Monday, February 1, 2016

Narcolepsy Explained

Narcolepsy is a chronic neurological disorder involving the loss of the brain's ability to regulate sleep-wake cycles.  Symptoms include excessive daytime sleepiness, comparable to how people who don't have narcolepsy feel after 24–48 hours of sleep deprivation, as well as disturbed sleep which often is confused with insomnia. Another common symptom of narcolepsy is cataplexy, a sudden and transient episode of muscle weakness accompanied by full conscious awareness, typically (though not necessarily) triggered by emotions such as laughing, crying, terror, etc. affecting roughly 70% of people who have narcolepsy.

The system which regulates sleep, arousal, and transitions between these states in humans is composed of three interconnected subsystems: the orexin projections from the lateral hypothalamus, the reticular activating system, and the ventrolateral preoptic nucleus.  In narcoleptic individuals, these systems are all associated with impairments due to a greatly reduced number of hypothalamic orexin projection neurons and significantly fewer orexin neuropeptides in cerebrospinal fluid and neural tissue, compared to non-narcoleptic individuals.  Those with narcolepsy generally experience the REM stage of sleep within 5 minutes of falling asleep, while people who don't have narcolepsy (unless they are significantly sleep deprived) do not experience REM until after a period of slow-wave sleep, which lasts for about the first hour or so of a sleep cycle.

The term narcolepsy derives from the French word narcolepsie created by the French physician Jean-Baptiste-Édouard Gélineau by combining the Greek νάρκη (narkē, "numbness" or "stupor"), and λῆψις (lepsis), "attack" or "seizure".

Signs and Symptoms

There are two main characteristics of narcolepsy: excessive daytime sleepiness and abnormal REM sleep.  The first, excessive daytime sleepiness (EDS), occurs even after adequate night time sleep. A person with narcolepsy is likely to become drowsy or fall asleep, often at inappropriate times and places, or just be very tired throughout the day. Narcoleptics are not able to experience the amount of restorative deep sleep that healthy people experience – they are not "over-sleeping". In fact, narcoleptics live their entire lives in a constant state of extreme sleep deprivation. Daytime naps may occur with little warning and may be physically irresistible. These naps can occur several times a day. They are typically refreshing, but only for a few hours or less. Vivid dreams may be experienced on a constant or regular basis, even during very brief naps. Drowsiness may persist for prolonged periods of time or simply never cease. In addition, night-time sleep may be fragmented with frequent awakenings. A second prominent symptom of narcolepsy is abnormal REM sleep. Narcoleptics are unique in that they enter into the REM phase of sleep in the beginnings of sleep, even when sleeping during the day.

The classic symptoms of the disorder, often referred to as the "tetrad of narcolepsy," are cataplexy, sleep paralysis, hypnagogic hallucinations, and excessive daytime sleepiness. Other symptoms may include automatic behaviors and night-time wakefulness.  These symptoms may not occur in all patients.

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