Daytime Sleepiness is Narcolepsy. This is not true. Narcolepsy is a complex medical diagnosis marked by a set of five principal symptoms. (What are the symptoms of Narcolepsy?) There are a large number of medical, behavioral, and Sleep Disorders that have sleepiness as a symptom. Only a physician can diagnose a medical problem.
To help you and your doctor determine if you have Narcolepsy or other Sleep Disorder, print out the applicable questionnaire on the Epworth Sleepiness Scale page, answer the questions and take them with you to your Primary Care Physician.
Question: How is Narcolepsy Diagnosed?
Answer: Narcolepsy can be diagnosed and treated by any physician but because Narcolepsy is a rare disorder and Cataplexy can be difficult to identify, it is best done by a physician who is specially trained, experienced and Board Certified in Sleep Medicine and familiar with Narcolepsy.
There are three ways of diagnosing Narcolepsy: by clinical evaluation of symptoms, by over night Sleep Study and day time nap test and by checking the Cerebrospinal Fluid for the Hypocretin (Orexin) level.
Despite narcolepsy with cataplexy having a high correlation with the HLA-DQB1* o602 marker, genetic testing is not a totally accurate way of determining if a person has Narcolepsy. About one in four people carry this marker but only one in about 500 of them actually have narcolepsy with cataplexy. It is also possible to have Narcolepsy with or without cataplexy and test negative for this marker.
The best place to start is with one’s Primary Care Physician. A person should discuss their sleep issues with their doctor. The doctor should take a thorough history and perform a comprehensive physical examination. The doctor is likely to order tests to rule out a wide variety of disorders that could be the cause of symptoms (Do I have Narcolepsy?”). It is done this way because these symptoms can so often resemble many other medical disorders that are far more dangerous, crippling and even deadly.
By far the easiest and safest and way to diagnose Narcolepsy is by observation and documentation of its symptoms. This only works in cases that have very clear symptoms of Excessive Daytime Sleepiness and Cataplexy (What is Narcolepsy?”). Even when symptoms are clear a sleep study is a good idea to rule out other possible co-existing sleep disorders and often it is required for treatment to be covered by insurance.
If after other disorders are ruled out or appropriately treated your sleep problems persist, a patient may be referred to a Sleep Specialist and/or sent for an overnight Sleep Study called a Polysomnogram (PSG). Follow the instructions provided by the doctor and the Sleep Center closely as many factors can affect sleep and the accuracy of the study. The results will be checked for disorders such as Sleep Apnea, Periodic Limb Movement Disorder, Restless Leg Syndrome, etc. If necessary, the patient will be treated for any of these that are found. Once these sleep disorders are dealt with and the sleepiness continues, the next step is to be sent for another set of Sleep Studies. This time another PSG followed by a daytime Sleep Study called a Multiple Sleep Latency Test (MSLT). Certain Diagnostic Criteria must be met to finally determine if the problem is Narcolepsy.
The latest and least common way to identify Narcolepsy is by testing for the proportion of Hypocretin in the Cerebrospinal Fluid (CSF). It is most commonly used to confirm a diagnosis in event of inconclusive tests. It will not help identify other Sleep Disorders. A doctor will perform a lumbar puncture to draw a sample of CSF from the spine through a needle. This can be a risky procedure with potentially serious complications. The laboratory part of the test is done at very few research centers in the world. There is as yet no way of accurately measuring Hypocretin in blood but there may be in the future.
Question: How is narcolepsy treated?
Answer: Narcolepsy is similar to Type I (also known as Juvenile or Insulin Dependent) Diabetes. In this kind of Diabetes the insulin producing cells in the Pancreas die or stop working. In Narcolepsy the special cells in the brain that produce Hypocretin (Orexin) die. Hypocretin is important for regulating the Sleep/Wake Cycle. Just as there is no cure for Diabetes, there is no cure for Narcolepsy. With Diabetes Insulin can be replaced by injections, but in Narcolepsy Hypocretin can not yet be safely replaced.
Because of this, the only way treat Narcolepsy is to relieve the symptoms. There are two types of symptoms, Sleep/Wake Irregularities and REM Intrusion. There are also two approaches to treatment, Medications and Supportive Therapy. By combining these two approaches and balancing between treatments for Sleepiness and REM Intrusion optimal control of symptoms can be reached.
Narcolepsy is notoriously difficult to treat. It is often best to be under the care of a physician who is specially trained, experienced and Certified in Sleep Medicine. It is also often best for this doctor to also be Board Certified in Neurology and specifically experienced in treating Narcolepsy.
