- Narcolepsy is a neurologic sleep disorder characterized by excessive daytime sleepiness (EDS) and may be associated with cataplexy (sudden loss of muscle control), hypnagogic hallucinations (vivid perceptual experiences while falling asleep), hypnopompic (vivid perceptual experiences while waking up from sleep), or sleep paralysis (temporary inability to move or speak that happens during transition from sleeping to awake state).
- Mainly two types identified by American Academy of Sleep Medicine:
- Type 1 (60–70%) (formerly narcolepsy with cataplexy) and type 2 (formerly narcolepsy without cataplexy) (1)
- No cure, symptomatic management
- Affects 0.03 to 0.16% general population with incidence 1/2,000 worldwide (2)
- Bimodal distribution; age of onset peak at 15 years and again at 35 years of age.
- Predominant sex: male > female (1.6:1)
- 5% familial cases (1)
- African Americans are more likely to present without cataplexy and at a younger age.
- Narcolepsy type 1: 25 to 50/100,000 people
- Narcolepsy type 2: 20 to 34/100,000 people (1)
Etiology and Pathophysiology
- Primarily caused by degeneration of hypothalamic neurons that produce orexin (hypocretin). Postmortem studies show 85% loss of these neurons in type 1 and about 33% loss in type 2 (1).
- Orexin is crucial to promote wakefulness, it stimulates the RAS (reticular activating system) and inhibits rapid eye movement.
- Neurodegeneration may be caused by autoimmune process, probably stimulated by infections (such as influenza A or group A Streptococcus) or environmental factors (occurs commonly in late spring).
- Alterations in the L-PGDS (lipocalin-type prostaglandin D synthase) level also seem to be involved in causing EDS in narcolepsy (1).
- International Classification of Sleep Disorders, 3rd edition (ICSD-3) classification:
- Narcolepsy type 1
- Narcolepsy type 2
- Narcolepsy due to medical conditions
- Usually sporadic but increased incidence in families with positive history: 1–2% in first-degree relative of the index case (10 to 40 times the general population)
- 98% of patients with narcolepsy type 1 have human leukocyte antigen (HLA) DQB1*0602; 40–50% of patients with narcolepsy type 2 express this antigen. HLA DQB1*0602 is present in 12–30% of the general population.
- Autosomal recessive inheritance pattern
- Affects 12% of Asians and 25% of whites, and 38% of African Americans are gene carriers.
- Age (peaks at 15 and 35 years), usually underdiagnosed before age of 18
- Head trauma
- Central nervous system infectious disease
- Psychological stress
- Positive family history
- Recent influenza A, streptococcal infection, or H1N1 vaccine (no evidence of COVID leading to narcolepsy yet) (3)
Commonly Associated Conditions
Obstructive sleep apnea (OSA) (up to 25%), obesity, anxiety
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Domino, Frank J., et al., editors. "Narcolepsy." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2020. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688548/all/Narcolepsy.
Narcolepsy. In: Domino FJF, Baldor RAR, Golding JJ, et al, eds. 5-Minute Clinical Consult. Wolters Kluwer; 2020. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688548/all/Narcolepsy. Accessed May 30, 2023.
Narcolepsy. (2020). In Domino, F. J., Baldor, R. A., Golding, J., & Stephens, M. B. (Eds.), 5-Minute Clinical Consult (27th ed.). Wolters Kluwer. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688548/all/Narcolepsy
Narcolepsy [Internet]. In: Domino FJF, Baldor RAR, Golding JJ, Stephens MBM, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2020. [cited 2023 May 30]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688548/all/Narcolepsy.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Narcolepsy ID - 1688548 ED - Domino,Frank J, ED - Baldor,Robert A, ED - Golding,Jeremy, ED - Stephens,Mark B, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688548/all/Narcolepsy PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -