Question 1 of 10

    Narcolepsy (Narcolepsy-Cataplexy)

    1R02 crystallography[1]

    Narcolepsy is a chronic neurological disorder characterized by the classic tetrad of excessive daytime sleepiness, cataplexy, sleep paralysis and hypnagogic hallucinations. The first descriptions of narcolepsy-cataplexy were reported in Germany by Westphal and Fisher in 1877/1878.

    The disorder is related to the following process: endocrine.


    The classic tetrad of narcolepsy consists of excessive daytime sleepiness (EDS), cataplexy, hypnagogic hallucinations, and sleep paralysis. In very few cases it is seen that a child presents with all 4 symptoms [13].

    EDS is the primary symptom of narcolepsy and must be present for at least 3 months to justify the diagnosis. In persons with narcolepsy, severe EDS leads to involuntary somnolence during activities that normally need attention, like driving, eating, or talking. Narcoleptic patient can suffer from severe and constant sleepiness, with bouts of falling asleep without any warning (i.e. sleep attacks).

    Patients with narcolepsy tend to take short and refreshing naps (i.e. rapid eye movement (REM)-type naps) during the day. Sometimes dreams also accompany the daytime naps.

    A significant number of narcolepsy patients have trouble sleeping at night [14]. In addition, patients may have nocturnal compulsive habits like, eating disorder in relation with sleep and smoking at night [15]. Obesity is commonly seen with narcolepsy.

    Cataplexy is a brief and sudden loss of muscle tone and represents REM sleep intrusion during wakefulness. The most characteristic feature of cataplexy is that it usually is triggered by emotions (especially laughter and anger). Cataplexy is seen in about 70% of patients with narcolepsy. The presence of cataplexy along with EDS is strongly suggestive of narcolepsy.

    Patients with narcolepsy may experience sleep paralysis, in which the patient is not able to move when he/she wakes up and sometimes when asleep with consciousness intact. Often hallucinations accompany it. Sleep paralysis occurs less frequently when patients sleep in uncomfortable positions. The person can be rescued from this condition by sensory stimuli, like touch or talking with that person.

    Sleep-related hallucinations may be either hypnagogic (i.e. occurring at sleep onset) or hypnopompic (i.e. occurring at awakening). These hallucinations are usually vivid (dreamy) either tactile, visual or auditory in character.

    At disease onset, children with narcolepsy and cataplexy may display a wide range of motor disturbances that do not corroborate with cataplexy as it is classically. The motor disturbances, can be negative (hypotonia) or active (e.g. perioral movements, dyskinetic-dystonic movements, or stereotypic movements), and are seen to settle later during the course of disease [16].

    In children aged 5-10 years, inattentiveness is the most common initial complaint, followed by recurring sleepiness and later on by difficulty in morning arousal associated with aggressive behavior and abrupt falls in school [17]. These children often were misdiagnosed as having attention deficit hyperactivity disorder (ADHD), learning disability, depression, or another neurological disorder. In children aged 10-12 years, poor academic performance is a common complaint [17].

    Physically, narcoleptic patients are normal.

