Narcolepsy
Encyclopedia
Narcolepsy is a chronic
Chronic (medicine)
A chronic disease is a disease or other human health condition that is persistent or long-lasting in nature. The term chronic is usually applied when the course of the disease lasts for more than three months. Common chronic diseases include asthma, cancer, diabetes and HIV/AIDS.In medicine, the...

 sleep disorder
Sleep disorder
A sleep disorder, or somnipathy, is a medical disorder of the sleep patterns of a person or animal. Some sleep disorders are serious enough to interfere with normal physical, mental and emotional functioning...

, or dyssomnia
Dyssomnia
Dyssomnias are a broad classification of sleeping disorders that make it difficult to get to sleep, or to remain sleeping.Dyssomnias are primary disorders of initiating or maintaining sleep or of excessive sleepiness and are characterized by a disturbance in the amount, quality, or timing of...

, characterized by excessive sleepiness and sleep attacks at inappropriate times, such as while at work. People with narcolepsy often experience disturbed nocturnal sleep and an abnormal daytime sleep pattern, which often is confused with insomnia
Insomnia
Insomnia is most often defined by an individual's report of sleeping difficulties. While the term is sometimes used in sleep literature to describe a disorder demonstrated by polysomnographic evidence of disturbed sleep, insomnia is often defined as a positive response to either of two questions:...

. Narcoleptics, when falling asleep, generally experience the REM stage of sleep within 10 minutes; whereas most people do not experience REM sleep until an hour or so later.

Another one of the many problems that some narcoleptics experience is cataplexy
Cataplexy
Cataplexy is a sudden and transient episode of loss of muscle tone, often triggered by emotions. It is a rare disease , but affects roughly 70% of people who have narcolepsy...

, a sudden muscular weakness brought on by strong emotions (though many people experience cataplexy without having an emotional trigger). It often manifests as muscular weaknesses ranging from a barely perceptible slackening of the facial muscles to the dropping of the jaw or head, weakness at the knees, or a total collapse. Usually speech is slurred and vision is impaired (double vision, inability to focus), but hearing and awareness remain normal. In some rare cases, an individual's body becomes paralyzed and muscles become stiff.

Narcolepsy is a neurological sleep disorder. It is not caused by mental illness or psychological problems. It is most likely affected by a number of genetic abnormalities that affect specific biologic factors in the brain, combined with an environmental trigger during the brain's development, such as a virus.

The term narcolepsy derives from the French word narcolepsie created by the French physician Jean-Baptiste-Édouard Gélineau
Jean-Baptiste-Édouard Gélineau
Jean-Baptiste-Édouard Gélineau was the French physician who first described narcolepsy....

 by combining the Greek
Greek language
Greek is an independent branch of the Indo-European family of languages. Native to the southern Balkans, it has the longest documented history of any Indo-European language, spanning 34 centuries of written records. Its writing system has been the Greek alphabet for the majority of its history;...

 νάρκη (narkē, "numbness" or "stupor"), and λῆψις (lepsis), "attack" or "seizure".

Signs and symptoms

The main characteristic of narcolepsy is Excessive Daytime Sleepiness
Excessive daytime sleepiness
Excessive daytime sleepiness is characterized by persistent sleepiness, and often a general lack of energy, even after apparently adequate night time sleep...

 (EDS), even after adequate night time sleep. A person with narcolepsy is likely to become drowsy or fall asleep or just be very tired throughout the day, often at inappropriate times and places. Daytime naps may occur with little warning and may be physically irresistible. These naps can occur several times a day. They are typically refreshing, but only for a few hours. Drowsiness may persist for prolonged periods of time. In addition, nighttime sleep may be fragmented with frequent awakenings.

The classic symptoms of the disorder, often referred to as the "tetrad of narcolepsy," are cataplexy
Cataplexy
Cataplexy is a sudden and transient episode of loss of muscle tone, often triggered by emotions. It is a rare disease , but affects roughly 70% of people who have narcolepsy...

, sleep paralysis
Sleep paralysis
Sleep paralysis is paralysis associated with sleep that may occur in healthy persons or may be associated with narcolepsy, cataplexy, and hypnagogic hallucinations. The pathophysiology of this condition is closely related to the normal hypotonia that occurs during REM sleep. When considered to be a...

, hypnagogic hallucinations, and excessive daytime sleepiness. Other symptoms include automatic behavior
Automatic behavior
Automatic behavior, from the Greek automatos or self-acting, is the spontaneous production of often purposeless verbal or motor behavior without conscious self-control or self-censorship...

s. These symptoms may not occur in all patients. Cataplexy is an episodic condition featuring loss of muscle function, ranging from slight weakness (such as limpness at the neck or knees, sagging facial muscles, or inability to speak clearly) to complete body collapse. Episodes may be triggered by sudden emotional reactions such as laughter
Laughter
Laughing is a reaction to certain stimuli, fundamentally stress, which serves as an emotional balancing mechanism. Traditionally, it is considered a visual expression of happiness, or an inward feeling of joy. It may ensue from hearing a joke, being tickled, or other stimuli...

