NARCOLEPSY

Definition

Narcolepsy is a chronic sleep disorder that involves poor control of sleep-wake cycles. People with narcolepsy experience periods of extreme daytime sleepiness and sudden, irresistible bouts of sleep that can strike at any time.  These “sleep attacks” usually last a few seconds to several minutes. 

Contrary to common beliefs, people with narcolepsy do not spend a substantially greater proportion of their time asleep during a 24-hour period than do normal sleepers.  In addition to daytime drowsiness and uncontrollable sleep episodes, most individuals also experience poor sleep quality that can involve frequent waking during nighttime sleep, and other sleep disorders. 

Narcolepsy affects both males and female equally and appears throughout the world.  Symptoms often start in childhood or adolescence, but can occur later in life.  The condition is life-long. 

Symptoms of Narcolepsy

In most cases, symptoms first appear when people are between the ages of 7 and 25.  In rare cases, however, narcolepsy may appear at younger age or in older adults.  These symptoms include:

Excessive Daytime Sleep (EDS).


EDS is experienced by almost all individuals with narcolepsy and is usually the first to become clinically apparent. Generally, EDS interferes with normal activities on a daily basis, whether or not individuals had sufficient sleep at night.  People with EDS describe it as a persistent sense of mental cloudiness, a lack of energy, a depressed mood, or extreme exhaustion with others experiencing memory lapses, and many have great difficulty maintaining their concentration while at school, work, or home.

People tend to awaken from such unavoidable sleeps feeling refreshed and finding that their drowsiness and fatigue subsides for an hour or two. Involuntary sleep episodes are sometimes very brief, lasting no more than seconds at a time.  Some people with narcolepsy are prone to automatic behavior during these sleep episodes.  Automatic behavior involves performing a task during a short period of sleep but without any apparent interruption.  During these episodes, people are usually engaged in habitual, essentially "second nature" activities such as taking notes in class, typing, or driving.  They cannot recall their actions, and their performance is almost always impaired.  Their handwriting may, for example, degenerate into an illegible scrawl, or they may store items in bizarre locations and then forget where they placed them.  If an episode occurs while driving, individuals may get lost or have an accident.

Cataplexy

Cataplexy is a sudden loss of muscle tone while the person is awake that leads to feelings of weakness and a loss of voluntary muscle control.  Attacks can occur at any time during the waking period, with individuals usually experiencing their first episodes several weeks or months after the onset of EDS.  But in about 10 percent of all cases, cataplexy is the first symptom to appear and can be misdiagnosed as a seizure disorder. 

Cataplectic attacks vary in duration and severity.  The loss of muscle tone can be barely perceptible, involving no more than a momentary sense of slight weakness in a limited number of muscles, such as mild drooping of the eyelids.  The most severe attacks result in a complete loss of tone in all voluntary muscles, leading to physical collapse during which individuals are unable to move, speak, or keep their eyes open.  But even during the most severe episodes, people remain fully conscious, a characteristic that distinguishes cataplexy from seizure disorders

Although cataplexy can occur spontaneously, it is more often triggered by sudden, strong emotions such as fear, anger, stress, excitement, or humor. Laughter is reportedly the most common trigger.

Sleep paralysis


Experiencing sleep paralysis resembles undergoing a cataplectic attack affecting the entire body.  As with cataplexy, people remain fully conscious.  Even when severe, cataplexy and sleep paralysis do not result in permanent dysfunction—after episodes end, people rapidly recover their full capacity to move and speak.




Hallucinations

Hallucinations can accompany sleep paralysis and occur when people are falling asleep, waking, or during sleep.  Referred to as hypnagogic hallucinations when occurring during sleep onset and as hypnopompic hallucinations when occurring during waking, these images are unusually vivid, seem real, and can be frightening.  Most often, the content is primarily visual, but any of the other senses can be involved. 


Disrupted nocturnal sleep

While individuals with narcolepsy have no difficulties falling asleep at night, most experience difficulties staying asleep.  Sleep may be disrupted by insomnia, vivid dreaming, sleep talking, acting out while dreaming, and periodic leg movements.