Several months of adjusting medications, dosages, and timing may be needed to arrive at the best treatment. This process also requires good and frequent communication between patient and doctor to assure safety and quality of life. One of the best ways to get accurate and objective information to the doctor is for the patient to keep a Sleep Diary or Journal. This practice can be a simple notebook in which the patient records everything that happens which affects her or his sleep: all sleep and wake times (including naps), quality of sleep, feelings before sleep and upon awakening; medications (including homeopathic and herbal remedies), times, dosages and how the patient feels before and subsequent to taking the dosage, when symptoms are experienced, how long and how strong any symptoms are; food intake including meals and snacks (especially stimulants like caffeine energy drinks and foods, and depressants like alcohol); activities such as work, leisure and exercise; etc. Another way of tracking these factors is to use an online service such as that found at the Med Help website. After several days to a few weeks of recording information, the individual may begin to see patterns emerge. These data are what the doctor needs to know to make important decisions about treatment.
Medications used to relieve sleepiness are all stimulants or wake promoting medications. These include various forms of Amphetamines [Dextroamphetamine (Dexedrine®, Dexedrine SR®), Methamphetamine (Desoxin®), Mixed Amphetamine Salts (Adderall®)]; Methylphenidate (Ritalin®),Armodafinil (Nuvigil ®) and Modafinil (Provigil®). Another medication, Pemolin (Cylert®), which worked well for some people, is no longer available because it could damage the liver and even cause death. Patients may find that some of these work well for them, some not as well, and others not at all. Some medications are quick acting, but their effects are often short lived. Other medications act much slower, but work for longer periods of time. Sometimes, it is necessary for the doctor to prescribe several medicines simultaneously to be taken at several times throughout the day so that the patient can become alert and stay alert for a reasonable length of time.
COMMON MEDICATIONS USED TO CONTROL SLEEPINESS
| Compound Generic Name Brand Names |
Usual Daily Dose |
Drug Class Schedule† |
Pregnancy Risk Category‡ |
Breastfeeding Risk |
Notes & Comments |
| Amphetamine | - | Stimulant, Amphetamine Schedule II | C | Excreted in milk, check with doctor | - |
| Amphetamine & Dextroamphetamine Mixed salts Adderall®, AdderallXR® | 5-60mg | Stimulant, Amphetamine Schedule II | C | Excreted in milk, check with doctor | - |
| ArmodafinilNuvigil ® | 150-250mg | Stimulant, Schedule IV | C | Not known, check with doctor | Similar level of potency to Modafinil but longer acting. |
| Dextroamphetamine Sulfate Dexedrine ® DexedrineSR® | 5-60mg | Stimulant, Amphetamine Schedule II | C | Excreted in milk, check with doctor | Variable duration of action. |
| Methamphetamine Desoxyn® | 5-60mg | Stimulant, Amphetamine Schedule II | C | Excreted in milk, check with doctor | More effective and potent than amphetamine |
| Methylphenidate Ritalin®, RitalinSR® | 10-60mg | Stimulant, Schedule II | C | Not known, check with doctor | The regular formulation is short acting |
| Modafinil Provigil® | 100-400 mg | Stimulant, Schedule IV | C | Not known, check with doctor | Less potent than amphetamines but fewer side effects |
| Pemoline Cylert® (No longer available in the US) | 20-115mg | Stimulant, Schedule IV | B | Not known, check with doctor | Less potent and effective, can cause severe Liver damage, Liver Function tests required every 2 weeks |
† Drug Schedule:
I High abuse potential with no legitimate medical use.
II High abuse and dependency potential.
III Less abuse potential than Schedule II and moderate dependency liability.
IV Less abuse potential than Schedule III and limited dependency liability.
V Limited abuse potential.
‡ Pregnancy Risk Category:
A Adequate studies in pregnant women have failed to show a risk to the fetus.
B Animal studies have not shown a risk to the fetus but controlled studies in pregnant women have not been conducted or Animal studies have shown adverse effects on the fetus but adequate studies in pregnant women have not shown a risk to the fetus.
C Animal studies have shown an adverse effect on the fetus but adequate studies have not been conducted in humans. Benefits from use in pregnant women may be acceptable despite the potential risks.
D The drug may cause risk to the human fetus, but the potential benefit may be acceptable despite the risk.
X Studies in animals or humans shows that the risks clearly out weigh the potential benefits.
Drug therapy for REM Intrusion is aimed at the most serious of these symptoms, Cataplexy, but also works to relieve Hypnagogic and Hypnopompic Hallucinations, Sleep Paralysis and Disrupted Nighttime Sleep. It is commonly treated by the use of Antidepressants which suppresses REM Sleep. There are several classes of Antidepressants: Serotonin Norepinephrine Reuptake Inhibitors like Venlafaxine (EffexorSR®); Norepinephrine Reuptake Inhibitors like Atomoxetine (Strattera®); Selective Serotonin Reuptake Inhibitors like Fluoxetine (Prozac®) and Sertraline (Zoloft®) ; and the older Tricyclic Antidepressants like Protriptyline (Triptil® and Vivactil®), Imipramine (Janimine® and Tofranil®), Desipramine (Norpramine® and Pertofran®), and Clomipramine (Anafranil®).