, anger
Anger
Anger is an automatic response to ill treatment. It is the way a person indicates he or she will not tolerate certain types of behaviour. It is a feedback mechanism in which an unpleasant stimulus is met with an unpleasant response....

, surprise, or fear
Fear
Fear is a distressing negative sensation induced by a perceived threat. It is a basic survival mechanism occurring in response to a specific stimulus, such as pain or the threat of danger...

, and may last from a few seconds to several minutes. The person remains conscious throughout the episode. In some cases, cataplexy may resemble epileptic seizures. Sleep paralysis is the temporary inability to talk or move when waking (or less often, when falling asleep). It may last a few seconds to minutes. This is often frightening but is not dangerous. Hypnagogic hallucinations are vivid, often frightening, dreamlike experiences that occur while dozing, falling asleep and/or while awakening.

Automatic behavior means that a person continues to function (talking, putting things away, etc.) during sleep episodes, but awakens with no memory of performing such activities. It is estimated that up to 40 percent of people with narcolepsy experience automatic behavior during sleep episodes. Sleep paralysis and hypnagogic hallucinations also occur in people who do not have narcolepsy, but more frequently in people who are suffering from extreme lack of sleep. Cataplexy is generally considered to be unique to narcolepsy and is analogous to sleep paralysis in that the usually protective paralysis mechanism occurring during sleep is inappropriately activated. The opposite of this situation (failure to activate this protective paralysis) occurs in rapid eye movement behavior disorder
Rapid eye movement behavior disorder
Rapid eye movement sleep behaviour disorder is a sleep disorder that involves abnormal behaviour during the sleep phase with rapid eye movement . It was first described in 1986....

.

In most cases, the first symptom of narcolepsy to appear is excessive and overwhelming daytime sleepiness. The other symptoms may begin alone or in combination months or years after the onset of the daytime naps. There are wide variations in the development, severity, and order of appearance of cataplexy, sleep paralysis, and hypnagogic hallucinations in individuals. Only about 20 to 25 percent of people with narcolepsy experience all four symptoms. The excessive daytime sleepiness generally persists throughout life, but sleep paralysis and hypnagogic hallucinations may not.

Although these are the common symptoms of narcolepsy, many people with narcolepsy also suffer from insomnia
Insomnia
Insomnia is most often defined by an individual's report of sleeping difficulties. While the term is sometimes used in sleep literature to describe a disorder demonstrated by polysomnographic evidence of disturbed sleep, insomnia is often defined as a positive response to either of two questions:...

 for extended periods of time. The symptoms of narcolepsy, especially the excessive daytime sleepiness and cataplexy, often become severe enough to cause serious problems in a person's social, personal, and professional life. Normally, when an individual is awake, brain wave
Brain wave
* Brain Wave, a science fiction novel by Poul Anderson* a neural oscillation* a layman term for the electric fields measured by Electroencephalography...

s show a regular rhythm. When a person first falls asleep, the brain waves become slower and less regular. This sleep state is called non-rapid eye movement (NREM
NREM
Non-rapid eye movement, or NREM is, collectively, sleep stages 1 – 3, previously known as stages 1 – 4. Rapid eye movement sleep is not included. There are distinct electroencephalographic and other characteristics seen in each stage. Unlike REM sleep, there is usually little or no eye movement...

) sleep. After about an hour and a half of NREM sleep, the brain waves begin to show a more active pattern again. This sleep state, called REM sleep (rapid eye movement sleep), is when most remembered dream
Dream
Dreams are successions of images, ideas, emotions, and sensations that occur involuntarily in the mind during certain stages of sleep. The content and purpose of dreams are not definitively understood, though they have been a topic of scientific speculation, philosophical intrigue and religious...

ing occurs. Associated with the EEG-observed waves during REM sleep, muscle atonia is present (called REM atonia).

In narcolepsy, the order and length of NREM and REM sleep periods are disturbed, with REM sleep occurring at sleep onset instead of after a period of NREM sleep. Thus, narcolepsy is a disorder in which REM sleep appears at an abnormal time. Also, some of the aspects of REM sleep that normally occur only during sleep—lack of muscular control, sleep paralysis, and vivid dreams—occur at other times in people with narcolepsy. For example, the lack of muscular control can occur during wakefulness in a cataplexy episode; it is said that there is intrusion of REM atonia during wakefulness. Sleep paralysis and vivid dreams can occur while falling asleep or waking up. Simply put, the brain does not pass through the normal stages of dozing and deep sleep but goes directly into (and out of) rapid eye movement (REM) sleep.