Obesity

After developing narcolepsy, many individuals suddenly gain weight, a side effect that can be prevented by active treatment.

Causes of Narcolepsy

Narcolepsy may have several causes.  Most people with narcolepsy have low levels of the neurotransmitter hypocretin, which promotes wakefulness.  Neurotransmitters are chemicals that neurons produce to communicate with each other and to regulate biological processes.

 Most cases of narcolepsy are sporadic, meaning the disorder occurs in individuals with no known family history of the disorder.  But clusters in families sometimes occur—up to 10 percent of individuals diagnosed with narcolepsy with cataplexy report having a close relative with the same symptoms. 

In extremely rare cases, narcolepsy is caused by a genetic defect that prevents normal production of hypocretin molecules.  While close relatives of people with narcolepsy have a statistically higher risk of developing the disorder than do members of the general population, that risk remains low when compared to diseases that are purely genetic in origin.

When cataplexy is present, the cause is most often the discrete loss of brain cells that produce hypocretin.  Although the reason for such cell loss remains unknown, it  appears to be autoimmune in nature (an autoimmune disorder is when the body’s immune system mistakenly attacks healthy cells or tissue).  That is, the body’s immune system selectively attacks hypocretin-containing brain cells. 
Other factors appear to play important roles in the development of narcolepsy.  Some rare cases are known to result from traumatic injuries to parts of the brain involved in REM sleep or from tumor growth and other disease processes in the same regions.
 
Infections, exposure to toxins, dietary factors, stress, hormonal changes such as those occurring during puberty or menopause, and alterations in a person's sleep schedule are just a few of the many factors that may exert direct or indirect effects on the brain, thereby possibly contributing to disease development.

EDS, the most common of all narcoleptic symptoms, can be the result of a wide range of medical conditions, including other sleep disorders such as sleep apnea, various viral or bacterial infections, mood disorders such as depression, and chronic illnesses such as anemia, congestive heart failure, and rheumatoid arthritis that disrupt normal sleep patterns. 

Some medications can also lead to EDS, as can consumption of caffeine, alcohol, and nicotine. 
Finally, sleep deprivation has become one of the most common causes of EDS. 

Behavioral Strategies that Help People Cope with Narcolepsy

Drug therapy should accompany various behavioral strategies according to the needs of the affected individual.

Many individuals take short, regularly scheduled naps at times when they tend to feel sleepiest. 
Improving the quality of nighttime sleep can combat EDS and help relieve persistent feelings of fatigue.  Among the most important measures people can take to enhance sleep quality are:
  • maintain a regular sleep schedule—go to bed and wake up at the same time every day
  • avoid alcohol and caffeine-containing beverages for several hours before bedtime
  • avoid large, heavy meals just before bedtime
  • avoid smoking, especially at night
  • maintain a comfortable, adequately warmed bedroom environment, and
  • engage in relaxing activities such as a warm bath before bedtime.

Exercising for at least 20 minutes per day at least 4 or 5 hours before bedtime also improves sleep quality and can help people with narcolepsy avoid gaining excess weight.

Safety precautions, particularly when driving, are particularly important for all persons with narcolepsy.  EDS and cataplexy can lead to serious injury or death if left uncontrolled.  Suddenly falling asleep or losing muscle control can transform actions that are ordinarily safe, such as walking down a long flight of stairs, into hazards.  People with untreated narcoleptic symptoms are involved in automobile accidents roughly 10 times more frequently than the general population.  However, accident rates are normal among individuals who have received appropriate medication.


Support groups frequently prove extremely beneficial because people with narcolepsy may become socially isolated due to embarrassment about or misunderstandings related to their symptoms.  Many people also try to avoid strong emotions, since humor, excitement, and other intense feelings can trigger cataplectic attacks. Support groups also provide individuals with a network of social contacts who can offer practical help and emotional support.

Adults can often negotiate with employers to modify their work schedules so they can take naps when necessary and perform their most demanding tasks when they are most alert.  Similarly, children and adolescents with narcolepsy may be able to work with school administrators regarding special needs, including medication requirements during the school day, and to modify class schedules. 

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