Narcolepsy is a chronic condition characterized by a disruption of the sleep-wake cycle and rapid eye movement (REM) sleep intrusion.
The classic tetrad of narcolepsy, includes excessive daytime sleepiness, cataplexy, sleep paralysis, and hypnagogic/hypnopompic hallucinations.
The only clinical manifestation that is specific to narcolepsy is cataplexy.
Disease onset is usually in the second decade of life.
Multiple sleep latency test usually shows shortened sleep latency and at least 2 sleep-onset REM periods.
Therapy involves lifestyle modification, stimulants for daytime sleepiness, and drug treatment for cataplexy.
Narcolepsy is a chronic sleep boundary disorder that affects the control of sleep and wakefulness with rapid eye movement sleep intrusion into the wake state. In its classic form, it is characterized by a tetrad of excessive daytime sleepiness, cataplexy (generalized muscle weakness leading to partial or complete collapse), hypnagogic/hypnopompic hallucinations (visual or auditory perceptions on falling asleep or on awakening), and sleep paralysis.
History and exam
Key diagnostic factors
- excessive daytime sleepiness
- hypnagogic/hypnopompic hallucinations
- sleep paralysis
Other diagnostic factors
- chronic fatigue or tiredness
- poor performance at work
- poor memory and concentration
- car accidents
- slurred speech
- blurred vision
- irregular breathing pattern
- sleep attacks
- fragmented nocturnal sleep
- symptoms of other sleep disorders
- status cataplecticus
- low cerebrospinal fluid hypocretin
- human leukocyte antigen (HLA)-DQB1*0602
- Prader-Willi syndrome
- Niemann-Pick disease type C
- linkage to 4p13-q21
- genes on chromosome 6 or chromosome 21
- hypothalamic tumors
- hypothalamic infarct/hemorrhage
- head trauma
- central nervous system infection
- central nervous system arteriovenous malformations
- multiple sclerosis
- myotonic dystrophy
1st investigations to order
- actigraphy and sleep diary
- overnight polysomnography
- multiple sleep latency test (MSLT)
Investigations to consider
- HLA typing
- cerebrospinal fluid hypocretin-1 level
- maintenance of wakefulness test
adults with excessive daytime sleepiness (EDS)
adults with cataplexy
children with excessive daytime sleepiness (EDS)
children with cataplexy
- Untreated sleep apnea
- Periodic limb movements of sleep
- Restless legs syndrome (RLS)
- Treatment of central disorders of hypersomnolence
- European guideline and expert statements on the management of narcolepsy in adults and children
Sleep apnea in adults (obstructive)
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