    • Occasionally there will be introductions of blurred vision or diplopia due to their sleepiness.[]
    • […] have HLA-DR2 and/or HLA-DQw1 Clinical Narcolepsy tetrad; if accompanied by cataplexy, Pt feels a sense of absolute urgency for sleep in often inappropriate situations–while standing, eating, carrying on conversations, accompanied by blurred vision, diplopia[]
    • Slurred speech and visual symptoms such as blurred vision or diplopia may be experienced.[]
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  • psychiatrical
    Auditory Hallucination
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  • musculoskeletal
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  • respiratoric
    Sleep Apnea
    • Narcolepsy VS Sleep Apnea Whilst often discussed in parallel as common Sleep Disorders, Narcolepsy and Sleep Apnea are distinctly different in cause, treatment and outcomes.[]
    • apnea) disorders or medical issues.[]
    • This test can also detect sleep apnea.[]
    • These include sleep apnea , circadian rhythm sleep disorders and restless legs syndrome .[]
    • Even when clear-cut cataplexy is present a sleep study is necessary to rule out sleep apnea and other possible sleep disorders contributing to EDS.[]
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  • neurologic
    Sleep Attack
    • These sleep attacks can last from a few seconds to more than an hour.[]
    • (n) a chronic and usually hereditary sleep disorder in which the person affected will suffer spontaneous bouts of sleep (or "sleep attacks") during the day.[]
    • Narcolepsy is a lifelong sleep disorder that makes you feel overwhelmingly tired, and in severe cases, have sudden uncontrollable sleep attacks.[]
    • The deficiency of hypocretin might produce sleep attacks.[]
    • These "sleep attacks" can occur at any time, during any activity.[]
    Automatic Behavior
    • Automatic behavior Automatic behavior is the performance of routine tasks by a person who is not consciously controlling the activity.[]
    • "Narcolepsy and automatic behavior: A case report".[]
    • Automatic behavior may occur in 60% to 80% of patients with narcolepsy.[]
    • Some describe “automatic behavior”, such as driving past a highway exit or writing off a page, with no memory of the previous few minutes.[]
    • Some people with narcolepsy experience automatic behavior during these brief episodes.[]
    • Randomized trial of modafinil as a treatment for the excessive daytime somnolence of narcolepsy: US Modafinil in Narcolepsy Multicenter Study Group.[]
    • It reclassifies narcolepsy without cataplexy as major somnolence disorder (MSD).[]
    • While cognitive impairment does occur, it may only be a reflection of the excessive daytime somnolence.[]
    • […] power in response to strong emotion - it always and only occurs as part of narcolepsy. [ 1 ] The term 'narcolepsy' was first used by Gelineau in 1880 to describe a pathological state of daytime sleepiness and is a term derived from the Greek 'seized by somnolence[]
    • Narcolepsy Type 1 (which used to be defined as Narcolepsy with Cataplexy) is described as a combination of hyper somnolence and have high levels of Hypocretin and also muscle weakness triggered by emotions Narcolepsy Type 2 (which used to be defined as[]
    • In the 1960s, fenethylline was widely recommended to people suffering from ailments like hyperactivity and narcolepsy .[]
    • Sleepiness doesn’t always LOOK sleepy Narcolepsy’s sleepiness may manifest as hyperactivity, irritability, moodiness, attention deficits, fogginess, or memory problems.[]
    • Early-stage Narcolepsy has some unique clinical features compared with later onset cases, including daytime sleepiness manifesting primarily as habitual napping or irritability and hyperactivity In Adults – Research indicates that over 50% of Narcolepsy[]
    Drop Attack
    • Cataplexy - may mimic drop attacks, syncope , seizures/epilepsy , transient ischaemic attack , periodic paralysis (channelopathies), psychiatric disorder, or cataplectic-like episodes (the latter can occur in healthy people).[]
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  • Entire body system
    • Loss of muscle tone in the legs may cause a person to fall down.[]
    • A person with signs of narcolepsy will experience extreme fatigue and may fall asleep at odd and inappropriate times, such as during work or school.[]
    • People with narcolepsy fall asleep without warning, anywhere, anytime.[]
    • Three additional symptoms are typically associated with narcolepsy: cataplexy (short-lived intermittent muscle weakness), hypnogogic and hypnopompic hallucinations (hallucinations while falling asleep or waking), and sleep paralysis (paralysis while falling[]
    Excessive Daytime Sleepiness
    • Narcolepsy, Hypersomnia and Excessive Daytime Sleepiness Narcolepsy and hypersomnias refer to a group of disorders for which excessive daytime sleepiness (ESD) is the core symptom, but when it is not due to insufficient sleep (sleep deprivation), disturbed[]
    • daytime sleepiness and abnormal REM sleep. [5] The first, excessive daytime sleepiness (EDS), occurs even after adequate night time sleep.[]
    • It causes fragmented night sleep and excessive daytime sleepiness.[]
    • In some cases, excessive daytime sleepiness is the only symptom.[]
    • Many medical conditions result in fatigue, thus physicians might not consider narcolepsy.[]
    • She battled depression in addition to having constant fatigue.[]
    • A person with signs of narcolepsy will experience extreme fatigue and may fall asleep at odd and inappropriate times, such as during work or school.[]
    • Many medical conditions result in fatigue, thus physicians might not consider Narcolepsy.[]
    • Other Causes of Persistent Fatigue.[]
    • The disorder is sometimes confused with insomnia due to its characteristic disturbed night-time sleep and with epilepsy because of unexplained sudden falls caused by cataplexy.[]
    • Although, sever symptoms do have the potential to impact lifestyle quality similar to that of Parkinson’s or Epilepsy.[]
    • Misdiagnoses include epilepsy, depression, and schizophrenia. 4.[]
    • According to his pastor, Myers suffers from narcolepsy and epilepsy.[]
    • Hypersomnias are subdivided into Idiopathic Hypersomnia (persistent sleepiness lasting more than 3 months without abnormal tendencies to enter REM sleep) and Recurrent Hypersomnia (recurrent episodes of sleepiness that are entirely reversible in between[]
    • 2018 Scholarship Application In 2018, Project Sleep plans to award seven scholarships of 1000 each , including one scholarship to a student with idiopathic hypersomnia, supported through a collaboration with the Hypersomnia Foundation .[]
    • . [24, 25 ] It is noteworthy that low CSF histamine levels have not been found in patients with hypersomnia secondary to obstructive sleep apnea syndrome. [25] The CSF histamine level may serve as a biomarker reflecting the degree of hypersomnia of central[]
    • The hypersomnia is not better explained by another condition (sleep disorder, medical or neurological condition, mental disorder, substance misuse or medication).[]
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  • Workup