This has several consequences. Night time sleep does not include as much deep sleep, so the brain tries to "catch up" during the day, hence EDS. People with narcolepsy may visibly fall asleep at unpredicted moments (such motions as head bobbing are common). People with narcolepsy fall quickly into what appears to be very deep sleep, and they wake up suddenly and can be disoriented when they do (dizziness is a common occurrence). They have very vivid dreams, which they often remember in great detail. People with narcolepsy may dream even when they only fall asleep for a few seconds.

Causes

Although the cause of narcolepsy was not determined for many years after its discovery, scientists had discovered conditions that seemed to be associated with an increase in an individual's risk of having the disorder. Specifically, there appeared to be a strong link between narcoleptic individuals and certain genetic conditions. One factor that seemed to predispose an individual to narcolepsy involved an area of Chromosome 6 known as the HLA
Human leukocyte antigen
The human leukocyte antigen system is the name of the major histocompatibility complex in humans. The super locus contains a large number of genes related to immune system function in humans. This group of genes resides on chromosome 6, and encodes cell-surface antigen-presenting proteins and...

 complex. There appeared to be a correlation between narcoleptic individuals and certain variations in HLA genes, although it was not required for the condition to occur. Certain variations in the HLA complex were thought to increase the risk of an auto-immune response to protein
Protein
Proteins are biochemical compounds consisting of one or more polypeptides typically folded into a globular or fibrous form, facilitating a biological function. A polypeptide is a single linear polymer chain of amino acids bonded together by peptide bonds between the carboxyl and amino groups of...

-producing neurons in the brain. The protein produced, called hypocretin or orexin
Orexin
Orexins, also called hypocretins, are the common names given to a pair of excitatory neuropeptide hormones that were simultaneously discovered by two groups of researchers in rat brains....

, is responsible for controlling appetite and sleep patterns. Individuals with narcolepsy often have reduced numbers of these protein-producing neurons in their brains. In 2009 the autoimmune hypothesis was supported by research carried out at Stanford University School of Medicine.

The neural control of normal sleep states and the relationship to narcolepsy are only partially understood. In humans, narcoleptic sleep is characterized by a tendency to go abruptly from a waking state to REM sleep with little or no intervening non-REM sleep. The changes in the motor and proprioceptive systems during REM sleep have been studied in both human and animal models. During normal REM sleep, spinal and brainstem alpha motor neuron
Alpha motor neuron
Alpha motor neurons are large lower motor neurons of the brainstem and spinal cord. They innervate extrafusal muscle fibers of skeletal muscle and are directly responsible for initiating their contraction...

 depolarization
Depolarization
In biology, depolarization is a change in a cell's membrane potential, making it more positive, or less negative. In neurons and some other cells, a large enough depolarization may result in an action potential...

 produces almost complete atonia of skeletal muscle
Skeletal muscle
Skeletal muscle is a form of striated muscle tissue existing under control of the somatic nervous system- i.e. it is voluntarily controlled. It is one of three major muscle types, the others being cardiac and smooth muscle...

s via an inhibitory descending reticulospinal pathway. Acetylcholine
Acetylcholine
The chemical compound acetylcholine is a neurotransmitter in both the peripheral nervous system and central nervous system in many organisms including humans...

 may be one of the neurotransmitter
Neurotransmitter
Neurotransmitters are endogenous chemicals that transmit signals from a neuron to a target cell across a synapse. Neurotransmitters are packaged into synaptic vesicles clustered beneath the membrane on the presynaptic side of a synapse, and are released into the synaptic cleft, where they bind to...

s involved in this pathway. In narcolepsy, the reflex inhibition of the motor system seen in cataplexy is believed to be identical to that seen in normal REM sleep.

In 2004 researchers in Australia induced narcolepsy-like symptoms in mice by injecting them with antibodies from narcoleptic humans. The research has been published in the Lancet providing strong evidence suggesting that some cases of narcolepsy might be caused by autoimmune disease. Narcolepsy is strongly associated with HLA-DQB1
HLA-DQB1
Major histocompatibility complex, class II, DQ beta 1, also known as HLA-DQB1, is a human gene and also denotes the genetic locus that contains this gene...

*0602 genotype
Genotype
The genotype is the genetic makeup of a cell, an organism, or an individual usually with reference to a specific character under consideration...

. There is also an association with HLA-DR2
HLA-DR2
HLA-DR2 of the HLA-DR serotype system, is a broad antigen serotype that is now preferentially covered by HLA-DR15 and HLA-DR16 serotype group...

 and HLA-DQ1
HLA-DQ1
HLA-DQ1 is a serotype that covers a broad range of HLA-DQ haplotypes. Historically it was identified as a DR-like alpha chain called DC1, later, it was among 3 types DQw1 , DQw2 and DQw3. Of these three serotyping specificities only DQw1 recognized DQ alpha chain. The serotype is positive in...

