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Narcolepsy

What is Narcolepsy?

Narcolepsy is a strange and frustrating disorder. It is a serious disorder, and can be dangerous.

Narcolepsy is a chronic neurological sleep disorder characterized by excessive sleepiness and attacks of unexpected, uncontrollable sleep even at inappropriate times, like while working.
The onset of symptoms is usually in adolescence or early adulthood.

These sleep attacks have 4 key features:

  1. Excessive daytime sleepiness
  2. Cataplexy
  3. Sleep paralysis
  4. Sleep hallucinations
  1. Excessive daytime Sleepiness
    • Excessive sleepiness is the most common and the most disabling symptom of Narcolepsy.
    • This sleepiness can result in naps which may be brief (for a few seconds or minutes) or longer (for 2-3 hours).
    • These sleep attacks are unexpected, occur without warning and are uncontrollable and irresistible.
  2. Cataplexy
    • Cataplexy is the sudden loss of muscle tone when the individual is awake causing the individual to fall or collapse, or feel weakness of the body.
    • It is usually triggered by extremes of emotion like anger, excitement and laughter. It can also occur due to strong coughing.
  3. Sleep Paralysis
    • Sleep paralysis is the temporary inability to move the body or even talk while waking up from sleep (hypnapompic) or while falling asleep (hypnagogic).
    • It can last for a few seconds, or even for a few minutes.
    • Although frightening, recovery is immediate and complete.
  4. Sleep Hallucinations
    • Sleep hallucinations are vivid dreamlike experiences and imagery that occur while waking up from sleep (hypnapompic) or while falling asleep (hypnagogic).
    • They can be intensely detailed and even involve multiple senses like visual, tactile (touch and feel) or kinetic (movements).

What Causes Narcolepsy?

Narcolepsy is thought to occur due to the deficiency of a protein called Hypocretin which controls sleep and appetite in the part of the brain called the hypothalamus.

The deficiency can be in Hypocretin 1 and/or Hypocretin 2.

Pathophysiology of Narcolepsy

  • Normally, when we go to sleep, it takes a short while to actually fall asleep. This is known as sleep latency.
  • The body cycles through different stages of sleep, REM and non-REM. REM sleep stage is normally entered after about 90 minutes of falling asleep. These REM cycles occur about 4-5 times through the night.
  • In Narcolepsy, the first REM cycle begins rapidly after falling asleep – within 8 minutes or less after sleep onset. This is called Sleep Onset REM, or SOREM.
  • The lack of muscle control, occurrence of dreams, and sleep paralysis that normally occur during REM sleep occur even at other times in individuals with Narcolepsy – even when they are not asleep.

Symptoms of Narcolepsy

The classic symptoms are known as the “Tetrad of Narcolepsy”.

  1. Excessive Sleepiness
    • Excessive sleepiness is the most common and the most disabling symptom of Narcolepsy.
    • This is usually the first symptom to appear and persists throughout life.
    • Individuals fall into deep sleep quickly and wake up suddenly. They have detailed vivid dreams and can dream even if they have been asleep only for a few seconds.
    • These sleep attacks are unexpected, occur without warning and are uncontrollable and irresistible.
  2. Cataplexy
    • Episodes of loss of muscle function ranging from slight weakness to complete collapse of the body occur.
    • These episodes are triggered by emotions, laughter, and coughing.
    • They last for a few seconds to a few minutes and the individual remains conscious throughout.
  3. Sleep Paralysis
    • Sleep paralysis is the temporary inability to move the body or even talk while waking or falling asleep.
    • It can last for a few seconds, or even for a few minutes.
    • Although frightening, recovery is immediate and complete.
  4. Sleep Hallucinations
    • Sleep hallucinations are vivid dreamlike experiences and imagery that occur while falling asleep, waking up, or dozing.

The classic tetrad of symptoms may not occur in all patients.

Lifestyle Impact of Narcolepsy

Narcolepsy needs to be taken seriously as it can have a negative impact on lifestyle.

  • Accidents, depression, social isolation, weight gain and decreased quality of life can occur due to the symptoms of Narcolepsy.