    Sleep studies are an essential part of the evaluation of patients with probable chances narcolepsy. An overnight polysomnogram (PSG) followed by a multiple sleep latency test (MSLT) can provide strongly suggestive evidence of narcolepsy while excluding other sleep disorders.

    Human leukocyte antigen (HLA) typing may provide collateral data, but it is more useful for excluding the diagnosis by documenting that the patient does not have either DQB1*0602 or DQA1*0602.

    Measurement of hypocretin (orexin) levels in the cerebrospinal fluid (CSF) may help establish the diagnosis [9]. CSF hypocretin levels lower than 110 pg/mL are included in the diagnostic criteria for narcolepsy in the second edition of the International Classification of Sleep Disorders (ICSD-2).

    Imaging studies such as MRI are useful for excluding rare causes of symptomatic narcolepsy. Idiopathic narcolepsy may be seen in cases where there are structural abnormalities of the brainstem and diencephalon. MRI of brain in patients suffering from secondary narcolepsy, may show different abnormalities that can be related to the underlying cause.


    HLA Type
    • […] driving or while at work and prefer shift work as 'drowsiness' is more socially acceptable; it may also be accompanied by sleep paralysis Prevalence 1:600–Japan, 1:2400–US, 1:500, 000–Israel, : ratio 1:1, onset age 15– 35; 98-100% of narcoleptics have HLA-DR2[]
    • DR2 and HLA DQ1.[]
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  • Test Results

    Other Test Results
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  • EEG
    Multiple Sleep Latency Test
    • Testing in a sleep laboratory, with polysomnography and multiple sleep latency testing, is needed to confirm the diagnosis.[]
    • A specific study called a multiple sleep latency test is a necessary part of the evaluation for narcolepsy.[]
    • Sleep Latency Test (MSLT).[]
    • For the multiple sleep latency test, a person is given a chance to sleep every 2 hours during normal wake times.[]
    • The multiple sleep latency test is done a few hours after the polysomnography.[]
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  • Treatment

    Treatment of narcolepsy has two components i.e medical and auxiliary management. Good sleep hygiene is a must. Improvement is seen when the patients have a regular sleep schedule, which is generally 7.5-8 hours of sleep every night. Scheduled naps during the day also may help [18].

    Pharmacologic treatment of narcolepsy involves the use of central nervous system (CNS) stimulants such as amphetamine, methylphenidate, modafinil, methamphetamine, and dextroamphetamine sulfate. With the help of these medicines daytime sleepiness can be reduced, thereby improving the symptoms in 65-85% of patients.