. This may represent linkage disequilibrium
Linkage disequilibrium
In population genetics, linkage disequilibrium is the non-random association of alleles at two or more loci, not necessarily on the same chromosome. It is also referred to as to as gametic phase disequilibrium , or simply gametic disequilibrium...

. Despite the experimental evidence in human narcolepsy that there may be an inherited basis for at least some forms of narcolepsy, the mode of inheritance remains unknown. Some cases are associated with genetic diseases such as Niemann-Pick disease
Niemann-Pick disease
Niemann–Pick disease refers to a group of fatal inherited metabolic disorders that are included in the larger family of lysosomal storage diseases .-Signs and symptoms:Symptoms are related to the organs in which they accumulate...

 or Prader-Willi syndrome
Prader-Willi syndrome
Prader–Willi syndrome is a rare genetic disorder in which seven genes on chromosome 15 are deleted or unexpressed on the paternal chromosome...

.

Currently a link between GlaxoSmithKline
GlaxoSmithKline
GlaxoSmithKline plc is a global pharmaceutical, biologics, vaccines and consumer healthcare company headquartered in London, United Kingdom...

's swine flu vaccine Pandemrix
Pandemrix
Pandemrix is an influenza vaccine for influenza pandemics, such as the H1N1 2009 flu pandemic colloquially called the swine flu. The vaccine was developed by GlaxoSmithKline and patented in September 2006....

 and childhood narcolepsy is being investigated due to increased prevalence of narcolepsy in Finnish and Swedish children after vaccinations. Finland's National Institute of Health and Welfare is recommending that Pandemrix vaccinations are suspended pending further investigation into 15 reported cases of recently vaccinated children developing narcolepsy.

In Finland in mid-November 2010, 37 cases of children's narcolepsy had been reported as suspected adverse events of Pandemrix. This can be compared to the normal average of 3 cases of children's narcolepsy per year.

Diagnosis

Diagnosis is relatively easy when all the symptoms of narcolepsy are present, but if the sleep attacks are isolated and cataplexy is mild or absent, diagnosis is more difficult. It is also possible for cataplexy to occur in isolation. Two tests that are commonly used in diagnosing narcolepsy are the polysomnogram and the multiple sleep latency test
Multiple Sleep Latency Test
The Multiple Sleep Latency Test is a sleep disorder diagnostic tool. It is used to measure the time elapsed from the start of a daytime nap period to the first signs of sleep, called sleep latency...

 (MSLT). These tests are usually performed by a sleep specialist
Sleep medicine
Sleep medicine is a medical specialty or subspecialty devoted to the diagnosis and therapy of sleep disturbances and disorders. From the middle of the 20th century, research has provided increasing knowledge and answered many questions about sleep-wake functioning. The rapidly evolving field has...

. The polysomnogram involves continuous recording of sleep brain waves and a number of nerve and muscle functions during nighttime sleep. When tested, people with narcolepsy fall asleep rapidly, enter REM sleep early, and may awaken often during the night. The polysomnogram also helps to detect other possible sleep disorders that could cause daytime sleepiness.

For the multiple sleep latency test, a person is given a chance to sleep every 2 hours during normal wake times. Observations are made of the time taken to reach various stages of sleep (sleep onset latency
Sleep onset latency
In sleep science, sleep onset latency is the length of time that it takes to accomplish the transition from full wakefulness to sleep, normally to the lightest of the non-REM sleep stages.-Sleep latency studies:...

). This test measures the degree of daytime sleepiness and also detects how soon REM sleep begins. Again, people with narcolepsy fall asleep rapidly and enter REM sleep early.

Treatment

Treatment is tailored to the individual, based on symptoms and therapeutic response. The time required to achieve optimal control of symptoms is highly variable, and may take several months or longer. Medication adjustments are also frequently necessary, and complete control of symptoms is seldom possible. While oral medications are the mainstay of formal narcolepsy treatment, lifestyle changes are also important.

The main treatment of excessive daytime sleepiness in narcolepsy is central nervous system
Central nervous system
The central nervous system is the part of the nervous system that integrates the information that it receives from, and coordinates the activity of, all parts of the bodies of bilaterian animals—that is, all multicellular animals except sponges and radially symmetric animals such as jellyfish...

 stimulants such as methylphenidate
Methylphenidate
Methylphenidate is a psychostimulant drug approved for treatment of attention-deficit hyperactivity disorder, postural orthostatic tachycardia syndrome and narcolepsy. It may also be prescribed for off-label use in treatment-resistant cases of lethargy, depression, neural insult and obesity...

, amphetamine
Amphetamine
Amphetamine or amfetamine is a psychostimulant drug of the phenethylamine class which produces increased wakefulness and focus in association with decreased fatigue and appetite.Brand names of medications that contain, or metabolize into, amphetamine include Adderall, Dexedrine, Dextrostat,...