Narcolepsy doesn’t just have a negative impact on lifestyle, it can be dangerous!

  • If sleep attacks or cataplexy occur while driving, operation machinery or using sharp objects, serious injuries and even death can occur.

Narcolepsy can affect mental health as well.

  • It can result in depression, anxiety, and impaired memory, attention and concentration.

Narcolepsy can negatively impact relationships.

  • Spousal relationships are often strained. Sleepiness can also cause decreased interest in sex and impotence.
  • Professional relationships become difficult to maintain. Co-workers may find it amusing, job performance is affected, and superiors may assume that the patient is lazy or faking.

Diagnosis of Narcolepsy

Narcolepsy is diagnosed based on the International Classification of Sleep Disorders 2 (ICSD-2) diagnostic criteria.

The criteria include:

  1. Excessive daytime sleepiness every day for at least 3 months.
  2. A definite history of cataplexy triggered by emotions or coughing.
  3. A polysomnogram (sleep study) followed by Multiple Sleep Latency Test showing.
    • Mean Sleep Latency less than 8 min.
    • 2 or more SOREMs
  4. hypocretin-1 level in the cerebrospinal fluid (fluid around the brain and spinal cord) less than 110pg/ml

The Multiple Sleep Latency Test (MSLT) is a sleep test where the patient is allowed to sleep every 2 hours during the day and it is observed how long it takes to reach different stages of sleep.)

Treatment of Narcolepsy

Narcolepsy is frustrating and dangerous. It affects not only the individual, but also the sleep partner and can pose a potential danger to others in dangerous situations.

However, Narcolepsy can be treated and controlled with lifestyle changes and medications.

Lifestyle Changes:

  1. Good sleep hygiene
    • Individuals need to have regular and strict bedtimes and waking times.
    • Caffeine intake in the afternoon, and exercise late in the evenings should be avoided.
    • Smoking should be avoided as well.
    • It is advised to go to bed only when really sleepy and to avoid using the bedroom for anything other than sleep or sex.
    • Distracters in the bedroom like electronic equipment (including TV, computers and radio) should be removed.
  2. Scheduling short naps 2-3 times a day
    • This is helpful in controlling excessive daytime sleepiness
    • School age children should be permitted to schedule these naps even during school hours. A doctor’s note to this effect will help.
    • Avoid activities that would be dangerous if a sudden sleep attack occurred.
    • Avoid driving, using heavy machinery, climbing ladders and using sharp objects.

Medications

  1. Medications to reduce sleepiness
    • Medications like Modafinil (Provigil) and Armodafinil (Nuvigil) are stimulants of the brain, known as Central Nervous System Stimulants.
  2. Medications to consolidate sleepiness
    • Xyrem Oral Solution, containing Sodium Oxybate (also known as Gamma Hydroxybutyrate or GHB) helps consolidate sleep and reduce fragmented sleep.
    • It is especially helpful when narcolepsy is associated with cataplexy, excessive daytime sleepiness, or disrupted sleep.
  3. REM Suppressors
    • Certain antidepressants suppress REM sleep. This is useful in treating Narcolepsy symptoms.
    • These drugs reduce cataplexy, relieve hypnagogic and hypnapompic hallucinations and reduce seep paralysis.
    • The commonly used drugs in this category are Serotonin Norepinephrine Reuptake Inhibitors (SNRIs) like Venlafaxine (EffexorSR) or Norepinephrine Reuptake Inhibitors like Atomoxetine (Strattera); Selective Serotonin Reuptake Inhibitors (SSRIs) like Fluoxetine (Prozac) and Sertraline (Zoloft) .
    • Older Tricyclic antidepressants like Protriptyline Triptil and Vivactil), Imipramine (Tofranil and Janimine), Desipramine (Norpramine and Pertofran) and Clomipramine (Anafranil) are also very useful.

Further Information on Narcolepsy

For further information regarding Narcolepsy, contact:

Narcolepsy Network

Call toll free : (888) 292-6522
129 Waterwheel Lane
North Kingstown, RI 02852