    Tricyclic antidepressants such as protriptyline, imipramine and clomipramine, are effective for cataplexy. Sodium oxybate is highly effective for cataplexy, as it helps in improving night time sleep, which is often poor in narcolepsy. Selective serotonin reuptake inhibitors (SSRIs) or serotonin and norepinephrine reuptake inhibitors (SNRIs) suppress REM sleep, thus helping to alleviate the symptoms of cataplexy, hypnotic hallucinations and sleep paralysis. They include fluoxetine and venlafaxine.


    If the disease is managed and treated properly, then patients suffering from narcolepsy can have good life at home and work too. But if not treated, then it can be psychosocially disastrous [12].

    Narcoleptic children may suffer from bad performance in school, social impairment, mocked by the colleagues, and there would be flaws in various activities seen in normal childhood development.


    Sleep Apnea Syndrome
    • apnea syndrome. [25] The CSF histamine level may serve as a biomarker reflecting the degree of hypersomnia of central origin. [ 24 , 25] CNS nuclei for wakefulness and the relevant neurotransmitters generated in those nuclei include the following: Locus[]
    • apnea syndrome (SAS), periodic limb movements in sleep syndrome (PLMS), or others.[]
    • See Insomnia , Narcolepsy tetrad, Sleep apnea syndrome , Sleep disorders . nar·co·lep·sy ( nahr'kō-lep-sē ) A sleep disorder that usually appears in young adulthood, consisting of recurring episodes of sleep during the day, and often disrupted nocturnal[]
    Obstructive Sleep Apnea
    • Emerging drugs for common conditions of sleepiness: obstructive sleep apnea and narcolepsy.[]
    • sleep apnea syndrome. [25] The CSF histamine level may serve as a biomarker reflecting the degree of hypersomnia of central origin. [ 24 , 25] CNS nuclei for wakefulness and the relevant neurotransmitters generated in those nuclei include the following[]
    • Other characteristics People with narcolepsy may have other sleep disorders, such as obstructive sleep apnea, a condition in which breathing starts and stops throughout the night, restless legs syndrome and even insomnia.[]
    • Narcolepsy also can be associated with other sleep disorders, such as obstructive sleep apnea , restless legs syndrome , and insomnia .[]
    • A PSG can help reveal disturbances in REM sleep that are commonly seen in narcoleptics, and can eliminate the possibility that symptoms are caused by another condition, such as obstructive sleep apnea.[]
    • These episodes can be frightening, and you may become depressed because of the sudden lack of control.[]
    • Although it has been linked with blood pressure management and depression, the genesis of narcolepsy is unknown.[]
    • Avoid alcohol and other central nervous system depressants.[]
    • Especially for young people, the condition can cause embarrassment, anxiety and depression, particularly if peers and loved ones are not supportive.[]
    • Narcolepsy is often mistaken for depression , epilepsy , or the side effects of medications.[]
    • Since 2009, clinicians have observed greater rates of abrupt onset in young children who are obese and likely to experience premature puberty.[]
    • Obesity People with narcolepsy are more likely to be overweight.[]
    • Accompanying Physical Problems Obesity.[]
    • Patients who have narcolepsy with cataplexy have been found to be more impulsive and more prone to binge eating than patients without cataplexy and controls. [ 28 ] There is a high risk of obesity and metabolic syndrome. [ 10 ] Prognosis [ 4 , 29 ] Symptoms[]
    • Sleep deprivation is associated with high blood pressure and obesity , and people who sleep less than six hours each night lower their resistance to viral infection by 50 percent.[]
