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Sleepiness, Daytime / Narcolepsy

Definition :
Narcolepsy is a chronic sleep disorder characterized by overwhelming daytime drowsiness and sudden attacks of sleep.

People with narcolepsy often find it difficult to stay awake for long periods of time, regardless of the circumstances. Narcolepsy can cause serious disruptions in your personal and professional lives.

Some people mistake narcolepsy for depression, seizure disorder, fainting, simple lack of sleep, or other conditions that may cause abnormal sleep patterns.

Narcolepsy is a chronic condition that doesn't go away completely. Although there's no cure for narcolepsy, medications and lifestyle changes can help you manage the symptoms. And talking to others — family, friends, employer, teachers — can help you cope better with narcolepsy.

Research continues to determine the cause of narcolepsy. Genetics and some sort of trigger may affect brain chemicals and contribute to the disorder. Only about 2 percent of people with narcolepsy have a close relative with the disease, which suggests that factors other than genetics are the cause of most narcolepsy.

Normal sleep pattern vs. narcolepsy
The normal process of falling asleep begins with a phase called non-rapid eye movement (NREM) sleep. During this phase, your brain waves slow down considerably. After an hour or two of NREM sleep, your brain activity picks up again, and REM sleep begins. Most dreaming occurs during REM sleep.

In narcolepsy, however, you suddenly fall into REM sleep without first experiencing NREM sleep and at abnormal times, such as during the day. Also, some of the aspects of sleep that normally occur only during REM sleep, such as sudden lack of muscle tone, sleep paralysis and vivid dreams, occur at other times during sleep in people with narcolepsy.

The role of brain chemicals
Scientists believe that people with narcolepsy who begin to feel drowsy and then drop instantly into "dream sleep" may have imbalances in certain brain chemicals important in regulating sleep. One example is hypocretin, a sleep-regulating chemical that plays an important role in arousing you from sleep and keeping you awake.

Hypocretin is found in low levels in people with narcolepsy. These low levels of hypocretin may even lead to the development of a diagnostic test for narcolepsy because hypocretin is normally present in spinal fluid of people without the disorder, but often isn't found in the spinal fluid of people with narcolepsy.

Research continues to focus on whether an abnormal gene may be responsible for narcolepsy or if the body's immune system may be involved in mistakenly attacking hypocretin-producing cells in the brain.

Risk Factor :
Narcolepsy affects males and females equally and occurs in all racial and ethnic groups. However, rates do seem to vary by country.

In the United States, about one in 2,000 people is affected by narcolepsy, while in Israel only one in 500,000 people has the disorder. Japan has the highest rate, affecting about one in 600 people.

The condition may run in families. A small percentage of people with narcolepsy have a close relative with the disease.

When to seek medical advice :
See your doctor if you experience excessive daytime sleepiness that seriously disrupts your personal or professional life.

Symptoms :
The signs and symptoms of narcolepsy include :

  • Excessive daytime sleepiness. The primary characteristic of narcolepsy is overwhelming drowsiness and an uncontrollable need to sleep during the day. People with narcolepsy fall asleep without warning, anywhere and at any time. For example, you may suddenly nod off while at work or talking with friends. You may sleep for just a few minutes or up to a half-hour before awakening and feeling refreshed, but then you fall asleep again.

    In addition to sleeping at inappropriate times and places, you also may experience decreased alertness throughout the day. Excessive daytime sleepiness usually is the first symptom to appear and is often the most troublesome, making it difficult for you to concentrate and function fully.

  • Sudden loss of muscle tone. This condition, called cataplexy, can cause a range of physical changes, from slurred speech to complete weakness of most muscles, and may last for a few seconds to a few minutes. Cataplexy is uncontrollable and is often triggered by intense emotions, usually positive ones such as such as laughter or excitement, but sometimes fear, surprise or anger. For example, your head may droop uncontrollably or your knees may suddenly buckle when you laugh.

    Some people with narcolepsy experience only one or two episodes of cataplexy a year, while others have numerous episodes each day. About 70 percent of people with narcolepsy experience cataplexy.

  • Sleep paralysis. People with narcolepsy often experience a temporary inability to move or speak while falling asleep or upon waking. These episodes are usually brief — usually lasting only several seconds to several minutes — but they can be frightening. You may be aware of the condition and have no difficulty recalling it afterward, even if you had no control over what was happening to you.

    This sleep paralysis mimics the type of temporary paralysis that normally occurs during rapid eye movement (REM) sleep, the period of sleep during which most dreaming occurs. This temporary immobility during REM sleep may prevent your body from acting out dream activity. Not everyone with sleep paralysis has narcolepsy, however. Many normal people experience a few attacks of sleep paralysis, especially in young adulthood.