, methamphetamine
Methamphetamine
Methamphetamine is a psychostimulant of the phenethylamine and amphetamine class of psychoactive drugs...

, modafinil
Modafinil
Modafinil is an analeptic drug manufactured by Cephalon, and is approved by the U.S. Food and Drug Administration for the treatment of narcolepsy, shift work sleep disorder, and excessive daytime sleepiness associated with obstructive sleep apnea...

 (Provigil), a new stimulant with a different pharmacologic mechanism, and/or armodafinil
Armodafinil
Armodafinil is a stimulant-like drug produced by the pharmaceutical company Cephalon Inc., which was approved by the FDA on June 15, 2007...

 (Nuvigil). In Fall 2007 an alert for severe adverse skin reactions to modafinil was issued by the FDA. Other medications used are codeine
Codeine
Codeine or 3-methylmorphine is an opiate used for its analgesic, antitussive, and antidiarrheal properties...

 and selegiline
Selegiline
Selegiline is a drug used for the treatment of early-stage Parkinson's disease, depression and senile dementia. In normal clinical doses it is a selective irreversible MAO-B inhibitor, however in larger doses it loses its specificity and also inhibits MAO-A...

. Another drug that is used is atomoxetine (Strattera), a non-stimulant and norepinephrine reuptake inhibitor
Norepinephrine reuptake inhibitor
A norepinephrine reuptake inhibitor or adrenergic reuptake inhibitor , is a type of drug which acts as a reuptake inhibitor for the neurotransmitters norepinephrine and epinephrine by blocking the action of the norepinephrine transporter...

 (NRI), that has little or no abuse potential. In many cases, planned regular short naps can reduce the need for pharmacological treatment of the EDS to a low or non-existent level.

Cataplexy and other REM-sleep symptoms are frequently treated with tricyclic antidepressant
Tricyclic antidepressant
Tricyclic antidepressants are heterocyclic chemical compounds used primarily as antidepressants. The TCAs were first discovered in the early 1950s and were subsequently introduced later in the decade; they are named after their chemical structure, which contains three rings of atoms...

s such as clomipramine
Clomipramine
Clomipramine is a tricyclic antidepressant . It was developed in the 1960s by the Swiss drug manufacturer Geigy and has been in clinical use worldwide ever since.- Indications :...

, imipramine
Imipramine
Imipramine , also known as melipramine, is an antidepressant medication, a tricyclic antidepressant of the dibenzazepine group...

, or protriptyline
Protriptyline
Protriptyline is a tricyclic antidepressant , specifically a secondary amine, indicated for the treatment of depression and ADHD...

, as well as other drugs that suppress REM sleep. Venlafaxine (branded as Effexor XR by Wyeth Pharmaceuticals), an antidepressant which blocks the reuptake of serotonin and norepinephrine, has shown usefulness in managing symptoms of cataplexy, however, it has notable side-effects including sleep disruption.

Another treatment option for narcolepsy is Xyrem
Xyrem
Xyrem is a prescription medication manufactured by Jazz Pharmaceuticals, and is approved by the U.S. Food and Drug Administration for the treatment of cataplexy associated with narcolepsy and Excessive Daytime Sleepiness associated with narcolepsy...

 (sodium oxybate) oral solution. Xyrem
Xyrem
Xyrem is a prescription medication manufactured by Jazz Pharmaceuticals, and is approved by the U.S. Food and Drug Administration for the treatment of cataplexy associated with narcolepsy and Excessive Daytime Sleepiness associated with narcolepsy...

 is a prescription medication manufactured by Jazz Pharmaceuticals, and is approved by the U.S. Food and Drug Administration (FDA) for the treatment of cataplexy
Cataplexy
Cataplexy is a sudden and transient episode of loss of muscle tone, often triggered by emotions. It is a rare disease , but affects roughly 70% of people who have narcolepsy...

 associated with narcolepsy and Excessive Daytime Sleepiness
Excessive daytime sleepiness
Excessive daytime sleepiness is characterized by persistent sleepiness, and often a general lack of energy, even after apparently adequate night time sleep...

 (EDS) associated with narcolepsy. The American Academy of Sleep Medicine
American Academy of Sleep Medicine
The American Academy of Sleep Medicine is a United States professional society for the medical subspecialty of sleep medicine. It was established in 1975. The AASM is the only professional society that is dedicated exclusively to the medical subspecialty of sleep medicine...

 (AASM) recently recognized Xyrem as a standard of care for the treatment of cataplexy, daytime sleepiness, and disrupted sleep due to narcolepsy in its Practice Parameters for the Treatment of Narcolepsy and other Hypersomnias of Central Origin. These recommendations are based upon careful review of the medical literature, and the designation “standard” of care “reflects a high degree of clinical certainty” based on strong empirical evidence..