    • For more insider perspectives, check out 5 Ridiculous Myths You Probably Believe About Schizophrenia and 4 Things You Learn Having A Disease Doctors Can't Diagnose .[]
    • Misdiagnoses include epilepsy, depression, and schizophrenia. 4.[]
    • The hallucinations can sometimes be so strong that this symptom can be mistaken for schizophrenia.[]
    • Even worse, hypnagogic hallucinations may result in diagnoses of schizophrenia or bipolar disorder, which are treated with potent antipsychotic drugs that have severe side effects and are useless for narcolepsy.[]
    • The disorder is sometimes confused with insomnia due to its characteristic disturbed night-time sleep and with epilepsy because of unexplained sudden falls caused by cataplexy.[]
    • Although, sever symptoms do have the potential to impact lifestyle quality similar to that of Parkinson’s or Epilepsy.[]
    • Misdiagnoses include epilepsy, depression, and schizophrenia. 4.[]
    • According to his pastor, Myers suffers from narcolepsy and epilepsy.[]
    • Narcolepsy Network Cure Narcolepsy Now Narcolepsy Institute National Narcolepsy Registry Stanford Center for Narcolepsy Volunteer Blood for Narcolepsy Research Center for Narcolepsy Research Narcolepsy & Cataplexy Foundation of America (NCFA) Florida[]
    • Help – Narcolepsy Networks & Support Groups Narcolepsy Network – Wake Up NarcolepsyNarcolepsy Association – UK – Center for Narcolepsy, Stanford School of Medicine – USA – We recommend[]
    Sleep Paralysis
    • The hallucinations are called “hypnagogic hallucinations” when they occur while the person is falling asleep, and “hypnopompic hallucinations” when they happen during awakening.[]
    • Hallucinations may occur at this time.[]
    • Sleep hallucinations Sleep hallucinations may occur at sleep onset or upon wakening.[]
    • Hypnopompic hallucinations refer to the same sensations while awakening from sleep.[]
    • Hypnagogic hallucinations and excessive daytime sleepiness (EDS) symptoms Hypnagogic hallucinations Hypnagogic hallucinations may be present in up to 50% of patients with narcolepsy.[]
    Auditory Hallucination
    Tactile Hallucination
    • Hypnagogic hallucinations: dreamlike visual, auditory or tactile hallucinations in which elements of the waking world are incorporated just before falling asleep.[]
    • Sometimes accompanied by visual, auditory or tactile hallucinations as REAL as reality.[]
    • Thomas Kilkenny, he laughed and assured her she wasn’t going crazy, but that, given her past sleeping problems and recent visual, auditory, and tactile hallucinations, she most likely had narcolepsy.[]
    • […] ptosis Management Pre-planned 'catnaps' throughout day, analeptics–ie, long-term stimulants–eg, methylphenidate, dextroamphetamine or tricyclic antidepressants that inhibit re-uptake of norepinephrine and serotonin; MAOIs may eventually cause tardive dyskinesia[]
    Hypnagogic Hallucination
    • Hypnagogic hallucinations Hypnagogic hallucinations are vivid dreamlike experiences that are difficult to distinguish from reality.[]
    • Hypnagogic hallucinations and excessive daytime sleepiness (EDS) symptoms Hypnagogic hallucinations Hypnagogic hallucinations may be present in up to 50% of patients with narcolepsy.[]
    • Hypnagogic hallucinations: vivid, often frightening, dream-like experiences that occur while dozing or falling asleep.[]
    • Hypnagogic hallucinations These occur at the onset of sleep (hypnagogic) or on awakening (hypnopompic).[]
    • Hypnagogic hallucinations are vivid, often frightening, dreamlike experiences that occur while dozing or falling asleep.[]