  • Hallucinations. These hallucinations, called hypnagogic hallucinations, may take place when a person with narcolepsy falls quickly into REM sleep, as they do at sleep onset at night and periodically during the day. Because you may be semiawake when you begin dreaming, you experience your dreams as reality, and they may be particularly vivid and frightening.

Other characteristics
Other signs and symptoms of narcolepsy include restless nighttime sleep and occasional automatic behavior. During episodes of automatic behavior, you continue to function during sleep episodes — even talking and putting things away, for example — but you awaken with no memory of performing such activities, and you generally don't perform these tasks well. As many as 40 percent of people with narcolepsy experience automatic behavior during sleep attacks. People with narcolepsy may also act out their dreams at night by flailing their arms or kicking and screaming.

Usually, the signs and symptoms of narcolepsy first develop between the ages of 10 and 25, but the condition can start before the age of 10 or in your 20s and 30s. It's rare for narcolepsy to begin after age 40. Narcolepsy is chronic, which means signs and symptoms may vary in severity, but they never go away entirely.

Your doctor may make a preliminary diagnosis of narcolepsy based on your experience of both excessive daytime sleepiness and sudden loss of muscle tone (cataplexy). After an initial diagnosis, your doctor may refer you to a sleep specialist for additional studies and evaluation.

Formal diagnosis may require staying overnight at a sleep center where you undergo an in-depth analysis of your sleep by a team of specialists. Methods of diagnosing narcolepsy and determining its severity include :

  • Sleep questionnaire. The Epworth Sleepiness Scale uses a series of short questions to diagnose narcolepsy. You'll rank on a numbered scale whether certain situations, such as sitting down after lunch, make you sleepy and, if so, how sleepy.
  • Polysomnogram. This test involves a variety of measuring tactics conducted through electrodes placed on your scalp before you fall asleep. For this test, you must stay overnight for observation at a medical facility. The test measures the electrical activity of your brain (electroencephalogram) and heart (electrocardiogram), and the movement of your muscles (electromyogram) and eyes (electro-oculogram).
  • Multiple sleep latency test. This method measures how long it takes for you to fall asleep during the day. You'll be asked to fall asleep for a series of four or five naps, each nap two hours apart. Specialists will observe your sleep patterns. People who have narcolepsy fall asleep easily and enter into rapid eye movement (REM) sleep quickly.

These tests also can help doctors rule out other possible causes of your signs and symptoms. Other sleep disorders, such as sleep apnea, can cause excessive daytime sleepiness.

Complications :
Narcolepsy may cause you to experience serious problems in both the professional and personal parts of your life. Others may perceive your undiagnosed condition as lazy, lethargic or rude. Your performance may suffer at school or work.

Narcolepsy can affect intimate relationships. Extreme sleepiness may cause low sex drive or impotence, and people with narcolepsy may even fall asleep while making love. The problems caused by sexual dysfunction may be further complicated by emotional difficulties. Intense feelings, such as anger or joy, can trigger some signs of narcolepsy, causing affected people to withdraw from emotional interactions.

Sleep attacks may result in physical harm to people with narcolepsy. You're at increased risk of a car accident if you have an attack while driving. Your risk of cuts and burns is higher if you fall asleep while preparing food.

Narcolepsy has no cure, but medications and lifestyle modifications can help you manage the symptoms. Medications include :

  • Stimulants. Drugs that stimulate the central nervous system are the primary treatment to help people with narcolepsy stay awake during the day. Modafinil (Provigil), a newer stimulant, isn't as addictive and doesn't produce the highs and lows often associated with older stimulants. Some people need treatment with methylphenidate (Ritalin) or various amphetamines. Although these medications are effective, they may cause side effects, such as nervousness and heart palpitations, and can be addictive.
  • Antidepressants. Doctors often prescribe antidepressant medications, which suppress REM sleep, to help alleviate the symptoms of cataplexy, hypnagogic hallucinations and sleep paralysis. These medications include the tricyclic antidepressants protriptyline (Vivactil) and imipramine (Tofranil).
  • Sodium oxybate (Xyrem). This medication controls cataplexy, sleep paralysis and hallucinations in people with narcolepsy. Sodium oxybate helps to improve nighttime sleep, which is often poor in narcolepsy. In high doses it may also help control daytime sleepiness, even though you take it only at night. However, because the use of this drug has been associated with serious side effects, such as trouble breathing during sleep, sleepwalking and bed-wetting, it's strictly regulated by the Food and Drug Administration.

If you have other health problems, such as high blood pressure or diabetes, ask your doctor how medications for existing conditions may interact with those taken for narcolepsy.

Certain over-the-counter drugs, such as allergy and cold medications, can cause drowsiness as a side effect. If you have narcolepsy, your doctor will likely recommend that you avoid taking these medications.

Medications to treat narcolepsy can help reduce your signs and symptoms, but they can't alleviate them entirely. Lifestyle changes also are an integral part of treating narcolepsy.

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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