Narcolepsy

Aka: Narcolepsy, Gelineau Syndrome, Paroxysmal Sleep, Narcoleptic Syndrome

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II. Epidemiology

  1. Prevalence: 40 per 100,000 (0.02 to 0.18% of adults)
  2. Men and women affected equally
  3. Onset in teens and young adults, ages 10 to 20 years old (rarely has onset after age 50 years)

III. Pathophysiology

  1. Excessive Sleepiness
  2. Abnormal REM Sleep

IV. Causes

  1. Idiopathic
    1. Hereditary related to DR-2
  2. Secondary causes
    1. Head Trauma
    2. Encephalopathy
    3. Brain Tumor
    4. Cerebrovascular insufficiency

V. Symptoms: Classic Tetrad

  1. Recurrent irresistible Daytime Sleepiness
    1. Occurs unexpectedly and at inappropriate times
  2. Cataplexy (25-30% of patients with Narcolepsy)
    1. Sudden decrease or loss of voluntary Muscle tone following emotional trigger (e.g. laughing, surprise)
    2. Episodes last seconds to minutes
      1. Localized hypotonia (e.g. jaw drop, head nod, knee sag)
      2. Generalized hypotonia (full collapse onto floor)
  3. Sleep Hallucinations
    1. Hypnagogic Hallucinations (on falling asleep)
    2. Hypnopompic Hallucinations (on awakening)
  4. Sleep Paralysis
    1. Transient, generalized inability to move or speak during sleep-wake transition

VI. Differential Diagnosis

  1. See Hypersomnolence
  2. Sleep Apnea
  3. Other Primary Hypersomnia (uncommon)
    1. Idiopathic Hypersomnia
    2. Menstrual Hypersomnia
    3. Kleine-Levin Syndrome (rare syndrome of male teens)

VII. Evaluation

  1. See Hypersomnolence

VIII. Diagnosis: Sleep Study (all patients)

  1. Sleep log or Actigraphy for 2 weeks
  2. Multiple Sleep Latency Test (daytime nap test)
    1. Polysomnogram performed for monitoring
    2. At least 2 naps with early onset REM Sleep (Rapid transition to REM)
    3. Shortened REM latency (<8 minutes compared with 15 minutes for unaffected patients)

IX. Management: General Measures

  1. Schedule naps
  2. Keep a consistent sleep schedule
  3. Practice Sleep Hygiene
  4. Plan Caffeine use prior to times of needed wakefulness

X. Management: Standard Medications

  1. See Hypersomnia
  2. First-Line Stimulants for Excessive Daytime Sleepiness
    1. Modafinil (Provigil)
      1. Best safety profile and lowest abuse potential of all stimulant agents
    2. Armodafinil (Nuvigil, generic)
    3. Solriamfetol (Sunosi, expensive)
  3. Other stimulants (risk of dependence) for Excessive Daytime Sleepiness
    1. Methylphenidate (Ritalin)
    2. Dextroamphetamine (Dexedrine)
  4. Symptomatic management of Cataplexy, Sleep Paralysis or hypnagogic Hallucinations
    1. SNRI (e.g. Venlafaxine) and SSRI agents (e.g. Fluoxetine) suppress REM Sleep
    2. Clomipramine (Anafranil)
  5. Combined stimulant and Cataplexy agents
    1. Gamma hydroxybutyric acid or Sodium oxybate (Xyrem)
      1. Given twice nightly
    2. Pitolisant (Wakix)
      1. Fewer adverse effects as Sodium oxybate with similar efficacy
      2. Xu (2019) Sleep Med 64:62-70 [PubMed]

XI. References

  1. Billiard (2008) Neuropsychiatr Dis Treat 4(3):557-66 [PubMed]
  2. Holder (2022) Am Fam Physician 105(4): 397-405 [PubMed]
  3. Pagel (2009) Am Fam Physician 79(5): 391-6 [PubMed]
  4. Thorpy (2006) CNS Drugs 20(1): 43-50 [PubMed]
  5. Ramar (2013) Am Fam Physician 88(4): 231-8 [PubMed]

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Related Studies

Ontology: Narcolepsy (C0027404)