Using stimulants to mask daytime sleepiness does not address the actual cause of the problem. Stimulants may provide some assistance with daytime activity, but the underlying cause will remain and potentially worsen over time due to the stimulant itself becoming an obstruction to delta wave sleep periods. Lifestyle changes involving reduced stress, more exercise (especially for overweight persons experiencing EDS caused by sleep apnea and snoring) and less stimulant intake (such as coffee and nicotine) are likely to be ideal forms of assistive treatment. Some people with narcolepsy have a nocturnal body clock and are helped by selecting an occupation that properly coincides with their body's natural sleep cycle (such as sleeping in the day and working at night). This allows sufferers to avoid the need to force themselves into the more common 9 to 5 schedule that their body is unable to maintain, and avoids the need to take stimulants to remain active during the times when their bodies are inclined to rest.

In addition to drug therapy, an important part of treatment is scheduling short naps (10 to 15 minutes) two to three times per day to help control excessive daytime sleepiness and help the person stay as alert as possible. Daytime naps are not a replacement for nighttime sleep, especially if a person's body is natively inclined towards a nocturnal life cycle. Ongoing communication between the health care provider, patient, and the patient's family members is important for optimal management of narcolepsy.

Finally, a recent study reported that transplantation of hypocretin neurons into the pontine reticular formation in rats is feasible, indicating the development of alternative therapeutic strategies in addition to pharmacological interventions.

Epidemiology

It is estimated that as many as 3 million people worldwide are affected by narcolepsy. In the United States
United States
The United States of America is a federal constitutional republic comprising fifty states and a federal district...

, it is estimated that this condition afflicts as many as 200,000 Americans, but fewer than 50,000 are diagnosed. It is as widespread as Parkinson's disease
Parkinson's disease
Parkinson's disease is a degenerative disorder of the central nervous system...

 or multiple sclerosis
Multiple sclerosis
Multiple sclerosis is an inflammatory disease in which the fatty myelin sheaths around the axons of the brain and spinal cord are damaged, leading to demyelination and scarring as well as a broad spectrum of signs and symptoms...

 and more prevalent than cystic fibrosis
Cystic fibrosis
Cystic fibrosis is a recessive genetic disease affecting most critically the lungs, and also the pancreas, liver, and intestine...

, but it is less well known. Narcolepsy is often mistaken for depression
Clinical depression
Major depressive disorder is a mental disorder characterized by an all-encompassing low mood accompanied by low self-esteem, and by loss of interest or pleasure in normally enjoyable activities...

, epilepsy
Epilepsy
Epilepsy is a common chronic neurological disorder characterized by seizures. These seizures are transient signs and/or symptoms of abnormal, excessive or hypersynchronous neuronal activity in the brain.About 50 million people worldwide have epilepsy, and nearly two out of every three new cases...

, or the side effects
Adverse effect (medicine)
In medicine, an adverse effect is a harmful and undesired effect resulting from a medication or other intervention such as surgery.An adverse effect may be termed a "side effect", when judged to be secondary to a main or therapeutic effect. If it results from an unsuitable or incorrect dosage or...

 of medications. It can also be mistaken for poor sleeping habits, recreational drug use, or laziness. Narcolepsy can occur in both men and women at any age, although its symptoms are usually first noticed in teenagers or young adults. There is strong evidence that narcolepsy may run in families; 8 to 12 percent of people with narcolepsy have a close relative with this neurologic disorder.

Narcolepsy has its typical onset in adolescence
Adolescence
Adolescence is a transitional stage of physical and mental human development generally occurring between puberty and legal adulthood , but largely characterized as beginning and ending with the teenage stage...

 and young adulthood. There is an average 15-year delay between onset and correct diagnosis
Diagnosis
Diagnosis is the identification of the nature and cause of anything. Diagnosis is used in many different disciplines with variations in the use of logics, analytics, and experience to determine the cause and effect relationships...

 which may contribute substantially to the disabling features of the disorder. Cognitive, educational, occupational, and psychosocial problems associated with the excessive daytime sleepiness of narcolepsy have been documented. For these to occur in the crucial teen years when education, development of self-image, and development of occupational choice are taking place is especially devastating. While cognitive impairment does occur, it may only be a reflection of the excessive daytime somnolence.

The prevalence of narcolepsy is about 1 per 2,000 persons. It is a reason for patient visits to sleep disorder centers, and with its onset in adolescence, it is also a major cause of learning difficulty and absenteeism from school. Normal teenagers often already experience excessive daytime sleepiness because of a maturational increase in physiological sleep tendency accentuated by multiple educational and social pressures; this may be disabling with the addition of narcolepsy symptoms in susceptible teenagers. In clinical practice, the differentiation between narcolepsy and other conditions characterized by excessive somnolence may be difficult. Treatment options are currently limited. There is a paucity in the literature of controlled double-blind
Double-blind
A blind or blinded experiment is a scientific experiment where some of the people involved are prevented from knowing certain information that might lead to conscious or subconscious bias on their part, invalidating the results....

 studies of possible effective drugs or other forms of therapy. Mechanisms of action of some of the few available therapeutic agents have been explored but detailed studies of mechanisms of action are needed before new classes of therapeutic agents can be developed. Narcolepsy is an underdiagnosed condition in the general population. This is partly because its severity varies from obvious to barely noticeable. Some people with narcolepsy do not suffer from loss of muscle control. Others may only feel sleepy in the evenings.