There is also a newer medication that can help relieve both Sleepiness and REM Intrusion, Sodium Oxibate (Xyrem®). In addition, it will also help with Disrupted Nighttime Sleep. This medicine is unlike any of the others. It is a liquid that is taken in divided doses at bed time and again about four hours later. Also, unlike the others it promotes Stages 3 and 4 Sleep also known as Deep or Consolidated Sleep. It can reduce the need for both alerting and REM Intrusion reducing drugs.
COMMON MEDICATIONS USED TO CONTROL REM SLEEP INTRUSION
| Compound Generic Name Brand Names |
Usual Daily Dose |
Drug Class † | Pregnancy Risk Category‡ |
Breastfeeding Risk |
Notes & Comments |
| Atomoxetine Strattera® | 10-80mg | SNRI Antidepressant | C | Not known, check with doctor | - |
| Clomipramine Anafrinil® | 10-150mg | TCA Antidepressant | C | Excreted in milk, check with doctor | Very effective, used mostly in Europe |
| Desipramine Norpramine®, Pertofane® | 25-100mg | TCA Antidepressant | Not Rated | Not known, check with doctor | - |
| Fluoxetine Prozac® | 20-60mg | SSRI Antidepressant | C | Excreted in milk, not recommended | Well tolerated, high doses often needed |
| Imipramine Janimine®, Tofranil® | 10-100mg | TCA Antidepressant | D | Excreted in milk, check with doctor | - |
| Protriptyline Triptil®, Vivactil® | 5-60mg | TCA Antidepressant | Not Rated | Excreted in milk, not recommended | - |
| Sertraline Zoloft® | 25-200mg | SSRI Antidepressant | C | Excreted in milk, check with doctor | - |
| Venlafaxine Effexor® | 75-225mg | SSNRI Antidepressant | C | - | - |
| Sodium Oxybate Xyrem® | 3-9mg | Hypnotic (sleep inducing) | B | Not known, check with doctor | May also alleviate EDS. |
‡ Pregnancy Risk Category:
A Adequate studies in pregnant women have failed to show a risk to the fetus.
B Animal studies have not shown a risk to the fetus but controlled studies in pregnant women have not been conducted or Animal studies have shown adverse effects on the fetus but adequate studies in pregnant women have not shown a risk to the fetus.
C Animal studies have shown an adverse effect on the fetus but adequate studies have not been conducted in humans. Benefits from use in pregnant women may be acceptable despite the potential risks.
D The drug may cause risk to the human fetus, but the potential benefit may be acceptable despite the risk.
X Studies in animals or humans shows that the risks clearly out weigh the potential benefits.
† Drug Classes:
SNRI Selective Norepinephrine Reuptake Inhibitor
SSNRI Selective Serotonin Norepinephrine Reuptake Inhibitor
SSRI Selective Serotonin Reuptake Inhibitor
TCA Tricyclic Antidepressant
There is continuing research on Narcolepsy, how the sleep/wake cycle works, and new medications. Our bodies and life styles change as we age, and our physiology can adapt to our medicines. Because of these factors, it is likely that it will be necessary to occasionally readjust prescription, dosages, and the times we take them.
Also, like Diabetes it is pointless to simply prescribe a medicine and let the patient do whatever she or he wants without regard to the consequences. In Diabetes, emphasis is placed on Supportive Therapy and counseling about appropriate diet, exercise, and frequent, regular monitoring of the sugar levels in the blood. Not enough emphasis is placed on counseling those with Narcolepsy about the need for regular and sufficient sleep including naps, good Sleep Hygiene, diet that can reduce sleepiness, Light Therapy that can promote alertness, participation in a Support Group where one can share problems and learn solutions and coping mechanisms, and exercise that can energize.
Question: What are the long-term problems of Narcolepsy?
Answer: The consequences of narcolepsy may be many and far-reaching. Cataplexy may interfere with physical activities, and efforts to avoid emotions may lead to social withdrawal. Sleep attacks and cataplexy in public are embarrassing and can cause serious social difficulties. Inability to work and/or drive may result in loss of independence, financial difficulties and various other problems. In these situations, a person can easily lose touch with others and become depressed.
Question: What is the Narcolepsy Network?
Answer: We are a national non-profit corporation, which was founded in 1986. Our members include individuals with narcolepsy, their families and friends, and professionals involved in the study and treatment of narcolepsy.
Our goals are to:
- Serve as a resource center
- Assist support groups
- Educate the public
- Facilitate early diagnosis
- Protect the rights of those with narcolepsy
- Encourage ongoing scientific research.
Our activities include:
- Maintaining a web site with current narcolepsy and hypersomnia information,
- Maintaining Message Boards to promote an active on line narcolepsy and hypersomnia community,
- Publishing a quarterly newsletter, and
- Sponsoring an annual conference about narcolepsy and hypersomnia and their treatments.
Join Narcolepsy Network today and become involved in improving your own life and the quality of life of others like you. Our membership is open to all who share an interest in narcolepsy and hypersomnia and other sleep disorders.
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Date Created: March 2nd, 2008
Last Updated: January 30th, 2012
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