Definition (MSHCZE) Záchvatové onemocnění, při němž postižený náhle proti své vůli upadá do krátkého spánku (imperativní spavost), a to často opakovaně během dne a ve zcela nečekaných situacích. Ohrožuje nemocného např. pádem nebo úrazem. Ze spánku jej lze obvykle probudit. Součástí nemoci bývá kataplexie. Patří mezi dyssomnie. Bývají přítomny disociované poruchy REM spánku – spánková obrna, hypnagogické halucinace. Příčina není plně objasněna, existuje vztah k antigenu HLADR2. Onemocnění vzniká obv. v dospívání, má chronický ráz, může mít invalidizující charakter, k zmírňování zpravidla dochází v pozdějším věku. V léčbě se uplatňují centrální stimulancia (amfetaminového typu), antidepresiva, clomipramin. Význam má i úprava denního režimu s možností opakovaného krátkého spánku přes den. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) A sleep disorder characterized by a tendency for excessive sleepiness during the day which occurs even after adequate sleep in the nighttime. The persons who suffer from this condition experience fatigue and may fall asleep at inappropriate times during the day.
Definition (MSH) A condition characterized by recurrent episodes of daytime somnolence and lapses in consciousness (microsomnias) that may be associated with automatic behaviors and AMNESIA. CATAPLEXY; SLEEP PARALYSIS, and hypnagogic HALLUCINATIONS frequently accompany narcolepsy. The pathophysiology of this disorder includes sleep-onset rapid eye movement (REM) sleep, which normally follows stage III or IV sleep. (From Neurology 1998 Feb;50(2 Suppl 1):S2-S7)
Definition (CSP) recurrent, uncontrollable brief episodes of sleep and lapses in consciousness, often associated with hypnagogic hallucinations, cataplexy, automatic behaviors and sleep paralysis.
Concepts Disease or Syndrome (T047)
MSH D009290
ICD9 347.0
ICD10 G47.419 , G47.41
SnomedCT 155059003, 267702006, 60380001
DSM4 347
English Narcolepsy, NARCOLEPSY, Syndrome, Gelineau's, paroxysmal sleep, narcolepsy, narcolepsy (diagnosis), Gelineau Syndrome, Gelineau's Syndrome, Gelineaus Syndrome, Syndrome, Gelineau, Narcoleptic Syndrome, Narcoleptic Syndromes, Syndrome, Narcoleptic, Syndromes, Narcoleptic, Paroxysmal Sleep, Sleep, Paroxysmal, Narcolepsy NOS, Narcolepsy [Disease/Finding], gelineau's syndrome, narcolepsies, gelineau syndrome, narcolepsy disorder, excessive uncontrollable daytime sleepiness, narcoleptic syndrome, Syndromes, Gelineau's, Gelineau's Syndromes, Gelineau's syndrome, Paroxysmal sleep, Narcoleptic syndrome, Narcolepsy (disorder), gélineau's syndrome
French NARCOLEPSIE, Narcolepsie, Syndrome de Gélineau, Syndrome narcoleptique, Maladie de Gélineau
Portuguese NARCOLEPSIA, Narcolepsia, Síndrome de Gélineau, Sono Paroxístico
Spanish NARCOLEPSIA, Sueño Paroxístico, narcolepsia (trastorno), narcolepsia, sueño paroxístico, síndrome de Gelineau, Narcolepsia, Síndrome de Gelineau
German NARKOLEPSIE, Narkolepsie, Schlafanfall, Gélineau-Syndrom
Swedish Narkolepsi
Japanese ﾅﾙｺﾚﾌﾟｼｰ, 睡眠発作, 持続睡眠, ナルコレプシー
Czech narkolepsie, Narkolepsie, paroxysmální poruchy spánku, narkoleptický syndrom
Finnish Narkolepsia
Russian NARKOLEPSIIA, НАРКОЛЕПСИЯ
Polish Senność napadowa, Narkolepsja
Hungarian Narcolepsia
Norwegian Narkoleptisk syndrom, Paroxysmal søvn, Narkolepsi, Gelineaus syndrom
Croatian NARKOLEPSIJA, Gelineauov sindrom
Italian Sindrome narcolettica, Sindrome di Gelineau, Narcolessia
Dutch narcolepsie, Gelineau-syndroom, Narcolepsie, Paroxismale slaap
Derived from the NIH UMLS (Unified Medical Language System)

Related Topics in Sleep Disorders

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