Famous people with narcolepsy

  • Arthur Lowe
    Arthur Lowe
    Arthur Lowe was a BAFTA Award winning English actor. He was best known for playing Captain George Mainwaring in the popular British sitcom Dad's Army from 1968 until 1977.-Early life:...

    , actor, as stated in documentaries and books about Dad's Army and this biography
  • Jimmy Kimmel
    Jimmy Kimmel
    James Christian "Jimmy" Kimmel is an American television host and comedian. He is the host of Jimmy Kimmel Live!, a late-night talk show that airs on ABC. Prior to that, Kimmel was best known as the co-host of Comedy Central's The Man Show and Win Ben Stein's Money...

    , TV host
  • Nicole Jeray, professional golfer
  • Brian Ferneyhough
    Brian Ferneyhough
    Brian John Peter Ferneyhough is an English composer. His music is characterized by the extensive use of complex rhythmic tuplet notation which features in all his works...

    , composer
  • George Church
    George Church
    George Church is an American molecular geneticist. He is currently Professor of Genetics at Harvard Medical School, Professor of Health Sciences and Technology at Harvard and MIT, and a core faculty member at the Wyss Institute for Biologically Inspired Engineering at Harvard University.With...

    , molecular geneticist
  • Tom Chasuk, author, "The Bean Gourmet"

Society and culture

Depictions of the disorder in fiction and pop culture can range greatly in the accuracy of how they portray the symptoms. Narcolepsy is often depicted in an exaggerated fashion in comedy films or TV shows. In the movie and book Fight Club
Fight Club (novel)
Fight Club is a 1996 novel by Chuck Palahniuk. It follows the experiences of an unnamed protagonist struggling with insomnia. Inspired by his doctor's exasperated remark that insomnia is not suffering, he finds relief by impersonating a seriously ill person in several support groups...

, the main character Tyler Durden is a narcoleptic and insomniac. Also in the movie Rat Race
Rat Race (film)
Rat Race is a 2001 ensemble cast comedy film directed by Jerry Zucker, written by Andy Breckman, and stars several comedians including Rowan Atkinson, Whoopi Goldberg, Cuba Gooding, Jr., Jon Lovitz, Lanei Chapman, Seth Green, Kathy Najimy, Dave Thomas, Vince Vieluf, John Cleese, Breckin Meyer,...

, one of the main characters (Enrico Pollini, played by Rowan Atkinson
Rowan Atkinson
Rowan Sebastian Atkinson is a British actor, comedian, and screenwriter. He is most famous for his work on the satirical sketch comedy show Not The Nine O'Clock News, and the sitcoms Blackadder, Mr. Bean and The Thin Blue Line...

) has narcolepsy.

The Little Sleep, a detective novel by Paul Tremblay
Paul G. Tremblay
Paul G. Tremblay is an American author and editor of contemporary horror, dark fantasy, and science fiction. Tremblay received two Bram Stoker Award nominations in 2007 and he is a juror for the Shirley Jackson Awards.- Biography :...

, portrayed the main character, Mark G., as having narcolepsy. The character deals with daily, even hourly frustrations due to the general cognitive fog, not being able to trust his own memory and even not being able to drive. Mark shares the frustration felt by many with narcolepsy, that his disease is not taken seriously and that he is "the punch line in a joke".

In Gus Van Sant
Gus Van Sant
Gus Green Van Sant, Jr. is an American director, screenwriter, painter, photographer, musician, and author. He is a two time nominee of the Academy Award for Best Director for his 1997 film Good Will Hunting and his 2008 film Milk, both of which were also nominated for Best Picture, and won the...

's independent, art house drama
Art film
An art film is the result of filmmaking which is typically a serious, independent film aimed at a niche market rather than a mass market audience...

 My Own Private Idaho
My Own Private Idaho
My Own Private Idaho is a 1991 independent drama film written and directed by Gus Van Sant, loosely based on Shakespeare's Henry IV, Part 1, Henry IV, Part 2, and Henry V, and starring River Phoenix and Keanu Reeves...

(1991), the lead character, Mike Waters (played by River Phoenix
River Phoenix
River Jude Phoenix was an American film actor, musician, and teen icon. He was the oldest brother of fellow actors Rain, Joaquin, Liberty, and Summer Phoenix.Phoenix began acting at age 10 in television commercials...

