Overview
Narcolepsy is a disorder that
causes a person to have difficulty staying awake. In extreme cases, narcolepsy
can cause a person to suddenly fall asleep during the day. These "sleep
attacks" occur even after getting enough sleep at night. The unusual sleep
pattern that people with narcolepsy have can affect their schooling, work, and
social life.
The two main types of sleep are rapid eye movement
(REM) sleep and nonrapid eye movement (NREM) sleep. During REM sleep, your eyes
move rapidly even though your eyelids stay shut. Dreaming mainly happens during
REM sleep, when your body becomes limp and you are unable to move your muscles.
This temporary inability to move prevents you from acting out any dreams that
you may be having.
Normally, when people fall asleep they first have
NREM sleep. Then they go through a period of REM sleep. People with narcolepsy
have a different sleep pattern. They often fall into REM sleep before NREM
sleep. Also, in people with narcolepsy, the fine line between being asleep and
being awake can be blurred. They often find that certain aspects of REM sleep
can happen while they are awake. This causes them to:
- Suddenly lose muscle tone and control when awake
(cataplexy). This can appear to be muscle weakness and sometimes can trigger
the body to collapse.
- Not be able to move or speak while falling asleep
or waking up (sleep paralysis).
- Have vivid dreams while falling asleep or waking
up (hallucinations).
Effects of Narcolepsy
People with narcolepsy often fall asleep without
warning at inappropriate times. Sleep attacks don't just happen during
quiet times, such as when reading or watching TV. These attacks can occur
during driving, eating, or other activities and can cause:
- Accidents and injuries
- Problems at work or at school
- Social problems
- Impaired memory, thinking, or ability to
concentrate
- Depression
Causes
Research suggests that the cause of narcolepsy is a
lack of the chemical in the brain called hypocretin. This chemical stimulates
brain cells and helps promote wakefulness. It is not known why hypocretin is
missing in people who have narcolepsy. Some factors that may work together to
cause a lack of hypocretin include:
- Infection
- Loss of certain brain cells due to brain injury,
toxins, and/or the body's destruction of its own tissues (autoimmune
reaction)
- Changes in hormones
- Stress
One out of 10 people with narcolepsy and cataplexy
has a close relative with the same symptoms. This suggests that some people may
inherit the tendency to develop narcolepsy.
Who Is At Risk for Narcolepsy?
Narcolepsy may affect 150,000 (1 in 2,000) people or
more in the United States. It usually first occurs between the ages of 15 and
30 and affects both men and women. The symptoms can start suddenly or appear
gradually. The condition is difficult to diagnose without medical tests. Often
people live with mild symptoms, such as daytime sleepiness and muscle weakness,
for several years before narcolepsy is diagnosed. Narcolepsy can also develop
later in life or in children, but it is rare before age 5. Factors that may
increase the risk of developing narcolepsy include having:
- A brother, sister, or parent with narcolepsy
- Certain thyroid disorders
- Diabetes
- A disease in which the body's infection
fighting system mistakenly attacks the body's own organs and tissues (an
autoimmune disorder)
Symptoms
The major symptom of narcolepsy is excessive daytime
sleepiness with sleep attacks. People with narcolepsy may also have one or more
of the following signs and symptoms:
- Sudden loss of muscle tone and control (muscle
weakness) over parts or all of the body while awake (cataplexy)
- Sudden inability to move or speak while falling
asleep or waking up (sleep paralysis)
- Vivid dreams while falling asleep or waking up
(hallucinations)
Daytime Sleepiness
All people with narcolepsy have excessive daytime
sleepiness. This is usually the first symptom of the condition. They also may
have sudden, irresistible urges to sleep during the day (sleep attacks). They
usually fall asleep for just a few seconds or minutes. Rarely, they may fall
asleep for as long as an hour or more. Daytime sleepiness can cause:
- Mental cloudiness or "fog"
- Memory problems
- Problems focusing
- Lack of energy
- Depression
- Extreme exhaustion
Cataplexy
Nearly 3 out of 4 people with narcolepsy (75
percent) experience sudden muscle weakness while they are awake. This is called
cataplexy, and it is similar to the paralysis that occurs during dream sleep.
Mild attacks of muscle weakness can cause:
- Head nodding
- Drooping eyelids
- Difficulty speaking
- Difficulty moving arms or hands or a weakened
grip
- Buckling of the knees
Severe attacks of narcolepsy may cause complete
paralysis and falls. Attacks often last less than 2 minutes, and they may only
last a few seconds. During both mild and severe attacks, the person stays fully
conscious.
The sudden attacks of muscle weakness in narcolepsy
can happen at any time. But these attacks are often triggered by strong
emotions, including:
- Laughter
- Anger
- Fear
- Excitement
Attacks of muscle weakness and tone are sometimes
the first symptom of narcolepsy that is noticed. But usually this symptom
occurs weeks or months after people who have narcolepsy first start to
experience excessive sleepiness during the day.
Sleep Paralysis
People with narcolepsy may suddenly not be able to
move or speak while falling asleep or waking up. They are fully conscious
during these periods of sleep paralysis. The paralysis usually lasts just a few
seconds or minutes, but it can be scary. Sleep paralysis is similar to the
paralysis that happens in rapid eye movement (REM) sleep. Not all people with
narcolepsy have sleep paralysis.
Hallucinations
Some people with narcolepsy have vivid dreams while
they are falling asleep, waking up, or dozing. These hallucinations differ from
normal dreams because they seem very real and include sights, sounds, smells,
tastes, and touch. People with narcolepsy may say these hallucinations are
scary like a nightmare. The hallucinations can occur with sleep paralysis.