    The exact cause of narcolepsy isn't known. Narcolepsy has a genetic component and tends to run in families. An estimated 8 to 10% of people with narcolepsy have a close relative who has the disorder. Narcolepsy most likely involves a combination of genetics and one or more environmental provoking factors like infection, trauma, hormonal changes, immune system disorders, or stress.


    Psychological Stress
    • stress may also play a role. [1] Diagnosis is typically based on the symptoms and sleep studies , after ruling out other potential causes. [1] Excessive daytime sleepiness can also be caused by other sleep disorders such as sleep apnea , major depressive[]


    Narcolepsy affects about 1 in 2,000 people. The prevalence of narcolepsy in the US is 0.02-0.18%, which is comparable to that of multiple sclerosis [2] [3]. The frequency among first-degree relatives is 1-2%. Narcolepsy with cataplexy affects 0.02% of adults worldwide [4].

    The male-to-female ratio in narcolepsy is 1.64:1. The disease has highest peak of occurrence at 15 years and a less marked peak at 36 years.

    Sex distribution
    Age distribution


    Narcolepsy is considered to be an outcome of susceptible genes, defective neurotransmitter functioning and sensitivity, and aberrant immune modulation. Recent studies blame some human leukocyte antigen (HLA) subtypes and abnormal hypocretin (orexin) neurotransmission, that causes unexpected changes in monoamine and acetylcholine synaptic transmissions, and more accurately in the reticular activating system of the pons [5] [6].

    The centrality of hypocretin transmission in the pathophysiology of narcolepsy was demonstrated when hypocretin knockout mice displayed cataplexy and sleepiness [7] [8]. Further evidence for impaired hypocretin functioning in humans was found with the discovery of low levels of hypocretin in the cerebrospinal fluid (CSF) of narcoleptic patients [9].

    Subsequently, abnormal immune modulation was associated with the clinical development of narcolepsy in children in Scandinavia and Finland. It was found that the chances of narcolepsy increased to 8-12 folds in Finnish children after vaccination against the H1N1 influenza virus with a vaccine that used a potent ASO3 adjuvant. Every affected child who went through HLA typing was seen having the HLA DQB*0602 allele [10] [11].


    Narcolepsy cannot be prevented but the frequency of the narcolepsy episodes can be reduced in those suffering from it by avoiding the triggering factors or the conditions that causes the attack.


    Narcolepsy is a chronic sleep disorder characterized by overwhelming daytime drowsiness and sudden bouts of sleep. It is difficult for a person suffering from narcolepsy to stay awake for long periods of time, whatever be the circumstances. It is characterized by the classic tetrad of excessive sleepiness during the daytime, cataplexy, hypnotic hallucinations, and sleep paralysis. It is also known as hypnolepsy, defined as a chronic neurological disorder caused by autoimmune destruction of hypocretin-producing neurons inhibiting the brain's ability to regulate sleep-wake cycles normally.[1]

    Patient Information

    Narcolepsy is a medical disorder characterized by excessive, sudden bouts of uncontrollable sleep during the day. These patients cannot sleep well at night and a polysomnogram is used to diagnose the condition.

    The condition cannot be prevented as the exact cause is not understood but the symptoms can be controlled very well lifelong with medications like antidepressants, and stimulants. It is not a fatal condition but it can lead to accidents that are life-threatening.

    Patients with narcolepsy should avoid heavy meals and alcohol. Patients should take scheduled short naps and participate in an exercise programs. They must avoid driving, operating heavy machinery, or any other activity that can have detrimental effect when sleepy. In one survey, almost 75% of patients with narcolepsy reported falling asleep while driving, and 56% reported nearly having accidents. In addition to these accidents, there are various other accidents that associated with narcolepsy like burns due to coming in close contact with hot objects, cuts from handling sharp objects, and breaking things. Patients with narcolepsy with cataplexy should wear a life preserver when engaged in water activities. They should not do water activities all by themselves. They should inform and also educate others about their cataplectic attacks.

    People with narcolepsy have problems with memory, thinking, and attention. They suffer from emotional and social difficulties caused by their uncontrollable sleep episodes and cataplexy. They are prone to suffer from depression, severe emotional and social dysfunction in all areas, be it work, relationships or any relaxing activity. Generally it is seen that men with narcolepsy suffer from sexual problems.


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    1. Narcolepsy Information Page: National Institute of Neurological Disorders and Stroke (NINDS)". 18 July 2013. Retrieved 3 March 2014.
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    3. Silber MH, Krahn LE, Olson EJ, Pankratz VS. The epidemiology of narcolepsy in Olmsted County, Minnesota: a population-based study. Sleep. 2002 Mar 15;25(2):197-202
    4. Dauvilliers Y, Arnulf I, Mignot E. Narcolepsy with cataplexy. Lancet. 2007 Feb 10;369(9560):499-511.
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    11. Nohynek H, Jokinen J, Partinen M, Vaarala O, et al. AS03 adjuvanted AH1N1 vaccine associated with an abrupt increase in the incidence of childhood narcolepsy in Finland. PLoS One. 2012;7(3):e33536.
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    Media References

    1. 1R02 crystallography, Public Domain