), has narcolepsy. In the opening scene, a dictionary definition of the condition is presented on screen. Phoenix's character's narcoleptic episodes are used as a transition tool; transporting the characters from place to place throughout the course of the film and are interpreted with dream-like sequences of time lapsed skies, barns falling from the air and salmon swimming upstream. The character's seizures are brought on by stressful situations, which come frequently as he is a male hustler by profession.

In Hill Street Blues
Hill Street Blues
Hill Street Blues is an American serial police drama that was first aired on NBC in 1981 and ran for 146 episodes on primetime into 1987. Chronicling the lives of the staff of a single police precinct in an unnamed American city, the show received critical acclaim and its production innovations ...

, one of the minor recurring characters was Vic Hitler, the "narcoleptic comedian". He would suddenly fall asleep at inopportune moments, including while performing on stage.

In Homestuck on mspaintadventures.com
MS Paint Adventures
MS Paint Adventures is a collection of webcomics written and illustrated by Andrew Hussie. Because of the frequency with which the site is updated, MS Paint Adventures is estimated to be the longest web comic on the Internet with over 6000 pages total among its four series.These comics are written...

, one of the main characters, Jade Harley, has narcolepsy. While sleeping, however, she can use a robot to interact with the world, albeit in a dream state.

In The Sopranos
The Sopranos
The Sopranos is an American television drama series created by David Chase that revolves around the New Jersey-based Italian-American mobster Tony Soprano and the difficulties he faces as he tries to balance the often conflicting requirements of his home life and the criminal organization he heads...

, Janice Soprano
Janice Soprano
Janice Soprano Baccalieri, played by Aida Turturro, is a fictional character on the HBO TV series The Sopranos. She is Tony Soprano's elder sister. A young Janice has appeared in flashbacks, played by Madeline Blue and Juliet Fox.-Biography:...

's Christian boyfriend, Aaron Arkaway, suffers from narcolepsy and falls asleep at inappropriate times.

In "One Piece
One Piece
is a Japanese shōnen manga series written and illustrated by Eiichiro Oda. It has been serialized in Weekly Shōnen Jump since August 4, 1997; the individual chapters are being published in tankōbon volumes by Shueisha, with the first released on December 24, 1997, and the 64th volume released as...

", the main character Monkey D. Luffy suffers from narcolepsy, as does his grandfather Garp and brother Ace.

In "Galactik Football
Galactik Football
Galactik Football is a French animated television series, co-produced by Alphanim, France 2, Jetix Europe and Welkin-Animation. It started airing its 3rd 26-episode season in territories around Europe in June 2010....

", Ahito, the goalkeeper of the Snow Kids, has narcolepsy.

Lead singer Ewan Apple of "Honor Among Thieves" is rumored to suffer from narcolepsy.

See also

  • Caffeine-induced sleep disorder
    Caffeine-induced sleep disorder
    Caffeine-induced sleep disorder is a psychiatric disorder that results from overconsumption of the stimulant caffeine. "When caffeine is consumed immediately before bedtime or continuously throughout the day, sleep onset may be delayed, total sleep time reduced, normal stages of sleep altered, and...

  • Cataplexy
    Cataplexy
    Cataplexy is a sudden and transient episode of loss of muscle tone, often triggered by emotions. It is a rare disease , but affects roughly 70% of people who have narcolepsy...

  • Dyssomnia
    Dyssomnia
    Dyssomnias are a broad classification of sleeping disorders that make it difficult to get to sleep, or to remain sleeping.Dyssomnias are primary disorders of initiating or maintaining sleep or of excessive sleepiness and are characterized by a disturbance in the amount, quality, or timing of...

  • Orexin
    Orexin
    Orexins, also called hypocretins, are the common names given to a pair of excitatory neuropeptide hormones that were simultaneously discovered by two groups of researchers in rat brains....

  • Microsleep
    Microsleep
    A microsleep is an episode of sleep which may last for a fraction of a second or up to thirty seconds. Often, it is the result of sleep deprivation, mental fatigue, depression, sleep apnea, hypoxia, narcolepsy, or hypersomnia...

  • List of people with narcolepsy
  • Sleep medicine
    Sleep medicine
    Sleep medicine is a medical specialty or subspecialty devoted to the diagnosis and therapy of sleep disturbances and disorders. From the middle of the 20th century, research has provided increasing knowledge and answered many questions about sleep-wake functioning. The rapidly evolving field has...

  • Sleep inertia
    Sleep inertia
    Sleep inertia is a physiological state characterised by a decline in motor dexterity and a subjective feeling of grogginess immediately following an abrupt awakening. The impaired alertness may interfere with the ability to perform mental or physical tasks...


External links

The source of this article is wikipedia, the free encyclopedia.  The text of this article is licensed under the GFDL.
 
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