Other Symptoms
Some people with narcolepsy have difficulty staying
asleep through the night. These frequent awakenings may start years after
experiencing the first symptoms of narcolepsy. Another symptom of narcolepsy is
to carry out certain actions without awareness. This is called automatic
behavior. For example, if people with this symptom are writing, they may
scribble rather than form words. When driving, people with automatic behavior
may get lost or have an accident because of periods when they aren't aware
of what they are doing. People usually do not remember such automatic
behavior.
Diagnosis
Doctors will base a diagnosis of narcolepsy on the
patient's symptoms, family history of narcolepsy, physical exam, and test
results. The symptoms people have are often distinct enough for doctors to
diagnose narcolepsy. But laboratory tests are needed to confirm the diagnosis
and determine the best treatment. These tests may require the patient to visit
a sleep disorders center for an overnight evaluation.
Symptoms and Family History
If your doctor suspects you have narcolepsy, he or
she will ask whether you have:
- Daytime sleepiness and sleep attacks
- Sudden and temporary losses of muscle tone and
control (cataplexy)
- Vivid dreams while falling asleep or waking up
(hallucinations)
- An inability to move or speak while falling
asleep or waking up (sleep paralysis)
If you have any of these symptoms, your doctor will
want to know when you first developed them and if they interfere with sleep or
daily activities. The doctor may also ask questions about your sleep habits and
how you feel and act during the daytime. To help you answer those questions,
you may be asked to keep a sleep diary for a few weeks. In this daily dairy you
may be asked to write:
- When you go to bed and get up
- How long it takes you to fall asleep, and how
often you wake up during the night
- If you snore loudly and frequently, or wake up
gasping or feeling out of breath
- How refreshed you feel when you wake up, and how
tired you feel during the day
- How often you doze off or have trouble staying
awake during the day
Doctors also usually ask whether you have symptoms
of other sleep disorders that cause daytime sleepiness. You may also be asked
if you have any relatives with narcolepsy.
Physical Exam
Your doctor will examine you to see if you have
signs of other possible causes for your symptoms. These causes include:
- Infections
- Certain thyroid diseases
- Drug or alcohol use
- Other medical or sleep disorders
This part of the diagnosis usually requires your
doctor to do a complete physical exam.
Sleep Specialist
If your doctor suspects you have narcolepsy, he or
she will likely suggest you see a sleep specialist who may recommend special
sleep tests. The sleep specialist will confirm a diagnosis of narcolepsy,
diagnose another sleep disorder, or rule out a specific sleep disorder as the
cause of your symptoms, based on:
- The results of your sleep tests
- Your symptoms
- Your sleep habits as recorded in a sleep
diary
Narcolepsy is easier to diagnose if you have more
symptoms than just daytime sleepiness and sleep attacks.
Sleep Tests
Sleep tests are usually done at a sleep disorders
center. For some sleep tests, you may need to sleep overnight at the center.
Other sleep tests can be done during the day. The three tests most often used
to diagnose narcolepsy are:
- Polysomnogram (PSG)
- Multiple sleep latency test (MSLT)
- Hypocretin test
Polysomnogram
For this study, you sleep overnight at a sleep
center. While you are sleeping, the staff at the center use various devices to
measure your brain activity, breathing, and movements. The signs of narcolepsy
this test can reveal include:
- Falling asleep quickly
- Entering rapid eye movement (REM) sleep soon
after falling asleep
- Waking up often during the night
Multiple Sleep Latency Test
This test is usually done during the day after an
overnight PSG. Also called a nap study, the MSLT measures how easy it is for
you to fall asleep during the day. You are asked to take short naps about every
2 hours. The test records eye movements, muscle tone, and brain activity with
small devices attached to the head. The signs of narcolepsy this test can
reveal are quickly falling asleep during the day (after a full night's
sleep) and entering REM sleep soon after falling asleep.
Hypocretin Test
This test measures the levels of hypocretin in the
fluid that bathes your spinal cord. Low levels of hypocretin make it likely
that you have narcolepsy.
Treatment
There is no cure for narcolepsy, but many of the
symptoms of this disorder can be relieved with medicines and lifestyle changes.
Treatment for narcolepsy is based on the type and severity of symptoms. Some
medicines help relieve daytime sleepiness, while other medicines may help
prevent the sudden loss of muscle tone and movement (cataplexy) or vivid dreams
while falling asleep or waking up (hallucinations) that some people with
narcolepsy have. Not all medicines work for everyone. It may take weeks to
months for your doctor to find the best treatment for you.
Medicines To Relieve Daytime
Sleepiness
Doctors may prescribe stimulants to increase daytime
alertness in narcolepsy patients, including:
- Modafinil
- Pemoline
- Methylphenidate
- Amphetamines
Modafinil is the newest stimulant for treating
daytime sleepiness in people with narcolepsy. Modafinil may not be strong
enough to relieve sleepiness in everyone with narcolepsy, and Modafinil is
sometimes prescribed with another stimulant to give more complete relief from
daytime drowsiness.
Medicines To Relieve Other
Symptoms
Several different antidepressant medicines can be
used to treat the sudden loss of muscle tone (cataplexy), vivid dreams while
falling asleep and waking up (hallucinations), and inability to move or speak
when falling asleep or waking up (sleep paralysis) that affect some people with
narcolepsy. These medicines affect the chemicals in the brain that seem to play
a role in narcolepsy. People with cataplexy need to check with their doctor to
find which treatment will work best for them.