Health Questions & Answers #64 ~ [Issue 0505-4]
May 27, 2005 09:41 PDT
The following information regarding the disorder known as
"narcolepsy" was excerpted from a FAQ (frequently asked questions)
document found at http://www.narcolepsynetwork.org/
Q & A #1:
What is narcolepsy?
Narcolepsy is a sleep disorder of neurological origin, characterized
by excessive daytime sleepiness. It can begin at any age and
continues throughout life. It frequently becomes noticeable during
the teens or early twenties but it can also appear later in life.
Predisposition to it seems to be hereditary. It is believed to affect
approximately 1 out of 1000 people of both sexes and all races. It is
not degenerative; people with narcolepsy can expect to live a normal
Q & A #2:
What are the symptoms?
There are four primary symptoms:
1. Excessive Daytime Sleepiness (EDS) includes daytime sleep attacks,
which may occur with or without warning (and for many are
irresistible); persistent drowsiness, which may continue for
prolonged periods of time; and "microsleeps", or fleeting moments of
sleep intruding into the waking state.
2. Cataplexy (the other hallmark symptom of narcolepsy) is a sudden
loss of voluntary muscle control, usually triggered by emotions such
as laughter, surprise, fear or anger. It occurs more frequently
during times of stress or fatigue. The cataplectic attack may involve
only a slight feeling of weakness and limp muscles (such as sagging
facial muscles, a nodding head, buckling knees, loss of arm strength,
'garbled' speech); but it may also result in immediate total body
collapse, during which the person may appear unconscious, but remains
awake and alert. These attacks may last from a few seconds up to
The two other symptoms are hypnagogic hallucinations - vivid,
realistic, often frightening dreams; and sleep paralysis, or a
temporary inability to move. Either one of these can occur during the
process of going to sleep or waking up, while the brain is partially
asleep and partially awake.
Q & A #3:
Are there other symptoms?
The following secondary or auxiliary symptoms may appear:
1. Automatic behavior, the performance of a routine task, without
conscious awareness of doing it, and often without later memory of it;
2. Disrupted nighttime sleep, involving multiple arousals.
Other difficulties may be caused by the primary symptoms, appear as
side effects of medication, or result from one's continuing struggle
to cope. Feelings of intense fatigue and continual lack of energy are
often reported, and depression is also common. The ability to
concentrate and memorize may become more difficult. Vision (focusing)
problems, eating "binges," weak limbs, and difficulties in handling
alcohol may also occur.
Q & A #4:
How are these symptoms all related to narcolepsy?
Narcolepsy is related to REM (rapid eye movement) sleep, the dreaming
portion of sleep. As a protection against acting out dreams, the
muscles become immobile or "paralyzed." For the normal person, a
sleep period first progresses for about 90 minutes of non-REM sleep
and then REM sleep begins. But for a person with narcolepsy, sleep
begins almost immediately with REM sleep. Since the brain may not be
totally asleep when dreaming begins, the dream is sometimes
experienced far more vividly and is thought of as a hallucination.
After waking, REM periods, or fragments of REM, occur inappropriately
throughout the day. This explains excessive daytime sleepiness.
Cataplexy is related to the muscle "paralysis" of REM. When automatic
behavior occurs, sleep has partially overtaken the brain, but the
body continues to perform familiar tasks.
Q & A #5:
Is narcolepsy a psychological or mental disorder?
Narcolepsy is a neurological disorder. Medical researchers have
recently identified the cause as the absence of a neurotransmitter,
normally present in the hypothalamus region of the brain, which
produces the hypocretin peptide essential to the human sleep-wake
cycle. Nevertheless, psychological problems can develop from
misunderstanding of and difficulty in coping with the symptoms. A
very difficult fact to understand for one with narcolepsy and those
around him or her, is that sleepiness and sleep attacks are
uncontrollable. Failure to accept this may seriously influence
self-esteem or personal relationships. Health care counseling for
persons and families with narcolepsy can help alleviate these
secondary problems. Educating the public, especially school, health,
and human resource personnel can help lessen or prevent many other
Q & A #6:
Does narcolepsy affect learning?
Although narcolepsy does not directly affect one's intelligence,
learning and education cannot help but be affected by the symptoms.
Study, concentration, memory, and attention span may be periodically
impaired by sleep. Children with narcolepsy should be identified at
the earliest possible age to avoid lowered self-esteem and a pattern
of failure adjustments in learning habits may be continually
necessary. This can be accomplished with the cooperation of school
Q & A #7:
Is cataplexy dangerous?
Mild cataplexy, while perhaps embarrassing, is not dangerous. One can
often find support for weakened head, neck, or arm muscles, so that
others may not even be aware of the momentary loss of control.
However, severe cataplexy, resulting in immediate and sudden body
collapse, can be dangerous. Companions should be told in advance what
to expect and how to help. They should always check for the person's
safety and comfort immediately relieving any unnatural bending of
limbs or unusual body positions, assuring complete relaxation, and
then allowing him or her to recover spontaneously. Cataplexy for
others can be so instantaneous that there is no time to prepare for
safety and serious injury can occur. Some deaths and near-deaths have
been reported. Obviously, potentially life-threatening situations
should be avoided unless cataplexy is controlled.
Q & A #8:
How is a diagnosis of narcolepsy determined?
Excessive daytime sleepiness (EDS) is often the first symptom to
appear, and for some, the ONLY symptom of narcolepsy. However, it is
also a symptom of various other medical conditions. Cataplexy, on the
other hand, is almost unique to narcolepsy. The combination of EDS
and cataplexy allow clinical diagnosis of narcolepsy, but the
presence of cataplexy is not required for a diagnosis of narcolepsy.
In most cases, laboratory tests are still needed to confirm diagnosis
and determine a treatment plan. The usual procedure includes an
overnight polysomnogram (PSG) at a sleep disorders center to
determine the presence of EDS and perhaps other underlying causes of
this symptom. This is followed by the Multiple Sleep Latency Test
(MSLT) which measures sleep onset and how quickly REM sleep occurs.
The MSLT is the most widely accepted diagnostic test for narcolepsy.
Finally, a genetic blood test has been developed which measures
certain antigens often found in people who have a predisposition to
narcolepsy. Positive results suggest but do not prove narcolepsy.
This test is sometimes used when the diagnosis is in question.
Q & A #9:
How is narcolepsy treated?
The goal is to increase daytime alertness and to lessen recurring
cataplexy, using minimal medication. Excessive Daytime Sleepiness
(EDS) and cataplexy are treated separately. Traditionally, central
nervous system stimulants (e.g. Ritalin, Dexedrine) have been used
for EDS. In 1999, Provigil (modafinil) was approved as the first
non-amphetamine wake-promoting drug for EDS. Tricyclic
antidepressants and serotonin reuptake inhibitors (e.g. Vivactil,
Tofranil) have been used for cataplexy and REM symptoms. A new drug,
Xyrem (sodium oxybate) also known as gamma-hydroxybutyrate (GHB)
received FDA approval and became available by prescription in October
2002. In addition to drug therapy, 2 or 3 short naps during the day
help to control sleepiness and maintain alertness. Proper diet and
regular exercise also help. And some report benefits from alternative
remedies, such as herbs, phosphates, and acupuncture. Continuing
doctor-patient communication is necessary. Equally important is
educating one's family, friends, teachers, and co-workers about
narcolepsy. Joining a support group is recommended.