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Source: Dopp JM, Phillips BG. Sleep Disorders. In: DiPiro JT, Talbert RL, Yee GC, Matzke GR, Wells BG, Posey LM, eds. Pharmacotherapy: A Pathophysiologic Approach. 8th ed. Accessed June 4, 2012.

  • Sleep disorder characterized by excessive sleepiness and sleep attacks at inappropriate times.

  • Precise cause unknown.
  • Genetic component (3% of patients have first-degree relative with disorder).
  • Possible environmental influences.

  • The hypocretin/orexin neurotransmitter system may be involved. Autoimmune process may cause destruction of hypocretin-producing cells.

  • Affects 0.03–0.06% of adult Americans.
  • Incidence may be higher in men than women.
  • Usually occurs in second decade of life and increases in severity through third and fourth decades.
  • Can occur in children and adolescents.

  • Family history of narcolepsy
  • Head injury
  • Obesity
  • History of meningitis or encephalitis

Signs and Symptoms

  • Complaints of:
    • Excessive daytime sleepiness
    • Sleep attacks that last up to 30 minutes
    • Fatigue
    • Impaired performance
    • Disturbed nighttime sleep
  • Multiple arousals during the night

Means of Confirmation and Diagnosis

  • Essential features include:
    • Sleep attacks
    • Cataplexy (sudden bilateral loss of muscle tone with collapse, often precipitated by emotional situations)
    • Hypnagogic hallucinations before sleep or during a sleep attack
    • Sleep paralysis (flaccid muscles with full consciousness while falling or waking from sleep)
  • Sleep study demonstrating abrupt transition into REM sleep necessary for diagnosis.

  • Maximize alertness during waking hours.
  • Improve quality of life.

  • Encourage good sleep hygiene.
  • Recommend 2 or more brief daytime naps daily; as little as 15 minutes may be beneficial.

  • Pharmacotherapy focuses on excessive daytime sleepiness and cataplexy (Table 1).
  • Excessive daytime somnolence
    • Modafinil 200 mg each morning standard treatment.
      • No evidence of tolerance or withdrawal after abrupt discontinuation.
      • Side effects include:
    • Armodafinil (the active R-isomer of modafinil) is also available.
    • Amphetamines and methylphenidate have higher likelihood of abuse and tolerance.
    • Selegiline may also be beneficial.
  • Cataplexy
    • Most effective treatments:
      • Imipramine
      • Protriptyline
      • Nortriptyline
      • Fluoxetine
      • Venlafaxine
    • Selegiline may also improve cataplexy.
  • Sodium oxybate (γ-hydroxybutyrate; potent sedative-hypnotic) improves excessive daytime sleepiness and decreases episodes of sleep paralysis, cataplexy, and hypnagogic hallucinations.
    • Taken at bedtime and repeated 2.5–4 hours later.
    • Side effects include:
      • Nausea
      • Somnolence
      • Confusion
      • Dizziness
      • Incontinence

Table 1. Pharmacotherapy of Narcolepsy

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